Pace + Localization = Syndrome
Syndrome + Context = Differential Diagnosis
Pace is the onset and evolution of patient's symptoms.
When did the symptoms start? How long did the symptoms last? How have the symptoms changed since onset? Has there ever been a time when they completely resolved and the patient was back to his usual self of before they had started?
A patient who presents with a fixed deficit from a stroke suffered years prior, but the pace would still be defined as quite rapid (hyperacute) if his initial stroke symptoms developed over seconds to minutes
Hyperacute (seconds to minutes). Patients or their families are able to recall exactly when and where their symptoms started. There is almost a clear demarcation between the patient's baseline and when their symptoms started.
Acute (minutes to hours to days). The demarcation between the patient's baseline normal and when symptoms started is still clear but becomes slightly blurred.
Subacute (days to weeks to few months). The demarcation between the patient's baseline normal and when the symptoms started becomes quite blurry; uncertainty becomes obvious in the history. Be careful, not to ask too many leading questions as the patient or the informant can become suggestible. However, look if there is any specific event when their symptoms first became noticeable. Questions that attempt to draw a frame around the timeline can be helpful: "Were your symptoms present 2 months ago when you took a trip with your family?" or "When was the last time your mother was able to handle her bills on her own?"
Chronic (months to years). The boundary between the patient's baseline and the onset of symptoms might not exist. There is no official start time, but typically, a patient or relative can tell you a time in the patient's life when the symptoms were not present.
Pace is a continuous spectrum and the categories (hyperacute - chronic) blend into one another.
Sometimes the pace of the underlying pathologic diagnosis is incongruent with the pace of the onset of symptoms. Take, for example, a benign tumor growing over a chronic time period. A patient may not develop any symptoms until the tumor causes a seizure. This would be an example of a hyperacute pace of presentation for a chronic pathology. Alternatively, a particular pathologic diagnosis might have different paces at different times. The most notorious would be the variable symptoms related to venous disease, such as venous thromboses or vascular malformations. Disease that can cause disruption of CSF flow, abnormal intracranial pressure, or other compression may have a varied pace of onset. There is no one-to-one correlation between syndromes and pathologic diagnoses.
For relapsing-remitting multiple sclerosis (RRMS), define pace for each distinct episode ("attack") which are generally hyperacute, as well as the pace of overall course of disease.
A patient with no prior history of epilepsy might present with a first-time seizure. Within 2 weeks she is now having multiple seizures per day. Each discrete episode of seizure has a hyperacute onset, but the overall trajectory of her illness seem to be subacute in that her symptoms evolved over the span of several weeks. In the example, even though each seizure has a hyperacute pace, we would suspect the underlying etiology of her seizure to be subacute process such as an inflammatory or infectious based on the evolution of her symptoms over several weeks.
Hyperacute: Vascular (Ischemic or H'gic stroke); seizure (GTC sz); headache (migraine HA, cluster HA); syncope (dysautonomia, cardiac arrhythmia); trauma/structural (fall/MVA); toxic/metabolic (hypoglycemia, opioid overdose)
Acute: Infectious (bacterial meningitis); inflammatory (optic neuritis); toxic/metabolic (uremia); structural (epidural hematoma).
Subacute: Infectious (tuberculous meningitis); inflammatory (autoimmune encephalitis); neoplastic (glioblastoma); toxic/metabolic (vitamin deficiencies); structural (subdural hematoma).
Chronic: Genetic (Huntington's disease); neurodegenerative (ALS - amyotrophic lateral sclerosis); neoplastic (meningioma); toxic/metabolic (lead poisoning); structural (cervical stenosis).
CRANIAL NERVES: Visual acuity- OU: 20/20 w/o correction. Visual fields full to confrontation without extinction to double simultaneous stimulation. Fundus yellow with sharp borders, no AV nicking, exudates, cotton wool spot, dot and blot H'ges, vitreous H'ge, Terson's sign (SAH), PERRL 4 => 2 mm bilaterally. Direct and consensual response noted. Eye movements with normal versions, smooth pursuit and saccades. No nystagmus. Facial sensation intact to light touch in all three trigeminal areas bilaterally. Brisk corneal reflex bilaterally. Good strength of masseter and temporalis muscles on jaw clenching. Facial strength normal. No obvious facial asymmetry noted. Hearing intact to finger rub. Palate and uvula elevates in midline symmetrically, no pooled salivary secretions. Able to cough. Head rotation from side to side, and shoulder shrug with good strength bilaterally. Tongue midline and patient able to move it from side to side, no fasciculations noted.
MOTOR EXAM: Body position at rest and movement. Gait is normal with symmetric stride and arm swing. Tandem walking (toe to heel), toe walking, heel walking, monopedal stance intact. Rising from a sitting position without arm support. Romberg’s sign and pronator drift negative.
Bulk and tone are normal without obvious atrophy, and are symmetric. No tremors, tics, fasciculation, or myoclonus noted. Non tender. No asterexis.
Strength: Right upper extremity and right lower extremity proximal and distal 5/5 strength.
"The neurological examination of patients remains first and foremost a bedside exercise."
"Patient assessment strategies are most effective when based on clinical hypotheses. These hypotheses should be formulated based on the principles of neurological localization, the chronological course of symptom development, and application of risk factor analysis. Tests and other investigations ordered rationally with the primary purpose of resolving a clinically established differential diagnosis or, if possible to prove the working diagnosis."
GENERAL PHYSICAL EXAMINATION
Vital Signs:
BP in both arms: significant asymmetry suggests extracranial cardiovascular occlusive disease. Increased BP with decreased HR occurs with raised ICP (Cushing reflex)
Orthostatic VS: supine, seated, and upright. Autonomic insufficiency due to peripheral causes such as diabetic neuropathy, or due to failure of central regulation, as in multisystem atrophy (MSA). Most frequent cause of orthostasis is a side-effect of anti-HTN therapy.
Bounding pulse: AR, hypothyroidism.
Small, slow pulse: AS
Irregular irregular pulse: AF, bradyarrhythmias, tachyarrhythmias
General Appearance: Pt. is a WD/WN, ethnicity, appears stated age, laying calmly on stretcher, wearing hospital gown. Grooming and personal hygiene, cooperation with exam, flat expression of face, poor eye contact. Abnormal posture of trunk, head, or extremities.
Does patient look ill, in pain, or distress. Debilitated, emaciated, obese.
Facies/facial expression:
Dysmorpthic features, gigantism, dwarfism, abnormal facies: masked facies/reptilian stare in parksinonism, diplegic face (MG, GBS), coarse facies (myxedema, acromegaly, mucopolysachridosis phenytoin use), cushingoid features, PSP (look of perpetual surprise), immobile face with precipitate laughter and crying seen in pseudobulbar palsy, MD, MG
Skin: pallor, cyanosis, erythema, rash, nail changes (dystrophic), clubbing of fingernails. Hair growth
Eyes: periorbital structures, conjunctivitis, ptosis, exophthalmos, lid lag, lid retraction, xanthelasma, jaundice, unilateral proptosis (thyroid eye disease, retro-orbital tumor, carotid-cavernous fistula, histiocytosis X), meningocele, encephalocele; corneal clouding (mucopolysacchridosis), Brushfield spots on iris due to Down's syndrome, Lisch nodules in neurofibromatosis; keratoconjunctivitis sicca due to Sjogrne's syndrome, HZ ophthalmicus, pnegueculae (Gaucher's disease), KF-ring (Wilson's disease), unilateral arcus senilis (carotid stenosis), tortuous conjunctival vessels (AT), scleritis (Wegener's granulomatosis), nonsyphilitic interstitial keratitis (Cogan's syndrome).
Inspect the width of palpebral fissures, inter-orbital distance (hypertelorism vs hypotelorism), epicanthic folds, Test pupillary light-reflexes, cover-uncover test, cross-cover test. convergence
HENT: Shape, symmetry, size and any evidence of deformities of head. The -cephalies (macro, micro, hydro, brachy, etc.). Intraoral lesions, leukoplakia. Perforated TM, glomus tumor (Jugular foramen synd), HZ infection (Ramsay-Hunt synd), cholesteatoma. CSF otorrhea, hemotypanum (basilar skull fracture), CSOM/Serous otitis media (air-fluid); perforated nasal septum (cocaine snorter), saddle nose (congenital syphilis), CSF rhinorrhea. Smooth tongue with atrophy of fungiform and filiform papillae (pernicious anemia).
Neck: bruit
Cardiovascular: Hear rate, rhythm, pulses
Musculoskeletal: swelling, deformity, varus, valgus, genu recurvatum, pretibial edema, leg length discrepancy. Pes cavus, hammertoes, hand arthropathy (swan-neck, Heberden's, Bouchard nodes).
Neurologic:
MENTAL STATUS:
Alertness: Patient is alert/lethargic, requires tactile stimulation to arouse; falls asleep when not stimulated/stupor/coma
Orientation: Pt. is oriented to person, place, and time. GCS EVM.
Speech and Language functions: Speech is fluent and prosodic without paraphasic errors, comprehension (close your eyes, open them. Touch your right ear with your thumb and stick your tongue out. Point to your knee, point to the floor), naming (pen, watch, coin) (parts of ball point pen or parts of watch), repetition (no ifs, ands, or buts; I am in the hospital), reading, and writing is intact.
Attention: WORLD/DLROW, naming months/days of the week forwards and backwards. Number 1-5, forwards and backwards
Memory: Registration of 3/3 objects. Short-term memory: Pt. is able to recall 3/3 objects accurately after 5 minutes. Long term memory: able to state his date of birth, home phone number, address, name of spouse, children, work, name past 5 presidents. Anything that is verifiable.
Mood/Affect:
Thought processes: circumstantiality, derailment, flight of ideas, neologisms, incoherence, blocking, confabulation, perseveration, echolalia, clanging, disscursive
Thought content: compulsions, obsessions, phobias, anxieties, feeling of unreality, feelings of depersonalization, delusions
Perceptions: illusions, hallucinations. Suicidal or homicidal ideation/intents.
Higher cognitive functions: Fund of information and vocabulary: hobbies, work, favorite TV programs, name of last 4 presidents, 5 large cities.
Calculations–serial 7’s, 3’s, or simple calculations.
Logic, Abstract reasoning/concrete reasoning: proverb interpretation
Judgment (scenario: fire in moving theater, letter on side walk, etc)
Insight (how the Pt. perceives his/her condition)
Neglect, Right and left confusion, finger agnosia, and construction tasks (intersecting pentagons, bisecting lines). Visuospatial tasks: right/left (draw clock face). Repeat with opposite side
Praxis:
Vestibular test not tested unless specifically indicated in the following important situations:
Vertigo. Perform maneuvers to distinguish central from peripheral lesions.
Limitations of horizontal or vertical gaze. Testing the vestibulo-ocular reflex can help localize the lesion. Perform oculocephalic maneuver or caloric testing
Patients in coma
What is being tested?
Hearing loss can be caused by lesions in the acoustic and mechanical elements of the ear, the neural elements of the cochlea, or the acoustic nerve (CN VIII). After the hearing pathways enter the brainstem, they cross over at multiple levels and ascend bilaterally to the thalamus and auditory cortex. Therefore, clinically significant unilateral hearing loss is invariably caused by peripheral neural or mechanical lesions.
Abnormalities in vestibular testing can be associated with lesions in the vestibular apparatus of the inner ear, vestibular portion of the CN VIII, and the vestibular nuclei of the brainstem, cerebellum, or pathways in the brainstem (such as medial longitudinal fasiculus) that connect the vestibular and oculomotor systems.
Palate elevation and Gag Reflex (CN IX, X).
Does the palate elevate symmetrically when the patient says, "Aah"
Gag reflex present when posterior pharynx is brushed. Gag reflex need to be tested only in patients with suspected brainstem pathology, impaired consciousness, or impaired swallowing.
What is being tested?
Palate elevation and the gag reflex are impaired in lesions involving CN IX, X, the neuromuscular junction, or the pharyngeal muscles.
Dysphagia and the risk of aspiration are best screened at the bedside by asking the patient to swallow a small quantity of water (3 oz); coughing indicates aspiration and inability to protect the airway.
Muscles of articulation (CN V, VII, IX, X, XII).
Is the patient's speech hoarse, slurred, quiet, breathy, nasal, low or high pitched, and so on? It is often important to ask if the patient's speech has changed from baseline. Note that dysarthria or abnormal pronunciation of speech, is not the same as aphasia, which is an abnormality in language production or comprehension.
What is Being Tested?
Abnormal articulation of speech can occur in lesions involving the muscles of articulation, the neuromuscular junction, or the peripheral or central portions of CN V, VII, IX, X, or XII.
Furthermore, speech production can be abnormal as a result of lesions in the motor cortex, cerebellum, basal ganglia, or descending pathways to the brainstem.
Sternocleidomastoid and trapezius muscles (CN XI)
Ask the patient to shrug their shoulders, turn their head in both directions, and raise their head from the bed, flexing forward against the force of your hands.
Contraction of the left sternocleidomastoid muscle turns the head to the right and vice versa.
What is Being Tested?
Weakness in the sternocleidomastoid or trapezius muscles can be caused by lesions in the muscles, neuromuscular junction, or lower motor neurons of the accessory spinal nerve (CN XI).
Unilateral upper motor neuron lesions in the cortex or descending pathways cause contralateral weakness of the trapezius, with relative sparing of sternocleidomastoid strength. This may be explained by bilateral upper motor neuron projections controlling the sternocleidomastoid, similar to the bilateral projections controlling the upper face.
Tongue Muscles (CN XII)
Note any atrophy or fasciculations (spontaneous quivering movements caused by firing of muscle motor units) of the tongue while it is resting on the floor of the mouth. Ask the patient to stick their tongue straight out and note whether it curves to one side or the other. Ask the patient to move their tongue from side to side and push it forcefully against the inside of each cheek.
Fasciculations and atrophy are signs of lower motor neuron lesions. Unilateral tongue weakness causes the tongue to deviate toward the weak side. Tongue weakness can result from lesions of the tongue muscles, the neuromuscular junction, the lower motor neurons of the hypoglossal nerve (CN XII), or the upper motor neurons originating in the motor cortex. Lesions of the motor cortex cause contralateral tongue weakness.
CNs proceed caudally in the brain stem as numbered.
Presence of corneal reflex (CN 5 + 7) denotes intact pons.
Intact gag reflex (CN 9 + 10) shows functioning upper medulla.
Motor system: focus on body position, involuntary movements, characteristics of the muscles (bulk, tone, and strength), and coordination.
Tremor should be evaluated at rest (rest tremor), with sustained posture (postural tremor), and with active movement (action or intention tremor).
Abnormal positions indicate neurologic deficits as paralysis, or guarding due to pain - antalgic.
Any obvious abnormality in the muscle(s) involved, needs detailed exam of that area. Think whether the abnormality is central or peripheral and learn which nerves innervate the affected muscles.
Involuntary movements: note their location, quality, rate, rhythm, and amplitude, and their relation to posture, activity, fatigue, and emotion.
Muscle bulk: atrophy - if unilateral or bilateral. Age appropriate, in thenar, hypothenar, dorsal interossei of hands, shoulders and thighs.
Atrophy: Diabetic neuropathy, motor neuron disease, myopathies, disuse atrophy as in rheumatoid arthritis, and protein-calorie malnutrition. Localized atrophy of thenar and hyothenar eminences suggest damage to median and ulnar nerves respectively.
Hypertrophy: increase in bulk and proportionate strength
Pseudohypertrophy: increase in bulk with diminished strength (Duchenne form of muscular dystrophy)
Fasciculations in atrophic muscles suggest peripheral nerve damage. If you see none, tap the muscle with a reflex hammer.
Muscle tone.
Decreased resistance or hypotonia suggest disease of the ipsilateral peripheral nervous system, ipsilateral cerebellar disease, or the acute stage of spinal cord injury, LMN.
Hypertonia
Spasticity: UMN. Sudden increased in tone or a "catch" as the limb is passively flexed or extended. Increased resistance
Clasp knife phenomenon: muscle tone is greatest at the beginning of movement and slowly decreases until there is a sudden loss of resistance. Seen in stroke patients.
Rigidity: Resistance present throughout the range in both directions is called lead-pipe rigidity. Increased rigidity present throughout the range of motion in lesions of the basal ganglia. Cog-wheel rigidity, characterized by a regular ratchet-like loss of resistance, is especially characteristic of Parkinsonism. Movement in a circular direction at the wrist is a very sensitive maneuver for detecting cog-wheel rigidity. Cog-wheel rigidity at wrist can be accentuated if the patient is asked to repeatedly open and close the opposite hand.
Paratonic rigidity or Gegenhalten (holding against), or paratonia, occurs with dementia and frontal lobe syndromes. This is an abnormal variable resistance to limb muscles from contralateral to a mesial frontal lobe lesion, where on testing passive range of movements there seems to be an inability to relax with an inclination to reassess, increasing with more rapid movement; it affects the whole limb, although may be bilateral and bifrontal disease (example with stroke or dementia).
Muscle strength. Know which side of patient is dominant, when comparing muscle strength. A person's dominant side is usually slightly stronger than the other side.
Paresis is impaired strength or weakness
Plegia is absence of strength or paralysis
Hemiparesis refers to weakness of one half of the body
Hemiplegia refers to paralysis of the one half of the body
Paraplegia refers to paralysis of the the legs
Quadriplegia (tetraplegia) refers to paralysis of all four limbs.
Isolate the muscle being tested, to prevent contamination by other muscle groups.
It is important to passively extend the wrist to properly test for finger abduction, and adduction,
Weakness in wrist extension is seen in peripheral nerve disease (radial nerve damage) and in central disease producing hemiplegia (stroke, or MS).
Weakness in grip strength may be due to either central or peripheral nervous system disease. It may also be due to painful disorders of the hands.
Weakness of finger abduction in ulnar nerve d/o.
Weakness of thumb opposition in median nerve d/o, such as CTS.
Scale 0/5 - 5/5 - British Medical Research Council (MRC) scheme
0/5: no contraction
1/5: muscle flicker, but no movement at a joint.
2/5: movement, but not against gravity. Tested in horizontal plane of joint.
3/5: movement against gravity, but not against resistance.
4/5: movement against gravity, and against some resistance
4-/5, 4/5, and 4+/5
5/5: normal strength.
Hand grip does not fit in this scheme. Describe hand grip subjectively: strong, weak.
Coordination. tests four areas of the nervous system function in an integrated way:
Motor system, for muscle strength
Cerebellar system, for rhythmic movement and steady posture
Vestibular system, for balance and for coordinating eye, head, and body movements
Sensory system, for position sense.
Cerebellar disease. One movement cannot be followed rapidly by its opposite and movements are clumsy, slow, irregular - dysdiadochokinesis. UMN and extrapyramidal disease may also impair rapid alternating movement, but not in the same manner.
Dysmetria is when movements are wavering, unsteady, clumsy and inappropriately varying in speed, force, and direction. The finger may initially overshoot its mark, but finally reaches it fairly well. An intention tremor may appear toward the end of the movement.
Coordination may be lost with eyes closed. This suggests loss of position sense, sensory system.
Repetitive and consistent deviation to one side (past pointing), worse with eyes closed, suggests cerebellar or vestibular disease.
The heel to shin test - heel may overshoot the knee and then oscillate from side to side down the shin with eyes open. When position sense is lost, the heel is lifted too high and the patient tries to open eyes look. Limb ataxia results in a side-to-side "tremor" as the test is performed.
Stance: Ask the patient to stand still with feet together and the eyes open. If the patient can stand steadily in this position, the examiner then asks the patients to close eyes. If the patient becomes unsteady with eyes closed, this is called a positive Romberg test. The usual cause of this is cerebellar ataxia affecting the trunk. Postural reflexes - retropulsion test for Parkinsonism
Gait: ataxia - cerebellar disease, loss of position sense, or intoxication
Tandem walking may reveal an ataxia not previously known.
Walking on toes and heels may reveal distal muscle weakness in the legs. Inability to heel walk is a sensitive test for UMN weakness.
Difficulty to do a monopedal stance may be due to weakness, lack of position sense, or cerebellar dysfunction.
Inability or difficulty in rising from a seated position without arm support suggests proximal muscle weakness involving the pelvic girdle and legs.
Rebound or lack of check
Sensory:
Evaluate pain, temperature, touch and position. Pain: pinch trapezius or supraorbital pressure. If no pain response, check centrally (across face) as problem could be related to transmission of the cord. Face is innervated by cranial nerves and not the spinal nerves.
"Glove and stocking" sensory loss - alcoholism and diabetic polyneuropathies.
Vibration sense (tuning fork 128 Hz or 256 Hz). First sensation to be lost in peripheral neuropathy (DM, alcoholism). Also diminished in posterior column disease (tertiary syphilis or vitamin B12 deficiency)
VBS testing was more sensitive and JPS testing was more specific in making a clinical diagnosis of DSP. For timed VBS, using 128 Hz tuning fork a duration of >8 s at the great toe was a useful test to rule out DSP, and for JPS testing at the great toe (at least 20 degree up or down excursions), obtaining two or more incorrect responses was a useful test in ruling in the diagnosis of DSP. (Ref)
Discriminative sensations - 2 point are a function of the sensory cortex to correlate, analyze, and interpret sensations.
Stereognosis, graphesthesia, and two point discrimination are also impaired by posterior column disease.
DTR (correct name: muscle stretch reflexes)
0: absent reflex
1: trace seen with reinforcement
2: normal
3: brisk
4: non-sustained clonus
5: sustained clonus
Reflexes: tested in SCI; compartment syndrome; patient's on magnesium drip; encephalopathic (liver disease) or to identify pathologic relfexes.
Hyporeflexia: associated with hypermagnesemia, hypercalcemia; hyponatremia; hypokalemia, and hypothyroidism
Hyperreflexia: associated with hypmagnesemia; hypocalcemia; hypernatremia, hepatic encephalopathy.
Babinski reflex: rapidly stroke the sole of the foot from heel to ball of the foot. Positive when the great toe goes up and other toes fan: pyramidal tract problem.
Grasp reflex: place 2 fingers in palm of hand (patient grasps): problem in frontal lobe.
Hoffman's sign: flick nail down on middle finger, watch for thumb adduction.
Cutaneous reflexes. Useful when SCI or a cauda equina lesion is suspected. Presence of these reflexes implies spinal cord is intact, and corresponding sensory and motor nerves at the level tested is intact.
Abdominal reflexes. Stroke lightly from lateral to medial section of the abdomen T8-T9 and below (T11-T12) the umbilicus. Normal response is local contraction of the ipsilateral rectus abdominis muscle.
Cremasteric reflexes. Striking the medial thigh (L1-L2) results in ipsilateral retraction of the scrotum (S1).
Bulbocavernous reflex and anal wink. Squeezing the head of the penis (S2-S3) or stroking the perianal skin (S3-S4) results in reflex contraction of the external anal sphincter (S3-S4).
Frontal release signs. snout, suck, root reflexes. Palmomental reflex (unilateral reflex implies contralateral frontal lobe disease). Grasp reflex, glabellar reflex.
Quick way to check motor strength/paresis:
Pronator drift
Wave hands fast at wrist
Tapping each finger to thumb in sequence rapidly
Tapping foot rapidly to examiners palm
Flexion and extension
Widened palpebral fissure
Flattened nasolabial fold
Hoffman sign
Digiti quinti sign: ask the patient to extend both arms with palms down. The digit quinti on the side of paralysis (ipsilateral) abducts (higer sensitivity than pronator drift)
Finger rolling test
Upgoing thumb signs
Babinski's sign
Mental Status examination terminology
abulia: loss of initiative, willpower, or drive
acalculia: inability to calculate
agnosia: inability to recognize one or more classes of environmental stimuli, even though the necessary intellectual and perceptual functions are intact
agraphia: inability to write
alexia: inability to read for comprehension
amnesia: inability to retain new information
anomia: inability to name objects or think of words; in practice, often used as a synonym for dysnomia
anosognosia: inability to recognize one's own impairment
anterior aphasia: acquired language disorder in which verbal output is nonfluent
aphasia: literally, a complete loss of language function, but in practice, used as a synonym for dysphasia
aphemia: complete loss of the ability to speak, but retained comprehension and writing ability
apraxia: inability to perform a previously learned set of coordinated movements even though the necessary component skills (including intellect, language function, strength, coordination, and sensation) remain intact
Broca's aphasia: acquired language disorder characterized by nonfluent verbal output with omission of relational words (prepositions, conjunctions, articles, and minor modifiers) and abnormal prosody, impaired repetition, and relatively intact comprehension
conduction aphasia: acquired language disorder characterized by prominent impairment of repetition, relatively intact comprehension, and verbal output that is fluent but contains literal paraphasias
delirium: an acute confusional state characterized by clouded, reduced, or shifting attention, often associated with sensory misperception or disturbed thinking
dementia: acquired impairment of memory and at least one other cognitive function, without clouding of the sensorium or underlying psychiatric disease
dysnomia: difficulty naming objects or finding the desired words
dysphasia: acquired disorder of language not due to generalized intellectual impairment or psychiatric disturbance
expressive aphasia: acquired language disorder in which verbal output is nonfluent
fluent: an adjective used to describe verbal output that is normal to excessive, easily produced, with normal phrase length (five or more words) and normal prosody
fluent aphasia: acquired language disorder in which verbal output is fluent
Gerstmann's syndrome: the constellation of (1) agraphia, (2) acalculia, (3) right-left confusion, and (4) finger agnosia; classically associated with lesions in the angular gyrus of the dominant hemisphere (but the subject of endless debate)
jargon: verbal output that contains so many literal paraphasias that the words are unrecognizable
nonfluent: an adjective used to describe verbal output that is sparse, with only one to four words per phrase
nonfluent aphasia: acquired language disorder in which verbal output is nonfluent
paraphasia: a substitution error in which the word produced is similar in sound or meaning to the intended word. A literal or phonemic paraphasia is a sound substitution error resulting in production of a word that is phonemically related to the intended word (eg, "greed" or "greeb" instead of "green"). A semantic or verbal paraphasia is a word substitution error in which the word produced is semantically related to the intended word (eg, "blue" instead of "green")
posterior aphasia: acquired language disorder in which comprehension is impaired
prosody: the rhythm and tempo of speech
prosopagnosia: inability to recognize faces
receptive aphasia: acquired language disorder in which comprehension is impaired
transcortical aphasia: acquired language disorder in which the ability to repeat is intact
Wernicke's aphasia: acquired language disorder characterized by markedly impaired comprehension and repetition, with verbal output that is fluent but contaminated by numerous paraphasias or, in severe cases, jargon.
Mental Status exam: Tests the overall global brain function.
Memory tests - Limbic and global functions (memory).
Language function tests the dominant (usually left) hemispheric language functions;
Calculation, writing, construction, and visuospatial tasks are tests of left parietal dysfunction (Gerstmann's syndrome)
Right parietal dysfunction: left sided neglect and construction.
Frontal lobe dysfunction: Drawing a silhouette line of alternating triangles and squares; Luria sequencing tasks, auditory go-no go test, motor impersistence, and frontal release signs
Tests that are less localizing but provide important clues about brain dysfunction (apraxia, logic and abstraction, delusions, hallucinations, and mood).
If the level of consciousness and alertness are not optimal, mental status exam is limited. Such neurological exams, must be qualified with a statement, noting the level of consciousness and alertness of the patient.
Level of consciousness is severely impaired when in lesions of the brainstem reticular formation, and in bilateral lesions of the thalami, diencephalic structures, or cerebral hemispheres. It is mildly impaired in unilateral cortical or thalamic lesions. Toxic and metabolic factors are also some common causes of impaired consciousness because these affect the structures mentioned.
Attention and cooperation impairment is not specific and can occur in many different focal brain lesions, in diffuse abnormalities such as dementia, or encephalitis, and in behavior or mood disorders.
Test attention: WORLD/DLROW, digit span 6 forwards and 4 or more backwards (123456/6543). Naming days of the week or months forwards and backwards
Orientation: Document specifically the questions that were asked and how they were answered. What the patient did and was not able to do. Pt. is oriented to person: "John Doe," place: "Hospital," but does not know which one, time: "2011," and does not season, month, day, or today's date.
What's being tested? Tests recent and long-term memory.
Memory: Have the patient repeat after you, once, 3 objects, e.g. (ball, dog, table). Assess patient's ability to recall these objects after 5 minutes. During the interval time, it is important you you provide other distractions in order to prevent patient from rehearsing the items repeatedly. Also time using a digital watch alarm to prevent yourself from forgetting to test for these items at the end of 5 mintues. Remote memory must be verifiable: address, date of birth, spouse, name of children, phone number, jobs, school, military service, name of past presidents or any verifiable personal events.
Impaired ability to register and recall something within a few seconds after it was said is an abnormality that ties into the category of impaired attention.
If registration is intact, but recall after 5 minutes is difficult, it signifies damage to the limbic memory structures of the medial temporal lobes and medial diencephalon. Dysfunction of these structures characteristically give rise to anterograde amnesia, whereby the patient has difficulty remembering new facts and events occurring after lesion onset. Retrograde amnesia, means that the patient has impaired memory of events for a period of time immediately before lesion onset, with relative sparing of remote memories.
What's being tested? Loss of memory without these time characteristics may signify damage to areas other than the medial temporal and medial diencephalic structures, and can also occur in psychogenic amnesia.
Language:
Speech fluency, phrase length, rate and effort, tone, modulation, labial (ppp, bbb) lingual (lll, ttt), dental (tttt/th th th), glottal (kkk, ggg); paraphasias (phonemic - table/tadle; semantic - door/window), neologisms, word salad, word finding difficulty, losing grammar and syntax, perseverative responses.
F test: Number of words beginning with F in 60 sec (>12 if high school education)
Verbal fluency: This may be impaired in dysphagia or functional dysfunction; I asked the patient to see as many words as possible excluding names of people and places in 1 minute each with the letter F, 80, and S; consideration of names is abnormal (most people get 15 words at least per letter; <34 FAS is usually abnormal). "Tell me the names of as many animals as you can in 1 minute." Around 20 is normal; 15 is low average; 10 is impaired.
Comprehension. Follows simple questions, answers appropriately to queries during the interview. Follows simple commands (3-step commands). Understanding of grammatical structure should be tested as well; for e.g., "Mike was shot by John. Is John dead?"
Naming. Easy ones (pen, watch, tie) objects. Naming parts of objects is often more difficult (parts of ball point pen, parts of a wrist watch).
Repetition. "no ifs, ands, or buts." Titrate upwards to difficult ones "today is a beautiful day", and write down what the patient says.
Reading: Ask patient to read aloud single words, a brief passage and test for comprehension.
Writing. Ask patient to write their name and write a sentence.
What's being tested? Language abnormalities are caused by lesions in the dominant (usually left) frontal lobe, including Broca's area; the left temporal and parietal lobes, including Wernicke's area; subcortical white matter and gray matter structures, including thalamus and caudate nucleus; as well as the nondominant hemisphere.
Calculations, right-left confusion, finger agnosia, agraphia. Impairment of all four of these functions in an otherwise intact patient is referred to as Gerstmann's syndrome. This is due to lesions in the inferior parietal lobule in the left hemisphere. It produces an interesting constellation of abnormalities:
Right and left confusion: Unable to identify right and left body parts.
Finger agnosia: Unable to identify each digit of hand (thumb, index finger, middle finger, ring finger, pinkie).
Acalculia: Inability to do simple math (addition, substraction, multiplication).
Agraphia: Unable to write own name and a sentence. Construction task.
+/- aphasia
Right-left confusion and finger agnosia can be both quickly screened for by the command, "Touch your right ear with left thumb, and stick your tongue out."
Each of the individual components of Gerstmann's syndrome is poorly localizing on its own, but they are worth documenting as part of the assessment of overall cognitive function. Each of the individual abnormalities can be seen in many different lesions and may be present in individuals with impaired attention, language praxis, constructions, logic and abstractions.
Apraxia. Inability to follow a motor command that is not due to a primary motor deficit or a language impairment.
Deficit in high-order planning or conceptualization of the motor task. Test apraxia by using commands such as "Pretend to comb your hair" to test for ideomotor apraxia.
In some patients, rather than affecting the distal extremities, apraxia can involve primarily the mouth and face, or movements of the whole body, such as walking or turning around.
Localization is difficult in apraxia. It commonly caused by lesions affecting the language areas and adjacent structures of the dominant hemisphere. Hence, it becomes difficult to prove that the deficit is apraxia rather than impaired language comprehension. To make a distinction, the examiner can ask the patient to perform a task, and then if they fail, demonstrate several tasks and ask them to chose the correct one.
Neglect and Constructions. Hemineglect is an abnormality in attention to one side of the universe. It is not due to a primary sensory or motor disturbance.
In sensory neglect, patients ignore visual, somatosensory, or auditory stimuli on the affected side, despite intact primary sensation. This is demonstrable by testing for extinction on double simultaneous stimulation. Thus, patients may detect a stimulus on the affected side when presented alone, but when stimuli are presented simultaneously on both sides, only the stimulus on the unaffected side may be detected.
In motor neglect, normal strength may be present; however, the patient does not move the affected limb unless attention is strongly directed towards it.
Sensory and motor neglect is tested during visual, auditory, somatosensory, and motor exam.
During reading and writing portions of the language exam, patients may be noted to neglect one side of the page.
Anosognosia may be tested by asking "Is there anything wrong with you right now?" Patients who have anosognosia may be unaware of severe deficits on the affected side of their body.
Drawing tasks, such as asking the patient to bisect a line or draw a clock face, crossing many small lines, can demonstrate neglect if they fail in executing these tasks.
Construction tasks involving drawing complex figures or manipulating blocks of other objects in space may be abnormal as a result of neglect or other visuospatial impairments. However, constructional abilities can also be abnormal because of other cognitive difficulties, such as impaired sequencing.
What's being tested?
Hemineglect is most common in lesions of the right (nondominant) parietal lobe, causing the patients to neglect the left side.
Left side neglect can also be seen in right frontal lesions, right thalamic or basal ganglia lesions, and, rarely, in lesions of the right midbrain.
In left parietal lesions a much milder neglect is usually seen affecting the patient's right side.
Frontal release signs: Patients with frontal lobe dysfunction/lesions may have difficulty in changing from one action to another when asked to perform a repeated sequence of actions. "Executive functions" are impaired in frontal lobe lesion.
Ask patient to continually draw a repeated sequence of actions. When asked to draw a silhouette pattern of alternating triangles and squares, they get stuck on one shape and keep drawing triangles. This phenomenon is called perseveration. Luria manual sequencing task, in which the patient is asked to tap their thigh or table with a fist (ulnar border), then open palm and strike with ulnar border, and then strike with the palmar aspect repetitiously.
Response inhibition and set shifting. Motor sequencing-Luria three-step test. "First-edge-palm" 3 times without verbal cues and asked the patient to repeat the sequence 3 times with you and then alone. Patient with functional deficits often failed even a few steps sequence of first time without prompts.
The alternate hand movement test. The examiner demonstrates the test by having 1 hand with fingers extended and the other with clenched fist; in the hand positions are reversed by alternating opening and closing each hand in a rhythmical sequence. Asked the patient to perform the sequence.
Motor impersistence. Test distractibility. Patients are given a sequence of tasks to do. They only briefly sustain motor respose to commands such as "Raise your arms...Look to your right." Auditory Go-No-Go Test in which the patient is asked to move a finger in response to one tap on the table, but must keep it still in response to two taps. Tests ability to suppress inappropriate stimuli.
Frontal release signs: grasp reflex, glabellar tap, palmomental reflex, snout and pout reflex, root reflex, and abulia. Changes in personality and judgement may be seen.
Grasp reflex. To elicit, will lightly stroking her hand across the patient's palm while distracting the patient with casual conversation. A positive response it is an involuntary grasping, which can be forceful or subtle (do not instruct the patient "not to grasp"). Usually, contralateral finding to the abnormal frontal lobe in the location, and bilateral and dementias with frontal involvement and metabolic/infective encephalopathies.
Utilization behaviors when the patient reaches out to pick up and uses objects around them in appropriate to the social setting, with a limited capacity to override such actions. It may occur spontaneously (example. Fiddling with your stethoscope or tie or a phone handing the patient an item without instruction, the unusually toy with it. This was seen in frontal lobe disorders and can be present in subcortical lesions, but is highly indicative of an underlying brain disease.
Logic and abstraction: Can the patient solve simple problems. "How is an apple and banana alike?" "If Mary is taller than Jane, and Jane is taller than Ann, who's the tallest?" Proverb interpretation: "Make hay while sun shines?" Education background must be taken into account before administering these test.
These functions may be abnormal in damage to a variety of brain areas involving higher-order association cortex and are not well localized.
Mood and Affect: Signs of anxiety, depression, and mania. Largely obtainable through history. Depression: SIG EM CAPS
Thought content: obsessions, complusions, delusions, suicidal ideation or intent, homicidal ideation or intent. Anxiety d/o characterized by preoccupation with worrisome thoughts.
Disorders of perception: Hallucinations, paranoia. Mania causes patients to be abnormally active and cognitively disorganized.
Seen in toxic, metabolic encephalopathy and other causes of diffuse brain dysfunction, primary pyschiatric d/o, automatism and other sensory phenomena caused by seizures arising form mesial temporal lobe - hippocampal, limbic cortex. Also in visual, somatosensory, or auditory cortex.
Thyroid dysfunction
Somatization d/o, conversion d/o
Brain tumors, strokes, metabolic derangements, encephalitis, vasculitis.
Cranial Nerves. It can raise red flags that suggest a specific neurologic dysfunction rather than a systemic disorder.
Lethargy, unsteadiness, headaches, dizziness, dysphagia, or aphasia have many medical causes. However, any of these symptoms together with cranial nerve abnormalities strongly suggests brainstem dysfunction as the cause.
Smell (CN I): Can the patient smell coffee, other aromas with each nostril? Noxious odors are not tested, since they stimulate pain fibers of CN V. CN I is not tested unless specific pathology as a subfrontal brain tumor is suspected.
Impairment can be due to nasal obstruction, damage to the olfactory nerves in the nasal mucosa, damage to the nerves as they cross the cribriform, or intracranial lesions affecting the olfactory bulbs.
Vision (CN II):
Examine both retinas with an opthalmoscope.
Mnemonic for DDx of papilledema, papillitis, and acute retrobulbar neuritis:
Papilledema: If the examiner sees a swollen disc and the patient is able to see as usual, although blurry.
Papillitis: If the examiner sees a swollen disc and the patient does not see well or at all.
Retrobulbar neuritis: If the examiner sees nothing (abnormal) and the patient sees nothing (blind).
Visual acuity. Test each eye individually using a near card (Rosenbaum's)
Color vision. Test each eye separately for ability to distinguish colors. Test for red desaturation, a sign of subtle asymmetry in optic nerve function, for example, as in optic neuritis. Ask the patient to cover each eye alternately while looking at the red object and to report any relative dullness of the color in one eye.
Visual fields. Test each eye separately and then together. Ask patient to fixate straight ahead and to report when a finger can be seen moving in each quadrant. Alternatively, ask the patient to report how many fingers are being shown in each quadrant. More precise mapping of visual fields can be done by automated testing for patients who will be followed over time. In comatose or uncooperative patients, visual fields can be tested roughly using blink-to-threat.
Visual extinction. Test for visual extinction on double simultaneous stimulation by asking the patient how many fingers are being held from both sides at the same time. In visual extinction, a form of hemineglect, patients do not report seeing the fingers on the affected (usually left) side of the visual field, although they can see fingers when they are presented to that side alone.
Damage to the visual pathway anywhere from the eye to the visual cortex can cause specific deficits in the visual fields of one or both eyes.
Lesions in front of the optic chiasm cause visual deficts in one eye, while lesions behind the optic chiasm cause visual field deficits that are similar for both eyes.
Visual hemineglect is seen in right hemisphere lesions.
Visual hemineglect and extinction is usually caused by contralateral parietal lesions, and less likely by frontal or thalamic lesions.
Pupillary response (C II, CN III):
Pupil size, shape at rest.
Direct and consensual response
APD: Decreased direct response caused by decreased visual function in one eye. Demonstrated with the swinging flashing test, in which the light is moved back and forth between the eyes every 2-3 seconds. The afferent pupillary defect becomes obvious when the flashlight is moved from the normal to the affected eye, and the affected eye pupil dilates in response to light. Under normal conditions, the pupil constricts in response to light. Brief oscillations of pupillary size called hippus occur normally in response to light and should not be confused with an afferent pupillary defect.
Pupillary response to accomodation. Normally pupils constrict while fixating on a near object or as the object is moved near towards the eyes.
What's being tested?
Direct response. Impaired in lesions of the ipsilateral optic nerve, the pretectal area, the ipsilateral parasympathetics travelling in CN III, or the .
Consensual response. Impaired in lesions of the contralateral optic nerve, the pretectal area, the ipsilateral parasympathetics travelling in CN III, or the pupillary constrictor muscle of the iris.
Accomodation. Impaired in lesions of the ipsilateral optic nerve, the ipsilateral parasympathetics traveling in CN III, or the pupillary constrictor muscle, or in bilateral lesions of the pathways from the optic tracts to the visual cortex. Accommodation is spared in lesions of the pretectal area that may impair the pupillary light response.
EOM (CN III, IV, VI). EOM. Ask if there is diplopia.
Test smooth pursuit by having he patient follow an object moved across their full range of horizontal and vertical movements.
Test convergence movements.
Check for nystagmus
Dysconjugate gaze (eyes not both fixated on the same point), resulting in diplopia (double vision).
Saccades are eye movements used to rapidly refixate from one object to another. Test by holding two widely spaced targets in front of the patient (examiner's thumb on one hand and examiner's index finger on the other hand); ask patient to look rapidly back and forth between the target (e.g., by saying, "Now look at my finger...no look at my thumb....finger.....thumb"). Test in both horizontal and vertical axis.
Ocular dysmetria is seen in cerebellar lesions. There is overshoot of visual targets during saccadic eye movements.
OKN (optokinetic nystagmus) by moving a strip with parallel stripes in front of the patient's eyes and asking them to watch as the stripes go by. Normally, rhythmic movement of the eye called nystagmus occur, consisting of an alternating slow phase with slow pursuit movements in the direction of the strip movement, and a rapid phase with quick, saccadic refixations back to the midline. Test in both horizontal and vertical axis.
Oculocephalic or caloric testing.
What is being tested?
Careful testing can often identify abnormalities in individual muscles or in particular cranial nerves (oculomotor, trochlear, or abducens) in their course from the brainstem to the orbit, in the brainstem nuclei, or finally, in the higher-order centers and pathways in the cortex and brainstem that control eye movements.
Spontaneous nystagmus can indicate toxic or metabolic conditions such as drug overdose or alcohol intoxication, or peripheral or central vestibular dysfunction.
Asymmetric loss of OKN results from frontal or parietal lobe lesions on the side to which the tape is moving.
Facial sensation and muscles of mastication (CN V).
Test facial sensation using a cotton wisp and a sharp object. Also test for tactile extinction using double simultaneous stimulation.
Corneal reflex, which involves both CN V and CN VII, is tested by touching each cornea gently with a cotton wisp and observing any asymmetries in the blink response.
Feel the masseter muscles during jaw clench and pterygoids by asking the patient to move the jaw from side to side. Test for a jaw jerk reflex by gently tapping on the jaw with the mouth slightly open.
What is being tested?
Facial sensation can be impaired by lesions of the trigeminal nerve (CN V), the trigeminal sensory nuclei in the brainstem, or ascending sensory pathways to the thalamus and somatosensory cortex in the postcentral gyrus
The corneal blink reflex is mediated by polysynaptic connections in the brainstem between the trigeminal (CN V) and facial (CN VII) nerves and can be impaired by lesions anywhere in this circuit. (EDX tested as Blink reflex).
Extinction in the presence of intact primary sensation is usually caused by right parietal lesions.
Weakness of the muscles of mastication can be due to lesions in the upper motor neuron (UMN) pathways synapsing onto the trigeminal (CN V) motor nucleus. It can be in the lower motor neurons (LMNs) of the trigeminal motor nucleus at the level of the pons or as the fibers exit the brainstem to reach the muscles of mastication. It can be due to lesions at the neuromuscular junction, or in the muscles themselves.
Presence of a jaw jerk reflex is abnormal, especially if it is prominent. It is a sign of hyperreflexia associated with lesions of UMN pathways projecting to the trigeminal motor nucleus. Both the afferent and the efferent limbs of the jaw jerk reflex are mediated by CN V.
Facial expression and Taste (CN VII).
Asymmetry in facial shape and expression, nasolabial fold, depth of furrows, blinking and widened palpebral fissure are indicative of facial weakness.
Check the strength of eye closure against active resistance.
Facial diplegia is when facial weakness occurs bilaterally and symmetrical.
Old photographs of the patient can often aid in recognition of subtle changes.
Ask patients to smile, puff out their cheeks, screw their eyes tight shut, lift their eyebrows.
Check taste with sugar, salt, or lemon only when specific pathology is suspected in facial nerve or lesions of the gustatory nucleus (nucleus solitarius). Absence of taste confirms a peripheral CN VII lesion proximal to the junction of the chorda tympani.
What is being tested?
UMN lesion in the contralateral motor cortex or descending CNS pathways
LMN lesion in the ipsilateral facial nerve nucleus or exiting nerve fibers, neuromuscular junction, or the face muscles.
Upper part of face has bilateral projections to the facial nuclei. UMN lesions such as stroke cause contralateral face weakness sparing the forehead, while LMN lesions such as facial nerve injury typically cause weakness involving the whole ipsilateral face.
Hearing and Vestibular Sense (CN VIII).
Finger rub or words whispered.
Rinne and Webber test using a 512-Hz tuning fork.
Strength: Mayo > MRC || 0 = 5 / normal/full strength || -1 = 4+ / mildly weak || -2 = 4 / moderately weak || -3 = 4- / severely weak but still antigravity || -3.25 = 3 / antigravity only || -3.5 = 2 / movement if gravity eliminated || -3.75 = 1 / muscle flicker/twitch || -4 = 0 / no movement || *pain limited || ** or GW = give way weakness
Reflexes: Mayo > conventional: -4 = 0 (absent) || -3 = 1+ (75% decreased) || -2 = 1+ (50% decreased) || -1 = 1+ (25% decreased) || 0 = 2+ (normal) || +1 = 3+ (25% increased) || +2 = 3+ (50% increased) || +3 = 3+ (75% increased) || +4 = 4+ (clonus)