Neurology Medication

Common medicines given to neurology patients on discharge from hospital: This leaflet contains brief information about some of the medicines we commonly supply on discharge to patients who have been admitted for various types of neurological conditions. You will not receive all these medicines; you should check to see which you have been given and then read the information for those medicine only. For more detailed information about the medicines you have been given, please read the information leaflet in the packet with the medicines. If you have a specific question about the medicines you have been prescribed, please call the pharmacy helpline number given below. Some of the medicines below may cause drowsiness, and some people may be affected more than others. If you feel drowsy or have difficulty concentrating, you should not drive or operate machinery. Before starting any other new medication, including medicines you buy, you should check with your doctor or a pharmacist. They will make sure the new medicine can safely be taken as well as the medicines you have received from hospital. Monday – Friday 2-4pm 0151 5293208. This is a direct line into the pharmacy for advice on medicines supplied to you by this hospital. For queries not related to medicines please phone the hospital switchboard on 0151 5253611 and ask for the relevant department. These are most commonly used in Parkinson’s disease. Nearly everyone notices a good improvement in symptoms after starting. A low dose is usually started at first. Over time, the dose often needs to be increased to control the symptoms. The main active ingredient of these drugs is levodopa and this is a very effective medicine for the symptoms of Parkinson’s disease. Side-effects sometimes occur when you first take these medicines but are unusual. Most people have no problems with low doses. Read the leaflet in the medicine pack-et for a full list of possible side-effects. Nausea (feeling sick) is the most common. This is less likely to occur if you take a small dose at first, at meal times, and then gradually build it up. If nausea is a problem, it can usually be eased by adding an anti-sickness drug called domperidone. This is usually only needed for the first few weeks. Excessive daytime sleepiness can rarely occur, so do not drive or operate machinery if you are affected in this way. Compulsive behaviour may also occasionally be a side effect, so excessive gambling or shopping, binge eating or excessive interest in sexual activity can develop. However the link between these problems and levodopa is very uncertain (in contrast to some other Parkinson’s drugs. Unfortunately, because Parkinson’s disease is progressive, these medications often work less well over time. Therefore, the dose usually needs to be increased every now and then. Despite this, typically after several years, patients may develop problems due to worsening of the Parkinson’s disease. These include 'on-off' effects. This is where you can switch quite suddenly between being 'on' and able to move, and being 'off' and unable to move freely. Some patients develop uncontrollable jerky movements and other effects (called dyskinesias). These problems related to long-term use can become disabling, tiring and uncomfortable. Neurologists often have to adjust your treatment to deal with these difficulties. Any medicines used in the treatment of Parkinson’s should not be stopped suddenly, unless advised by your doctor, as problems can occur. Duodopa®. contains co-careldopa (see previous page), but instead of being in tablet form, it is a gel that can be continually administered directly into the intestine via a tube, using a pump. Duodopa®. is reserved for advanced Parkinson’s disease with severe symptoms. It is tested first with a non-permanent tube from the nose to the intestine, and if a good response to treatment is seen, a permanent tube is inserted. Any complications with the pump device or the tube should be reported without delay, as this could mean the medicine is not reaching the intestine where it is absorbed into your system. Any medicines used in the treatment of Parkinson’s disease should not be stopped suddenly, unless advised by your doctor, as serious problems can occur These medicines are known as dopamine agonists, and are used in the treatment of Parkinson’s disease, or sometimes in the treatment of restless legs. Ropinirole and pramipexole come as oral tablets and rotigotine comes as a patch that is changed every 24 hours. Replacement rotigotine patches should be placed on different areas (avoiding using the same area for 14 days). The possible side-effects when a dopamine agonist is first started are similar to co-careldopa and co-beneldopa (nausea, etc). However, the side-effects tend to ease off over several days or weeks. If nausea is a problem – it is more likely to happen with these drugs than with levodopa—then it can usually be eased by the anti-sickness drug called domperidone. Drowsiness can occur with dopamine agonists, and it may be severe and affect your ability to drive. Some patients develop sudden sleep episodes. If you develop this side effect you must stop driving and contact your Parkinson’s nurse or neurologist. Compulsive behaviour can also be a side-effect and is more common with this group of drugs than with co-careldopa/co-beneldopa. Compulsive behaviour such as excessive gambling or shopping, binge eating or excessive interest in sexual activity can develop over time. Other side-effects can include confusion, hallucinations and ankle swelling. Please report the development of any of these side effects to your Parkinson’s nurse, GP or neurologist as it is likely that the dose of dopamine agonist will have to be reduced (or the dopamine agonist stopped). There are other less common side effects. Read the leaflet in the medicine packet for a full list of possible side-effects. Any medicines used in the treatment of Parkinson’s disease should not be stopped suddenly, unless advised by your doctor, as problems can occur. Apomorphine is another dopamine agonist, which is used in combination with co-careldopa/co-beneldopa. It needs to be given by an injection under the skin. It is used in people who have Parkinson’s disease in its later stages who have severe 'off' episodes where they become immobile. It can help to reverse these 'off' episodes but its effect only lasts for about an hour, so frequent injections are needed. If very frequent injections are needed, apomorphine may be given as a continuous infusion, using tubing with a needle or cannula at the end that is inserted under the skin so that the drug can flow into your body. Apomorphine is started in hospital under the supervision of a doctor experienced in the treatment of Parkinson’s disease. The anti-sickness medicine domperidone is always needed in the first four to six weeks of apomorphine treatment and needs to be started 48 hours before the first injection is given. Some patients can develop confusion, hallucinations or compulsive behaviours (excessive gambling, shopping, binge eating or increased sexual behaviour), as with other dopamine agonists (see previous page). Skin problems can also occur at injection sites. Please report any of these side effects to your Parkinson’s disease nurse, your GP or your neurologist. Any medicines used in the treatment of Parkinson’s should not be stopped suddenly, unless advised by your doctor, as problems can occur. Entacapone and tolcapone help to prolong the effect of each dose of co careldopa or co beneldopa. These medicines are sometimes advised in addition to co-careldopa or co-beneldopa when symptoms are not well controlled. Stalevo®. contains entacapone and co-careldopa in one tablet. Entacapone should be taken at the same time as dos-es of co-careldopa or co-beneldopa. It may colour the urine reddish-brown but this is harmless. Other people develop too much movement (dyskinesia) which may require a dose reduction. Some patients develop diarrhoea as a side effect of entacapone or tolcapone. If this occurs it will not get better while you are still on the drug. Therefore please contact your Parkinson’s nurse, GP or neurologist as soon as possible as it is very likely that the drug will need to be stopped and an alternative used. Any medicines used in the treatment of Parkinson’s should not be stopped suddenly, unless advised by your doctor, as problems can occur. Rasagiline / Selegiline These medicines are alternatives to co-careldopa and co-beneldopa for early Parkinson’s disease. Most people will require co-careldopa or co-beneldopa eventually. However, if you take rasagiline or selegiline, it may delay the need for co-careldopa or co-beneldopa for months or years. Sometimes these medicines are used in combination with co-careldopa or co-beneldopa in the later stages of Parkin-son’s disease. Certain other medicines should be avoided while you are on rasagiline or selegiline, so check with your doctor or pharmacist before you start on any new medicines, especially antidepressants. This includes “over-the-counter” medicines that are not prescribed. Any medicines used in the treatment of Parkinson’s disease should not be stopped suddenly, unless advised by your doctor, as problems can occur. Amantadine Amantadine is added in to other therapies for the management of symptoms in Parkinson’s disease. Some patients find that amantadine can keep them awake at night time. If this happens the dosing times can be changed so the last dose of the day is taken at lunchtime instead of at night. A few patients develop confusion, hallucinations, a rash or ankle swelling. Any medicines used in the treatment of Parkinson’s disease should not be stopped suddenly, unless advised by your doctor, as problems can occur. Phenytoin / Sodium Valproate / Carbamazepine / Lamotrigine / Oxcarbazepine / Eslicarbazepine / Gabapentin / Pregabalin / Levetiracetam / Topiramate / Phenobarbital / Primidone / Clonazepam / Clobazam / Zonisamide / Ethosuximide / Lacosamide / Retigabine / Vigabatrin There are many medicines used to treat epilepsy. They each come with different brand names. (The brand names are not listed above.) These are all medicines used to treat or prevent seizures (fits). Some of them are also used to treat other conditions such as nerve pain, or to prevent migraines. One of the-se medicines will be prescribed if you have had one or more seizures, or if your doctor thinks you may be at risk of having a seizure, or alternatively to help control pain or prevent migraines. The following information applies to people who are taking these medicines for any of these reasons. You may be started on a low dose to reduce side effects, and then gradually increase up to a higher dose. Some of the more common side effects are drowsiness, dizziness, nausea, headaches, confusion and difficulty co-ordinating movement. Some of these problems may be worse when starting the medicine or increasing the dose, but then improve as you get used to taking the medicine. If these side effects are causing you problems please speak to your doctor. In a very small number of people more serious side effects can occur, such as allergies or problems with your liver, blood or skin. It is important that you read the leaflet provided with your medicine which warns about the symptoms to watch out for which might indicate these more serious effects. If you develop a rash, fever, flu like symptoms, swollen glands or feeling ill in any oth-er way within the first few weeks of starting it may be an allergic reaction. You should consult your GP immediately in case the medicine needs to be stopped. Some of these medicines may reduce the effectiveness of contraceptive pills (‘the pill’ or ‘the mini-pill’) and contraceptive injections, implants and vaginal rings. This could make the contraceptive fail, leading to unwanted pregnancy. Please discuss this with your neurologist or specialist nurse if you are unsure whether this applies to you. Women of child bearing age should also be aware that some of these medicines, if taken during pregnancy, can affect the baby, causing various abnormalities. The risk of this happening, and the type of abnormality possible depends on the exact medicine. You should have been informed about this potential problem. If you would like further information please ask your neurologist or specialist nurse straight away. It is important to take your medicine as prescribed, particularly if you are taking it for epilepsy. Try to get into a daily routine. Forgetting an occasional dose is not a problem for some people who have epilepsy; however, for others this could lead to breakthrough seizures. One of the reasons why seizures can recur is if the medicine to stop them is not taken regularly as prescribed. Note: You should not stop taking a medicine suddenly except on medical advice. If you notice a side-effect, you should ask a doctor for advice before stopping the medicine. Diazepam / Midazolam Some people with epilepsy are prescribed a medicine that a relative or friend can administer in emergencies to stop a prolonged seizure. In most people with epilepsy, seizures do not last more than a few minutes. However, in some cases a seizure lasts longer and a medicine can be used to stop it. A doctor or nurse should give instruction on how and when to administer the medicine. One commonly used medicine for this is diazepam. This can be squirted from a tube into the person’s back passage ('rectal diazepam'). The medicine is absorbed quickly into the bloodstream and so works quickly. More recently a medicine called midazo-lam is often used which is easier to administer. It is squirted into the sides of the mouth (‘buccal midazolam’) where it is absorbed directly into the bloodstream. At present, strictly speaking, midazolam is only licensed in children but it is recommended in national epilepsy guidelines and is commonly prescribed for adults. Pyridostigmine (Mestinon®) Pyridostigmine is used in the treatment of myasthenia gravis (a disease causing muscle weakness) to improve the strength of the muscles. It often needs to be taken frequently throughout the day to manage symptoms. Possible side effects include nausea (feeling sick) and vomiting, increased saliva production, diarrhoea and abdominal cramps. Propantheline Propantheline works in the gut to help to reduce any nausea, vomiting, increased saliva production, diarrhoea and abdominal cramps due to pyridostigmine treatment. It should be taken at least one hour before food or on an empty stomach. Prednisolone Steroids such as prednisolone suppress the immune system. Prednisolone can be used in a variety of different diseases where the body’s own immune system is harming itself. It is different to the 'anabolic' steroids which some athletes and bodybuilders use, which have very different effects. For many diseases, the benefit of taking steroids usually outweighs the side-effects. However, side effects can sometimes be troublesome. You should read the information leaflet that comes with your medicine packet for a full list of possible side-effects. Prednisolone may cause mood or behavior changes especially at the beginning of your treatment or if you are taking a high dose. If you become confused, irritable or start having worrying thoughts about harming yourself, speak with your doctor as soon as possible. Steroids can also increase appetite, and may cause weight gain. If you have diabetes, be aware that steroids may increase your blood sugar levels. Your diabetes treatment may need adjusting when prednisolone is started, stopped or the dose is changed, so discuss this with your doctor or nurse. You should usually take the full day’s dose of prednisolone in one go, in the morning after breakfast, unless you have been told to take it in a different way. If you have been taking prednisolone for more than 3 weeks, you must not stop taking it suddenly. If you need to stop treatment, speak with your doctor who will advise you on how to reduce your dose gradually. If you become ill or come into contact with anyone who has measles, shingles or chicken pox (or suspects they might have them) you must see your doctor as soon as possible. If you have been given a steroid treatment card, carry it with you at all times. Prednisolone tablets are often used in the treatment of myasthenia gravis. A low dose, often on alternate days, is usually enough for people where symptoms only affect muscles around the eye. Higher doses may be needed to prevent symptoms if muscles elsewhere in the body are affected. Patients on steroids for more than a few weeks often need additional medicines to protect them from osteoporosis (bone thinning), which can occur with steroid treatment. Azathioprine An immunosuppressant medicine such as azathioprine may be advised in addition to a steroid medicine. These medicines work by suppressing the immune system. You will need regular blood tests and your doctor here will advise you and your GP about this. There are certain side effects you should watch out for. If you experience any of these speak with your doctor immediately or go to your local accident and emergency department without delay. These side effects include unexplained bruising or bleeding, a high temperature or other sign of an infection, signs of an allergic reaction such as extreme tiredness, dizziness, being sick, diarrhoea, fever, muscle pain or stiffness, skin rash or kidney problems such as a change in the amount or colour of your urine. Methotrexate Methotrexate is an anti-cancer medicine which is also used in other conditions to suppress your immune system. When being used for these other conditions, methotrexate must only be taken once weekly, on the same day each week. Never take it more often than this. You should have been given an information leaflet about methotrexate. If you have not, please tell your nurse, doctor or pharmacist. You should also read the manufacturer’s information leaflet in the packet. You will need regular blood tests and your doctor here will advise you and your GP about this. You should also be given another tablet called folic acid to take regularly while you are on methotrexate. This can help prevent some side effects of methotrexate. If you have not been given folic acid, please ask your doctor about this.CyclophosphamideCyclophosphamide is an anti-cancer medicine that is also used in other conditions to suppress your immune system. If you have been started on cyclophosphamide you should have been given an information leaflet. If you have not, please tell your nurse, doctor or pharmacist. You will need regular blood tests and your doctor here will advise you and your GP about this. You should also read the manufacturer’s information leaflet in the packet. Calcium and colecalciferol (Adcal D3®) There are various brands of calcium and colecalciferol tablets, and we usually use Ad-cal D3®. Colecalciferol is a form of vitamin D. Calcium and vitamin D are important for bone health. Your body needs adequate supplies of vitamin D in order to absorb (take up) the calcium that you eat or drink in your diet. If you are taking steroid tablets you may be given Adcal D3® to help prevent osteoporosis (thinning of the bones). The usual dose is one tablet twice daily. The chewable tablets should be sucked or chewed, not swallowed whole. Dispersible tablets are also available and these should be dispersed in water before taking. Alendronic Acid (Alendronate) Alendronic acid is used to prevent or treat osteoporosis (thinning of the bones). It can help to restore some lost bone, and help to prevent further bone loss. If you are taking steroid tablets you may be given alendronic acid to help prevent osteoporosis as a side effect of the steroid. Read the information sheet that comes with the tablets, and carefully follow the instructions on how to take them. The majority of patients will be started on alendronic acid 70mg tablets. These are taken as a once weekly dose (one tablet each week) that should be taken on the same day every week. You need to take alendronic acid tablets whilst you are sitting up and with plenty of water, as they can cause irritation of your oesophagus (gullet). This can lead to indigestion-type symptoms such as heartburn or difficulty swallowing. After you have taken the tablet, remain sitting up or standing (do not lie down) for at least half an hour. Other side-effects may include diarrhoea or constipation. You should NOT take alendronic acid at the same time as food or milk. It is best to take the tablet first thing in the morning (once weekly only) on an empty stomach, and wait at least half an hour before eating, drinking milk or taking calcium tablets. A rare side-effect from alendronic acid is a condition called osteonecrosis of the jaw. This condition can result in severe damage to the jaw bone. So, if you take alendronic acid, if you experience pain, swelling or numbness of the jaw, a heavy jaw feeling or loosening of a tooth, you should tell your doctor. You should also brush and floss your teeth regularly and go for regular dental check-ups whilst taking alendronic acid. Tell your dentist that you are taking it. Calcium and vitamin D supplements are usually prescribed by your doctor as well as alendronic acid, unless they are sure that you are already getting adequate intake in your dietOmeprazole / Lansoprazole / Ranitidine These medicines all reduce the production of stomach acid, and are used as a treatment for indigestion, heartburn or stomach ulcers. They are also used to prevent such problems developing as side effects from other medicines such as steroids or anti-inflammatory painkillers. If you have been prescribed them just as a preventative while taking medicines such as prednisolone, diclofenac, or ibuprofen, then when the-se medicines are stopped, you should also stop taking the omeprazole / lansoprazole / ranitidine Riluzole Riluzole is a medicine which has been shown to have a beneficial effect on the survival of people with amyotrophic lateral sclerosis – motor neurone disease. It can slow down the progression of the disease. You will need regular blood tests while taking this medicine. Your doctor here will advise you and your GP about these. Propranolol Propranolol is used to prevent migraine attacks. It belongs to a group of medicines called beta blockers and it can also lower blood pressure and heart rate. Propranolol can cause tiredness, coldness of the extremities (fingers, toes and nose), disturbed sleep, nightmares, upset tummy, skin rashes (including worsening of psoriasis) or dry eyes. It may not be suitable if you have asthma or heart disease. You should report any shortness of breath or wheezing to your doctor. Beta blockers should not be stopped suddenly unless on the advice of a doctor. Verapamil Verapamil is often used by neurologists as a first-line treatment for cluster headache. Please note cluster headache will not be mentioned in the manufacturer’s leaflet in the box, since verapamil is not formally licensed for cluster headache. It is more commonly used for various heart problems, but its use by neurologists for cluster headache is well established. Verapamil can cause constipation, and may lower blood pressure and heart rate. High doses are sometimes needed to manage cluster headache, so a heart trace known as an ECG may be required when starting treatment or when the dose is increased. Amitriptyline Amitriptyline is a type of antidepressant but it is often used in the treatment of nerve pain. It may ease the nerve pain within a few days, but it may take two to three weeks. It can take several weeks before you get maximum benefit. Some people give up on their treatment too early. It is best to persevere for at least four to six weeks to see how well it is working. Amitriptyline can sometimes cause drowsiness as a side-effect. This often eases over time. To try to avoid drowsiness, a low dose is usually started at first, and then built up gradually if needed. Also, the full daily dose is often taken at night because of the drowsiness side-effect. A dry mouth is another common side-effect. Frequent sips of water may help with a dry mouth. Blurred vision and sweating can also occur. See the leaflet that comes with the medicine packet for a full list of possible side-effects. Ami-triptyline can cause problems with driving or operating machinery – please do not do these things if you experience drowsiness or blurred vision. Alcohol is best avoided with amitrityline unless specifically advised otherwise by your doctor. Baclofen Baclofen is used to relieve muscle spasms in conditions which affect the nervous system. These include multiple sclerosis, motor neurone disease, cerebral palsy and long-term injuries to the head or back. In these conditions the muscles may cramp or tighten causing spasms. Baclofen works by causing the muscles to relax which reduces pain and discomfort. This can also cause the muscles to feel a little weaker than usual. If your muscle spasms increase after taking baclofen, or if your muscles become weak so that you have difficulty doing things, let your doctor know as your dose may need adjusting. Baclofen should be taken exactly as your doctor tells you and you should read the manufacturer's printed information leaflet in the packet. Try to take baclofen at the same times each day, with or after food where possible to avoid missing any doses. If you do forget to take a dose, take one as soon as you remember unless it is nearly time for your next dose, in which case skip the missed dose. Do not take two doses at the same time to make up. Common side effects of baclofen include drowsiness, nausea and lightheadedness. These may be worse to start with and lessen as you continue taking baclofen. Do not stop taking this medicine without speaking to your doctor first as stopping suddenly may cause unwanted side-effects. If your doctor decides you should stop taking baclofen, your dose will be reduced gradually to prevent these from occurring. If your muscle spasms increase after taking baclofen, or if your muscles become weak so that you have difficulty doing things, let your doctor know as your dose may need adjusting. Neurological examThe type of neurological exam will depend on a person's age. The neurologist may ask questions and conduct relatively simple tests in the office to judge muscle condition, movement, well-being and how well the senses are functioning.Brain imagingImaging tests can help diagnose hydrocephalus and identify underlying causes of the symptoms. These tests may include:Ultrasound. Ultrasound imaging, which uses high-frequency sound waves to produce images, is often used for an initial assessment for infants because it's a relatively simple, low-risk procedure. The ultrasound device is placed over the soft spot (fontanel) on the top of a baby's head. Ultrasound may also detect hydrocephalus prior to birth when the procedure is used during routine prenatal examinations.Magnetic resonance imaging (MRI) uses radio waves and a magnetic field to produce detailed 3D or cross-sectional images of the brain. This test is painless, but it is noisy and requires lying still.MRI scans can show enlarged ventricles caused by excess cerebrospinal fluid. They may also be used to identify underlying causes of hydrocephalus or other conditions contributing to the symptoms.Children may need mild sedation for some MRI scans. However, some hospitals use a very fasversion of MRI that generally doesn't require sedation.Computerized tomography (CT) scan is a specialized X-ray technology that can produce cross-sectional views of the brain. Scanning is painless and quick. But this test also requires lying still, so a child usually receives a mild sedative.Drawbacks to CT scanning include less detailed images than an MRI, and exposure to a small amount of radiation. CT scans for hydrocephalus are usually used only for emergency exams.Care at Mayo Clinic One of two surgical treatments may be used to treat hydrocephalus.Shunt Shunt systemOpen pop-up dialog boxThe most common treatment for hydrocephalus is the surgical insertion of a drainage system, called a shunt. It consists of a long, flexible tube with a valve that keeps fluid from the brain flowing in the right direction and at the proper rate.One end of the tubing is usually placed in one of the brain's ventricles. The tubing is then tunneled under the skin to another part of the body where the excess cerebrospinal fluid can be more easily absorbed_ such as the abdomen or a chamber in the heart.People who have hydrocephalus usually need a shunt system for the rest of their lives, and regular monitoring is required.Endoscopic third ventriculostomy Endoscopic third ventriculostomy is a surgical procedure that can be used for some people. In the procedure, your surgeon uses a small video camera to have direct vision inside the brain. Your surgeon makes a hole in the bottom of one of the ventricles or between the ventricles to enable cerebrospinal fluid to flow out of the brain. Complications of surgery Both surgical procedures can result in complications. Shunt systems can stop draining cerebrospinal fluid or poorly regulate drainage because of mechanical malfunctions, blockage or infections. Complications of ventriculostomy include bleeding and infections.Any failure requires prompt attention, surgical revisions or other interventions. Signs and symptoms of problems may include:Fever Irritability Drowsiness Nausea or vomiting Headache Vision problems Redness, pain or tenderness of the skin along the path of the shunt tube Abdominal pain when the shunt valve is in the abdomen Recurrence of any of the initial hydrocephalus symptoms Other treatments Some people with hydrocephalus, particularly children, may need additional treatment, depending other severity of long-term complications of hydrocephalus. A care team for children may include a: Pediatrician or physiatrist, who oversees the treatment plan and medical care Pediatric neurologist, who specializes in the diagnosis and treatment of neurological disorders in children Occupational therapist, who specializes in therapy to develop everyday skills Developmental therapist, who specializes in therapy to help your child develop age-appropriate behaviors, social skills and interpersonal skills Mental health provider, such as a psychologist or psychiatrist Social worker, who assists the family with accessing services and planning for transitions in care Special education teacher, who addresses learning disabilities, determines educational needs and identifies appropriate educational resources Adults with more-severe complications also may require the services of occupational therapists, social workers, specialists in dementia care or other medical specialists.Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition.

Coping and support With the help of rehabilitative therapies and educational interventions, many people with hydrocephalus live with few limitations. There are many resources available to provide emotional and medical support as you parent a child with hydrocephalus. Children with developmental problems due to hydrocephalus may be eligible for government-sponsored health care and other support services. Check with your state or county social services agency. Hospitals and voluntary organizations serving people with disabilities are good resources for emotional and practical support, as are doctors and nurses. Ask these resources to help you connect with other families who are coping with hydrocephalus. Adults living with hydrocephalus may find valuable information and support from organizations dedicated to hydrocephalus education and support, such as the Hydrocephalus Association. Should you be vaccinated against meningitis? Ask your doctor if you or your child should receive a vaccine against meningitis, once a common cause of hydrocephalus. The Centers for Disease Control and Prevention recommends meningitis vaccination for preteen children and boosters for teenagers. It's also recommended for younger children and adults who may be at increased risk of meningitis for any of the following reasons: Traveling to countries where meningitis is common Having an immune system disorder called terminal complement deficiency Having a damaged spleen or having had the spleen removed Living in a college dormitory Joining the military Preparing for your appointment The timing of diagnosing a child with hydrocephalus may depend on how severe the symptoms are, when problems first appear, and whether there were any significant risk factors during the pregnancy or delivery. In some cases, hydrocephalus may be diagnosed at birth or prior to birth.Well-baby visits It's important to take your child to all regularly scheduled well-baby visits. These visits are an opportunity for your child's doctor to monitor your child's development in key areas, including: Head size, rate of head growth and overall body growth Muscle strength and tone Coordination Posture Age-appropriate motor skills Sensory abilities — vision, hearing and touch Questions you should be prepared to answer during regular checkups might include the following: What concerns do you have about your child's growth or development? How well does he or she eat? How does your child respond to touch? Is your child reaching certain milestones in development, such as rolling over, pushing up, sitting up, crawling, walking or speaking? Preparing for other doctor visitsIf you're seeing a doctor because of the recent onset of symptoms, you'll likely start by seeing your primary care doctor or your child's pediatrician. After an initial evaluation, your doctor may refer you to a doctor who specializes in the diagnosis and treatment of conditions that affect the brain and nervous system (neurologist).Be prepared to answer the following questions about your symptoms or on your child's behalf:In a paper published today in Nature Communications, an international group of collaborators led by researchers at UPMC Children's Hospital of Pittsburgh have identified a genetic cause of a rare neurological disorder marked by developmental delay and loss of coordination, or ataxiaThe disorder, scientists found, is caused by mutations in a protein called GEMIN5--one of the key building blocks of a protein complex that controls RNA metabolism in neurons. No mutations in GEMIN5 were previously linked to any genetic disease."It's just like building a house," said senior author Udai Pandey, Ph.D., associate professor of pediatrics, human genetics and neurology at the University of Pittsburgh School of Medicine. "You take out the most important brick at the base and the whole building falls apart."GEMIN5 is part of a protein complex that regulates a slew of important cellular processes, including development of specialized outgrowths from nerve cells called dendrites and axons. Interestingly, mutations in another key protein of the complex, named survival motor neuron protein, cause a different devastating disorder--spinal muscular atrophy.To gather material for the study, Pittsburgh researchers contacted pediatricians, geneticists and neurologists from all over the globe, eventually collecting data from 30 patient families in 12 different countries.Because isolating live neurons from people isn't possible, researchers had to come up with another way of getting samples for future testing. They collected blood samples from pediatric patients who were referred to neurogenetic clinics with undiagnosed neurological symptoms. Blood samples were then processed to isolate cells that, with careful tinkering in the lab, were reprogrammed into neurons.After comparing genetic material of reprogrammed neurons from sick children with that of unaffected relatives, scientists linked neurologic manifestations of the disease to 26 mutations in the GEMIN5 gene that cause damage to the structure of the protein.Children came into the clinic with non-specific symptoms, such as developmental delay and abnormal gait. Their doctors ran all the possible tests, including assessing a child's metabolic function, to no avail--their conditions had no easy explanation. It was not until we did an extensive genome analysis that we found that these patients had mutations in the GEMIN5 gene."Deepa Rajan, M.D., assistant professor of pediatrics, Pitt School of Medicine, neurologist at UPMC Children's Hospital and co-first author of the study. Thalidomide, sold under the brand names Contergan and Thalomid among others, is a medication used to treat a number of cancers (including multiple myeloma), graft-versus-host disease, and a number of skin conditions including complications of leprosy.^[3] While it has been used in a number of HIV-associated conditions, such use is associated with increased levels of the virus.^[3] It is administered orally.^[3]Common side effects include sleepiness, rash, and dizziness.^[3] Severe side effects include tumor lysis syndrome, blood clots, and peripheral neuropathy.^[4] Use in pregnancy may harm the foetus, including resulting in malformation of the limbs.^[3] In males who are taking the medication, contraception is essential if a partner could become pregnant.^[4] It is an immunomodulatory medication and works by a number of mechanisms including stimulating T cells and decreasing TNF-α production.^[3]Thalidomide was first marketed in 1957 in West Germany, where it was available over the counter.^[5][6] When first released, thalidomide was promoted for anxiety, trouble sleeping, "tension", and morning sickness.^[6][7] While it was initially thought to be safe in pregnancy, concerns regarding birth defects arose in 1961 and the medication was removed from the market in Europe that year.^[6][5] The total number of embryos affected by use during pregnancy is estimated at 10,000, of which about 40% died around the time of birth.^[6][3] Those who survived had limb, eye, urinary tract, and heart problems.^[5] Its initial entry into the US market was prevented by Frances Kelsey at the FDA.^[7] The birth defects caused by thalidomide led to the development of greater drug regulation and monitoring in many countries.^[7][5]Thalidomide causes birth defects.^[10][9][16] The U.S. Food and Drug Administration (FDA) and other regulatory agencies have approved marketing of the drug only with an auditable risk evaluation and mitigation strategy that ensures that people using the drug are aware of the risks and avoid pregnancy; this applies to both men and women, as the drug can be transmitted in semen.^[16]There is a high risk that thalidomide can cause excessive blood clots. There is also a high risk that thalidomide can interfere with formation of various kinds of new blood cells, creating a risk of infection via neutropenia, leukopenia, and lymphopenia, and risks that blood will not clot via thrombocytopenia. There is also a risk of anemia via lack of red blood cells. The drug can also damage nerves, causing peripheral neuropathy that may be irreversible.^[10][9]Thalidomide has several cardiovascular adverse effects, including risk of heart attacks, pulmonary hypertension, and changes in heart rhythm including syncope, bradycardia, and atrioventricular block.^[10][9]It can cause liver damage and severe skin reactions like Stevens–Johnson syndrome. It tends to make people sleepy, which creates risk when driving and operating other machinery. As it kills cancer cells, it can cause tumor lysis syndrome. Thalidomide can prevent menstruation.^[10][9]Other than the above, very common (reported in more than 10% of people) adverse effects include tremor, dizziness, tingling, numbness, constipation, and peripheral edema.^[10][9]Common adverse effects (reported by 1–10% of people) include confusion, depressed mood, reduced coordination, heart failure, difficulty breathing, interstitial lung disease, lung inflammation, vomiting, dry mouth, rashes, dry skin, fever, weakness, and a sense of unwellness.^[10][9]There are no expected pharmacokinetic interactions between thalidomide and other medicines due to its neutral effects on P-glycoprotein and the cytochrome P450 family. It may interact with sedatives due to its sedative action and bradycardic agents, like beta-blockers, due to its bradycardia-inducing effects. The risk of peripheral neuropathy may be increased by concomitant treatment with other agents known to cause peripheral neuropathy.^[17] The risk of venous thromboembolisms with thalidomide seems to be increased when patients are treated with oral contraceptives or other cytotoxic agents (including doxorubicin and melphalan) concurrently. Thalidomide may interfere with various contraceptives, and hence it is advised that women of reproductive age use at least two different means of contraception to ensure that no child will be conceived while they are taking thalidomide.^[17][10][9]As of 2013, eighteen cases of overdoses had been reported with doses of up to 14.4 grams, none of them fatal.^[17] No specific antidote for overdose exists and treatment is purely supportive.^[17]The precise mechanism of action for thalidomide is not known, although efforts to identify thalidomide's teratogenic action generated 2,000 research papers and the proposal of 15 or 16 plausible mechanisms by the year 2000.^[18] As of 2015, the main theories were inhibition of the process of angiogenesis, its inhibition of cereblon, a ubiquitin ligase, and its ability to generate reactive oxygen species which in turn kills cells.^[19][20] In 2018, results were first published which suggested that thalidomide's teratogenic effects are mediated through degradation of the transcription factor, SALL4, an as yet unverified finding.^[21]Thalidomide also binds to and acts as an antagonist of the androgen receptor (AR) and hence is a nonsteroidal antiandrogen (NSAA) of some capacity.^[22] In accordance, it can produce gynecomastia and sexual dysfunction as side effects in men.^[23]Thalidomide is provided as a racemic mixture of two enantiomers; while there are reports that only one of the enantiomers may cause birth defects, the body converts each enantiomer into the other through mechanisms that are not well understood.^[16]Thalidomide is racemic; while R-thalidomide is the bioactive form of the molecule, the individual enantiomers can racemize to each other due to the acidic hydrogen at the chiral centre, which is the carbon of the glutarimide ring bonded to the phthalimide substituent. The racemization process can occur in vivo.^{[2][24][25][26]}Celgene Corporation originally synthesized thalidomide using a three-step sequence starting with L-glutamic acid treatment, but this has since been reformed by the use of L-glutamine.^[27] As shown in the image below, N-carbethoxyphthalimide (1) can react with L-glutamine to yield N-phthaloyl-L-glutamine (2). Cyclization of N-phthaloyl-L-glutamine occurs using carbonyldiimidazole, which then yields thalidomide (3).^[27] Celgene Corporation's original method resulted in a 31% yield of S-thalidomide, whereas the two-step synthesis yields 85–93% product that is 99% pure.In 1952, thalidomide was synthesised by Chemical Industry Basel (CIBA), but was found "to have no effect on animals and was discarded" on that basis.^[28] In 1957, it was acquired by Chemie-Grunenthal in Germany.^[28] The German company had been established as a soap maker after World War II ended, to address the urgent market need for antibiotics.^{[citation needed]} Heinrich Mueckter^[29] was appointed to head the discovery program based on his experience working with the German army's antiviral research. While preparing reagents for the work, Mueckter's assistant Wilhelm Kunz isolated a by-product that was recognized by pharmacologist Herbert Keller as an analog of glutethimide, a sedative. The medicinal chemistry work turned to improving the lead compound into a suitable drug: the result was thalidomide. The toxicity was examined in several animals, and the drug was introduced in 1956 as a sedative, but it was never tested on pregnant women.^[30]Researchers at Chemie Grünenthal found that thalidomide was a particularly effective antiemetic that had an inhibitory effect on morning sickness.^[31] On October 1, 1957, the company launched thalidomide and began marketing it under the trade name Contergan.^[32][33] It was proclaimed a "wonder drug" for insomnia, coughs, colds and headaches.^[34]During that period, the use of medications during pregnancy was not strictly controlled, and drugs were not thoroughly tested for potential harm to the fetus.^[31] Thousands of pregnant women took the drug to relieve their symptoms. At the time of the drug's development, scientists did not believe any drug taken by a pregnant woman could pass across the placental barrier and harm the developing fetus.^[35] There soon appeared reports of abnormalities in children being born to mothers using thalidomide. In late 1959, it was noticed that peripheral neuritis developed in patients who took the drug over a period of time, and it was only after this point that thalidomide ceased to be provided over the counter.^[36]While initially considered safe, the drug was responsible for teratogenic deformities in children born after their mothers used it during pregnancies, prior to the third trimester. In November 1961, thalidomide was taken off the market due to massive pressure from the press and public.^[37] Experts estimate that were thalidomide led to the death of approximately 2,000 children and serious birth defects in more than 10,000 children, about 5,000 of them in West Germany.^{[citation needed]} The regulatory authorities in East Germany did not approve thalidomide.^[38] One reason for the initially unobserved side effects of the drug and the subsequent approval in West Germany was that at that time drugs did not have to be tested for teratogenic effects. They were tested on rodents only, as was usual at the time.^[39]In the UK, the British pharmaceutical company The Distillers Company (Biochemicals) Ltd, a subsidiary of Distillers Co. Ltd (now part of Diageo plc), marketed thalidomide throughout the UK, Australia and New Zealand, under the brand name Distaval, as a remedy for morning sickness. Their advertisement claimed that "Distaval can be given with complete safety to pregnant women and nursing mothers without adverse effect on mother or child ... Outstandingly safe Distaval has been prescribed for nearly three years in this country."^[38] Globally, more pharmaceutical companies started to produce and market the drug under license from Chemie Grünenthal. By the mid-1950s, 14 pharmaceutical companies were marketing thalidomide in 46 countries under at least 37 different trade names.In the US, representatives from Chemie Grünenthal approached Smith, Kline & French (SKF), now GlaxoSmithKline (GSK), with a request to market and distribute the drug in North America. A memorandum, rediscovered in 2010 in the archives of the FDA, shows that in 1956–57, as part of its in-licensing approach, Smith, Kline and French conducted animal tests and ran a clinical trial of the drug in the US involving 875 people, including pregnant women.^{[citation needed]} In 1956, researchers involved in clinical trials at SKF noted that, even when used in very high doses, thalidomide could not induce sleep in mice.^{[citation needed]} And when administered at doses 50 to 650 times larger than that claimed by Chemie Grünenthal to be "sleep inducing", the researchers could still not achieve the hypnotic effect in animals that it had on humans.^{[citation needed]} After completion of the trial, and based on reasons kept hidden for decades, SKF declined to commercialize the drug. In 1958, Chemie Grünenthal reached an agreement with William S Merrell Company in Cincinnati, Ohio (later Richardson-Merrell, now part of Sanofi), to market and distribute thalidomide throughout the US.^[38]The US FDA refused to approve thalidomide for marketing and distribution. However, the drug was distributed in large quantities for testing purposes, after the American distributor and manufacturer Richardson-Merrell had applied for its approval in September 1960.^{[citation needed]} The official in charge of the FDA review, Frances Oldham Kelsey, did not rely on information from the company, which did not include any test results. Richardson-Merrell was called on to perform tests and report the results. The company demanded approval six times, and was refused each time. Nevertheless, a total of 17 children with thalidomide-induced malformations were born in the US. Oldham Kelsey was given a Presidential award for distinguished service from the federal government for not allowing thalidomide to be approved for sale in the US.^[40]In Canada, the history of thalidomide dates back to April 1, 1961. There were many different forms sold, with the most common variant being called Talimol.^[41] Two months after Talimol went on sale, pharmaceutical companies sent physicians letters warning about the risk of birth defects.^[41] It was not until March 2, 1962, that both drugs were banned from the Canadian market by the FDD and, soon afterward, physicians were warned to destroy their supplies.^[41]In 1964, Israeli physician Jacob Sheskin administered thalidomide to a patient critically ill with leprosy. The patient exhibited erythema nodosum leprosum (ENL), a painful skin condition, one of the complications of leprosy. The treatment was attempted despite the ban on thalidomide's use, and results were favourable: the patient slept for hours and was able to get out of bed without aid upon awakening. A clinical trial studying the use of thalidomide in leprosy soon followed.^[42]Thalidomide has been used by Brazilian physicians as the drug of choice for the treatment of severe ENL since 1965, and by 1996, at least 33 cases of thalidomide embryopathy were recorded in people born in Brazil after 1965.^[43] Since 1994, the production, dispensing, and prescription of thalidomide have been strictly controlled, requiring women to use two forms of birth control and submit to regular pregnancy tests. Despite this, cases of thalidomide embryopathy continue,^[44][45] with at least 100 cases identified in Brazil between 2005 and 2010.^[46] 5.8 million thalidomide pills were distributed throughout Brazil in this time period, largely to poor Brazilians in areas with poor access to healthcare, and these cases have occurred despite the controls.In 1998, the FDA approved the drug's use in the treatment of ENL.^[47] Because of thalidomide's potential for causing birth defects, the drug may be distributed only under tightly controlled conditions. The FDA required that Celgene Corporation, which planned to market thalidomide under the brand name Thalomid, establish a system for thalidomide education and prescribing safety (STEPS) oversight program. The conditions required under the program include limiting prescription and dispensing rights to authorized prescribers and pharmacies only, keeping a registry of all patients prescribed thalidomide, providing extensive patient education about the risks associated with the drug, and providing periodic pregnancy tests for women who take the drug.^[47]In 2010, the World Health Organization (WHO) stated that it did not recommend thalidomide for leprosy due to the difficulty of adequately controlling its use, and due to the availability of clofazimine.^[48]Shortly after the teratogenic properties of thalidomide were recognized in the mid-1960s, its anti-cancer potential was explored and two clinical trials were conducted in people with advanced cancer, including some people with multiple myeloma; the trials were inconclusive.^[49]Little further work was done with thalidomide in cancer until the 1990s.^[49]Judah Folkman pioneered studies into the role of angiogenesis (the proliferation and growth of blood vessels) in the development of cancer, and in the early 1970s had shown that solid tumors could not expand without it.^[50][51] In 1993 he surprised the scientific world by hypothesizing the same was true of blood cancers,^[52] and the next year he published work showing that a biomarker of angiogenesis was higher in all people with cancer, but especially high in people with blood cancers, and other evidence emerged as well.^[53] Meanwhile, a member of his lab, Robert D'Amato, who was looking for angiogenesis inhibitors, discovered in 1994 that thalidomide inhibited angiogenesis^[54] and was effective in suppressing tumor growth in rabbits.^[55] Around that time, the wife of a man who was dying of multiple myeloma and whom standard treatments had failed, called Folkman asking him about his anti-angiogenesis ideas.^[51] Folkman persuaded the patient's doctor to try thalidomide, and that doctor conducted a clinical trial of thalidomide for people with multiple myeloma in which about a third of the subjects responded to the treatment.^[51] The results of that trial were published in the New England Journal of Medicine in 1999.^[51][56]After further work was done by Celgene and others, in 2006 the U.S. Food and Drug Administration granted accelerated approval for thalidomide in combination with dexamethasone for the treatment of newly diagnosed multiple myeloma patients.^[51][57]It was also evaluated whether thalidomide can be combined with melphalan and prednisone for patients with multiple myeloma. This combination of drugs probably results in an increase of the overall survival.^[58]Baby born to a mother who had taken thalidomide while pregnantIn the late 1950s and early 1960s, more than 10,000 children in 46 countries were born with deformities, such as phocomelia, as a consequence of thalidomide use.^[59] The severity and location of the deformities depended on how many days into the pregnancy the mother was before beginning treatment; thalidomide taken on the 20th day of pregnancy caused central brain damage, day 21 would damage the eyes, day 22 the ears and face, day 24 the arms, and leg damage would occur if taken up to day 28. Thalidomide did not damage the fetus if taken after 42 days gestation.^[37]It is not known exactly how many worldwide victims of the drug there have been, although estimates range from 10,000 to 20,000.^[60] Despite the side effects, thalidomide was sold in pharmacies in Canada until 1962.^[41][61]The disaster prompted many countries to introduce tougher rules for the testing and licensing of drugs, such as the Kefauver Harris Amendment^[68] (U.S.), Directive 65/65/EEC1 (E.U.),^[69] and the Medicines Act 1968 (UK).^[70][71] In the United States, the new regulations strengthened the FDA, among other ways, by requiring applicants to prove efficacy and to disclose all side effects encountered in testing.^[59] The FDA subsequently initiated the Drug Efficacy Study Implementation to reclassify drugs already on the market.In the 1960s, thalidomide was successfully marketed as a safer alternative to barbiturates. Due to a successful marketing campaign, thalidomide was widely used by pregnant women during the first trimester of pregnancy. However, thalidomide is a teratogenic substance, and a proportion of children born during the 1960s were afflicted with a syndrome known as thalidomide embryopathy (TE).^[72] Of these babies born with TE, "about 40% of them died before their first birthday".^[73] The surviving individuals are now middle-aged and they report experiencing challenges (physical, psychological, and socioeconomic) related to TE, Individuals born with TE frequently experience a wide variety of health problems secondary to their TE. These health conditions include both physical and psychological conditions. When compared to individuals of similar demographic profiles, those born with TE report less satisfaction with their quality of life and their overall health.^[72] Access to health care services can also be a challenge for these people, and women in particular have experienced difficulty in locating healthcare professionals who can understand and embrace their needs.^[73]Main article: Development of analogs of thalidomideThe exploration of the antiangiogenic and immunomodulatory activities of thalidomide has led to the study and creation of thalidomide analogs.^[74][75] Celgene has sponsored numerous clinical trials with analogues to thalidomide, such as lenalidomide, that are substantially more powerful and have fewer side effects — except for greater myelosuppression.^[76] In 2005, Celgene received FDA approval for lenalidomide (Revlimid) as the first commercially useful derivative. Revlimid is available only in a restricted distribution setting to avoid its use during pregnancy. Further studies are being conducted to find safer compounds with useful qualities. Another more potent analog, pomalidomide, is now FDA approved.^[77] Additionally, apremilast was approved by the FDA in March 2014. These thalidomide analogs can be used to treat different diseases, or used in a regimen to fight two conditions.^[78]Interest turned to pomalidomide, a derivative of thalidomide marketed by Celgene. It is a very active anti-angiogenic agent ^[75] and also acts as an immunomodulator. Pomalidomide was approved in February 2013 by the U.S. Food and Drug Administration (FDA) as a treatment for relapsed and refractory multiple myeloma.^[79] It received a similar approval from the European Commission in August 2013, and is expected to be marketed in Europe under the brand name Imnovid.^[80]There is no conclusive evidence that thalidomide or lenalidomide is useful to bring about or maintain remission in Crohn's disease.^[81][82]Thalidomide was studied in a Phase II trial for Kaposi's sarcoma, a rare soft-tissue cancer most commonly seen in the immunocompromised, that is caused by the Kaposi's sarcoma-associated herpesvirus (KSHV).^[83][31]