and Gunhild Waldemar1
Department of Neurology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark
After finishing the history, the neurologist should have a distinct anatomical hypothesis that can be confirmed (or rejected) during the bedside examination. Specifically, the neurologist seeks to elicit the signs compatible with this hypothesis, to confirm the absence of signs irreconcilable with it, and to verify that the rest of the examination is normal. A standard bedside examination includes evaluation of consciousness and cognition, cranial nerves, sensorimotor and cerebellar function, and gait and a general medical assessment. In cooperative patients, this can often be done in less than 10 min. In addition, tactful observation of the patient before, during, and after the consultation can reveal a wealth of information regarding neurological function. In this chapter, the reader will find in-depth information and practice tips concerning the examination of neurological patients, including those with decreased consciousness, epileptic seizures, and functional deficits.
KeywordsBedside examinationCerebellar functionCognitionConsciousnessCranial nerve examinationGaitMental functionMotor functionSensory functionSystem overview
The neurological examination has three goals:
To elicit the signs that confirm the anatomical hypothesis
To verify the absence of neurological signs incompatible with the anatomical hypothesis
To assess whether the remainder of the neurological examination is normal based on a complete bedside examination
As the neurologist gains experience, much can be deduced from simply observing the patient during the consultation, and given that time is limited, choosing not to do a complete bedside examination is often an option. Although often acceptable and sometimes inevitable, it is important to be aware of the fact that crucial information may easily be missed. For instance, if the CN examination in a patient presenting with a symmetric proximal weakness is omitted, the ophthalmoplegia that is essential for the diagnosis of a mitochondrial disorder may be overlooked.
With some experience and unless detailed cognitive testing is necessary, a neurological bedside examination can be performed in less than 10 min. To avoid confusion, arranging the examination according to functional systems and assessing one functional system at a time are advisable (Table 3.1).
Suggestions for a standard bedside examination in the cooperative patient
Cognitive examination: arousal, consciousness, attention; orientation to personal data, time, place, and situation; episodic memory, praxia, spontaneous language, comprehension, behavior, and mood
Cranial nerve examination:
Visual fields and acuity (CN II)
Direct and indirect pupillary reflexes (CN II, III)
Full range of eye movements and smooth pursuit (CN III, IV, VI)
Facial sensation (CN V)
Facial muscle power (CN VIII)
Palatal contraction (CN IX, X)
Head turning and shoulder lifting (CN XI)
Tongue power and diadochokinesia (CN XII)
Straight arm test
Formal power testing, including:
Elevation and abduction of the arm at 90° (deltoid muscle)
Adduction in the same position (major pectoralis muscle)
Elbow flexion with forearm supinated (biceps muscle)
Elbow extension (triceps muscle)
Extension of the wrist (extensor carpi radialis longus muscle)
Pincer grip (flexor pollicis brevis, flexor digitorum superficialis, and opponens pollicis muscles)
Finger abduction and adduction (interossei muscles)
Hip flexion (iliopsoas muscle)
Knee extension (quadriceps femoris muscle)
Knee flexion (biceps femoris, semitendinosus, and semimembranosus muscles)
Dorsiflexion of foot (tibialis anterior muscle)
Plantar flexion of foot (gastrocnemius, soleus muscles)
Muscle tone in arms and legs
Reflex status, including:
Finger flexor (C6/C7)
Sensory examination: touch at hands and feet (pin prick only if patient has sensory complaints); vibration and proprioception in the great toe
Cerebellum: finger-(nose)-finger test, knee-heel test; gait (including Romberg’s test)
Gait: normal speed, turning around, walking on toes and heels, walking on a line; classification of involuntary movements, if present
System overview: auscultation of heart, carotids, lungs; pulse, blood pressure; temperature
The following neurological examination may be regarded as routine at the bedside:
Assessment of consciousness, orientation, higher mental functions, behavior, and mood
CN examination including ophthalmoscopy
Motor examination including muscle power, tonus, inspection for possible muscular atrophy, deep tendon reflexes, and plantar reflexes
Sensory examination including assessment of pain, temperature, vibration, and proprioception
System overview, e.g., vital parameters and auscultation of the heart, lungs, and carotid arteries
Assessment of mental function, CN examination, and evaluation of power in the upper extremities are best performed with the patient sitting. Assessment of power in the legs, muscle tone, and reflexes in the upper and lower extremities, sensory function, and cerebellar function (e.g., finger-nose, heel-knee test), as well as system overview, is most conveniently performed with a supine patient. At the end—or at the very beginning of the examination—gait is assessed, which provides an invaluable overview of total performance. (Indeed, informal observation of gait should start when the patient walks down the corridor to the neurologist’s office.)
It cannot be overemphasized that neurological assessment of the patient is not restricted to the formal bedside examination. Before, during, and after consultation, careful but discreet observation of the patient may reveal a wealth of information regarding neurological function. To name but a few examples, difficulties with finger movements of one hand while undressing as well as outward rotation of the ipsilateral foot while walking into the office may disclose a mild hemiparesis; gait performance of the patient with a functional disorder tends to be much better outside the office than during the consultation; and a spontaneous smile during conversation may reveal a mild facial palsy. Also, when a patient is in bed, as typically happens during ward rounds, make every attempt to get him out of it. In a patient with a midline cerebellar lesion, for instance, muscle power and coordination in the extremities can be normal, and truncal instability and gait ataxia may only become apparent when the patient is out of bed.
The following section provides an overview of the formal neurological examination. Table 3.1 highlights routine tests, but these and more comprehensive testing of each functional system are reviewed below in greater detail.
3.1 Cognitive and Mental Functions
Bedside examination of the patient with cognitive or mental impairment is challenging, because the abilities used by the patient to construct a history and description of symptoms are also the potentially defective abilities that are under scrutiny. Some patients may not have a clear understanding of the reason for attending the clinic. Memory impairment as well as anosognosia (unawareness of deficits), aphasia, and disturbed mood or behavior may lead to an inadequate or unreliable history when obtained from the patient alone. Therefore, also obtaining a history from an informant is of the utmost importance. Whenever possible, the informant interview should be conducted in the patient’s absence. It is particularly helpful in getting an overview of the impact on activities of daily living of the presenting symptoms and in identifying psychotic and behavioral symptoms. Furthermore, discrepancies in the accounts from the patient and the informant help to assess the level of anosognosia.
3.1.1 The History as Part of the Cognitive Examination
Taking the history from the patient also forms an important part of the cognitive examination and serves to identify areas of particular interest for further formal assessment. During the patient interview, the neurologist will get an impression of memory from the patient’s ability to recall events in the autobiographical and medical history and information about language from the patient’s spontaneous conversation and ability to comprehend. The examiner will also have the opportunity to get an impression of the patient’s insight, mood, behavior, and possible psychotic manifestations. Be sure to inquire into the patients’ understanding of the reason for the visit. During the interview, the patient with amnesia or dementia may turn his head back to the spouse or caregiver, as if he is silently looking for some cues directly to the question. This characteristic behavior is called the head-turning sign. Many of the following symptoms are delicate—although important—issues and are best enquired about with the informant in the absence of the patient. Explain to the patient why you need to talk with the informant.
220.127.116.11 Cognitive Symptoms
The cognitive history should explore the different cognitive domains:
Are there problems with attention, slips, and poor concentration (working memory)?
Is knowledge about facts and objects impaired and is there loss of memory for words or vocabulary (semantic memory)?
Does the patient have impaired recall of specific events? Does he have difficulties remembering appointments and events, e.g., are there problems recalling details of a recent phone conversation, television program, or a birthday party last Sunday (episodic memory)?
Language and speech:
Expression: Are there any problems with naming, with word order, or with word endings?
Comprehension: Does the patient have problems with understanding conversations or with “hearing” when many people are gathered in a group?
Are there any problems with reading or writing?
Is the patient easily distracted?
Are there problems with organizing, shopping, and other household activities or with using new appliances or electronic devices in the home?
Does the patient appear disorganized?
Is the patient’s ability to plan and set goals impaired?
Does the patient show impaired handling of money, e.g., getting correct change in the supermarket and difficulties handling bills or bank accounts?
Is there disturbed planning of motor functions, e.g., getting dressed and brushing teeth?
Does the patient have problems in finding his way around?
Are there difficulties reaching out for objects? Is there a tendency to miss steps (suggesting biparietal pathology)?
Does the patient have trouble recognizing faces (prosopagnosia, suggesting bilateral occipito-temporal pathology)?
18.104.22.168 Mental Functions
Brief assessment scales for depression (e.g., Geriatric Depression Scale, Cornell Scale for Depression in Dementia, or Hamilton Depression Scale) and for neuropsychiatric symptoms (e.g., Neuropsychiatric Inventory) are available for systematic enquiry of mental functions:
Personality changes may arise insidiously many years prior to other impairments in patients with dementia. Disengagement, loss of empathy, apathy, agitation, and/or disinhibition is characteristic for specific dementia disorders.
Behavioral changes may be very disturbing and include aggression, agitation, stereotypical behavior, psychomotor restlessness, and wandering (Case 3.1).
A person with Godot’s syndrome is anxious and repeatedly asks questions about an upcoming event—a behavior which may result from decreased cognitive abilities and from the inability to apply remaining thinking capacities productively.
Depression is a common differential diagnosis to dementia, and depressive symptoms occur often in degenerative dementias. Mood disturbances should always be assessed and classified, e.g., anxiety, tiredness, pessimistic ruminations, and suicidal ideation (mania or euphoria is rarer).
Hallucinations in cognitively impaired patients most often occur in the visual modality but may also affect auditory, gustatory, olfactory, and tactile modalities. Hallucinations are a particular feature of dementia with Lewy bodies (DLB) but may also occur in patients with other dementias and delirium.
Delusions are false beliefs, occurring often in patients with neurodegenerative dementias, and sometimes they are associated with misidentifications. The most common delusions are:
Abandonment: The belief that the caregiver plans to abandon the patient
Theft: The belief that someone has stolen a misplaced object
Infidelity of the spouse
The delusion that one’s home is not one’s home
Capgras’ phenomenon: The belief that the spouse or another relative has been replaced by an imposter who looks exactly like him/her
Phantom boarder syndrome: The belief that someone uninvited is living in the patient’s house
De Clerambault’s syndrome: The belief that someone is in love with the patient
Misidentification of the patient’s own self (the picture sign): The belief that the person in the mirror is not the patient but some other person (self-prosopagnosia)
Misidentification of events on television (the patient imagines these events are occurring in real three-dimensional space)
Bizarre delusions may occur in semantic dementia (SD)
Cotard’s syndrome: The belief that the patient is dead, or that parts of his body no longer exist.
Alzheimer’s disease with atypical onset (behavioral symptoms). A 68-year-old male was referred for diagnostic evaluation of possible frontotemporal dementia. Two years earlier, his wife had noticed gradual onset of personality change with inappropriate (and sometimes disinhibited) social behavior and word-finding difficulties. His symptoms had been progressing rapidly. The neurological and neuropsychological examination revealed a global aphasia with severely impaired comprehension, word finding, and writing, as well as impaired calculation and a mild apraxia. The MMSE score was 11/30. Axial and coronal MRI showed temporoparietal atrophy (more pronounced in the right hemisphere) (a, b). FDG PET demonstrated classical bitemporoparietal hypometabolism, which was also more pronounced on the right side (c, d). Amyloid PET (11C-PIB PET) was positive. The diagnosis was AD with atypical onset (PET images courtesy of Ian Law, Department of Clinical Physiology and Nuclear Medicine, Rigshospitalet, Copenhagen)
Sleep pattern, eating pattern, and sexual behavior may be altered as a result of neurodegenerative disorders. For instance, preference to certain (sweet) foods or even pica-type syndromes1 may be characteristic in frontotemporal dementia and can lead to overweight. Common sleep disturbances are REM sleep disorder, fragmented sleep, day-night reversal, nightmares, and nocturnal hallucinations. Changes in sexual behavior include increased or decreased libido.
Other neurological symptoms of particular interest in patients with cognitive impairment are gait difficulty (pointing to involvement of the extrapyramidal system or the cerebellum or to the presence of a hydrocephalus), visual disorientation (suggesting impaired eye movements), parkinsonism, dysarthria, dysphagia, myoclonus, and motor weakness.
22.214.171.124 Onset and Progression
Was the onset sudden or insidious? Was there any event eliciting the onset of symptoms? Even when there was an acute event, carefully ask about any subtle symptoms prior to the event. Is the condition static (e.g., Korsakoff’s syndrome), or is there a gradual (neurodegenerative) or stepwise (multi-infarct) progression? Are the symptoms progressing very rapidly (for rapidly progressive dementias, see Chap. 4)?
126.96.36.199 Past Medical History
Of particular interest is a history of seizures, stroke, encephalitis, traumatic brain injury, and depression. Drug history and establishing the level of alcohol intake are important elements of the history. Non-neurological conditions such as malignancy, metabolic conditions, and infections may also be related to cognitive impairment.
188.8.131.52 Family History
Ask for family history of memory impairment, dementia, psychiatric illness, movement disorder, and ages and causes of death.
184.108.40.206 Activities of Daily Living, Social Consequences, and Driving
Brief assessment scales are available for informant interview on activities of daily living. Ask the informant to describe the patient’s typical daily activities at home and at work, if relevant. Ask about the ability to use electronic appliances, personal hygiene, handling finances, as well as shopping, cooking, and other household activities. Interview the caregiver about driving abilities and about accidents while driving.
Further examination is indicated if the history suggests mental changes or cognitive dysfunction.
3.1.2 Bedside Examination of Mental and Cognitive Functions
During the bedside examination of the mental functions, pay attention to the following:
The appearance of the patient: The patient’s general appearance should be noted, including dressing, grooming, makeup, body odor, overall behavior and attitude (hostile, guarded, cooperative?), and psychomotor features (restlessness, psychomotor retardation?).
Mood (level and stability) and affect: Is the patient depressed, anxious, or euphoric? Is the mood fluctuating/labile? Does the patient have an appropriate or blunt affect?
Behavior: Is the patient’s behavior in the consultation room appropriate or not (e.g., agitated, disinhibited)?
Bedside examination of cognitive functions may seem complex, but it often reveals striking features in patients with cognitive impairment, and a global overview of cognitive functions with a few supplementary tests in specific cognitive domains can be performed in less than 20 min. Be aware that it may not be possible to assess certain specific cognitive domains due to impairment in others. For example, patients with delirium have severely impaired attention, which is why tests for episodic memory are meaningless.
Several brief screening instruments for global cognitive evaluation are available for the clinician, including:
The Mini-Mental State Examination (MMSE) is a good starting point (Folstein et al. 1975). Sometimes there is a striking difference between the well-preserved facade and a low score on the MMSE. Occasionally, it is helpful to skip the history and start with an MMSE as doing an extensive auto-history is a waste of effort, for example, in a patient with a score of 10. Instead, much time can be saved if a relative or another informant is contacted right away. At the traditional cutoff of 23 points (used in population studies), the MMSE has high sensitivity for cognitive impairment but rather low specificity. Remember that a score of 25 may be perfectly normal for a low-educated worker, but 28 points in an academic may already be pathological. Low scores may also be seen in patients with depression. It is possible (and highly advisable) to learn the MMSE by heart. With practice, it is administered at the bedside in less than 10 min. Note, however, that the MMSE is heavily weighted toward verbal tasks and does not assess executive function. For instance, a patient with frontotemporal dementia (FTD), unable to behave in a socially appropriate manner, and a patient with a severe right hemisphere dysfunction may score 28/30. Also, well-educated patients with early-phase AD may have very high scores.
The Rowland Universal Dementia Assessment (RUDAS) (Storey et al. 2004) is a brief cognitive screening instrument specifically designed to minimize the effect of culture, language, and education.
Addenbrooke’s Cognitive Examination (ACE) (Mathuranath et al. 2000) may also be suited for the bedside examination and has a higher sensitivity (traditional cutoff is 83/100).
The Montreal Cognitive Assessment (MoCA) is another easy-to-use cognitive screening test designed to assist in the detection of mild cognitive impairment and Alzheimer’s disease; it is freely available online. The MoCA may be more sensitive than MMSE in detecting mild cognitive impairment.
A full and detailed examination of cognitive function requires referral to a formal neuropsychological examination (see Chap. 5). However, the neurologist may be interested in quickly assessing specific cognitive domains in more detail, as outlined below, putting most emphasis on apparently impaired functions:
Orientation and attention: Orientation to time and place, digit span forward and backward (a normal person should be able to repeat at least six digits forward and a couple less backward), and subtracting backward (e.g., 100 − 7).
Language (naming, comprehension, reading, and writing): Characterize the patient’s spontaneous language (fluency, vocabulary, syntax, grammar, and phonology). Ask the patient to repeat multisyllabic words (impaired in fluent aphasia). Ask the patient to name three objects (e.g., stethoscope, chalk, and toothbrush); then hide each of them in the consultation room in order to assess memory, as outlined below. Ask the patient to name objects on drawings. It is important to use objects of middle-range familiarity and not very familiar objects. Check verbal comprehension by asking the patient to define the meaning of words and concepts (e.g., retirement, artichoke). Let the patient read a text from a magazine or book and check pronunciation of orthographically irregular words (words not pronounced as spelled, e.g., island). Difficulty with reading irregular words (surface dyslexia) is pronounced in SD. Ask the patient to write a sentence.
Episodic memory: Let the patient learn and recall (after 5–10 min of delay) a name and address; ask the patient to recall the name and location of the three hidden objects mentioned above. The patient with AD or amnesic syndrome will be able to learn and repeat an address or word list—but will have difficulty recalling all items after a delay.
Semantic memory: Ask the patient to name the three most recent prime ministers or presidents and three major capital cities, to name objects, and to define the meaning of words.
Visuoperceptual and visuospatial abilities:
Bilateral posterior pathology may lead to difficulty describing a complex picture, and the patient may only be able to describe individual details (simultanagnosia). There are validated drawings available for this test, but a suitably complex picture in a magazine will also suffice.
Visuospatial skills are tested by letting the patient draw a cube, overlapping pentagons, or a clock face. The clock drawing test checks neglect as well as general visuospatial skills and executive functions. Give the patient a sheet of paper and ask him or her to draw a circle on it. Instruct the patient to draw numbers in the circle to make it look like the face of a clock and then to draw the hands of the clock to read “20 min past 10.” The patient with neglect tends to ignore the objects on one side, usually the left. Tests of line bisection or letter cancellation serve similar purposes.
Praxia: Apraxia is usually bilateral but may be unilateral or asymmetric in corticobasal degeneration (CBD). Check for apraxia using verbal instructions (“Show me how to brush teeth, how to comb your hair.”); if this fails, use visual instructions (“You brush teeth like this—now can you imitate me?”). Also, the patient can be asked to imitate a few complex hand movements. A patient with normal praxia should be able to follow these instructions completely. Minor deviations, e.g., using the finger as a virtual toothbrush instead of showing how to actually hold the toothbrush, may already be pathological. Oral (buccofacial) apraxia (e.g., difficulties licking the lips, blowing out matches) is common in nonfluent aphasia.
Stereognosis: Ask the patient to close his eyes and to identify an object, e.g., a coin or a key, using tactile sensation of one hand only.
In category fluency, the patient is asked to produce as many examples as possible in 1 min from a given category (e.g., animals) and in letter fluency, to generate as many words as possible starting with a given letter (e.g., “S”). Normal subjects can name more than 15 words for letter fluency and a few more on category fluency. The tests are differentially impaired in frontal-subcortical dementias (the normal difference between category and word fluency is exaggerated) and AD and SD (worse on category fluency).
Abstraction may be tested by asking the patient to explain the meaning of sayings like “too many cooks spoil the soup” or by asking the patient to explain differences and similarities, e.g., “How are a table and a chair similar?” or “What is the difference between a canal and a river?” Judgment may be tested by asking the patient to estimate the size of objects unknown to them, e.g., “How tall is the Eiffel tower?” or “How many people fit into a London sightseeing bus?”
Disinhibition may be revealed by the go/no-go test in which the patient with frontal pathology is unable to stop. Ask the patient to tap the desk once if the examiner does so, but if the examiner taps twice, the patient should not tap at all. Further, the so-called applause test is useful in a patient with suspicion of a neurodegenerative disorder. When asked to clap three, and only three, times as quickly as possible after seeing a demonstration by the examiner, prolonged clapping is suggestive of a neurodegenerative disease. Reports have suggested that the specificity of the applause sign is 100% in distinguishing parkinsonian patients from normal subjects with the highest sensitivity in patients with CBD and PSP, although the applause sign can also be seen in other neurodegenerative disorders, including AD.
3.1.3 General Neurological Examination in the Cognitively Impaired Patient
The following aspects of the general neurological examination require particular attention in the cognitively impaired patient:
Primitive reflexes are those that are present in early life but suppressed during adolescent brain maturation. They commonly reemerge during advanced stages of dementia and are then called frontal release signs (e.g., snout, rooting, palmomental, and grasp reflexes). It is important to note, however, that some of them such as the palmomental reflex may be elicited in perfectly healthy individuals, and they all lack localizing value.
Utilization behavior may be pronounced in FTD: The patient will begin to use any object placed in his hand, even if instructed not to. Give the patient several pairs of glasses, one at a time, and the patient will attempt to put them all on, one after the other. Imitation behavior may also be a striking feature in FTD, e.g., the patient will imitate the examiner’s movements automatically.
Speech: Dysarthria raises the suspicion of MND and parkinsonian syndromes/parkinsonism. Echolalia (repeating the examiner’s words), palilalia (repeating sounds), and palilogia (repeated utterance of words) may be observed in patients with frontal pathology.
Dysphagia suggests MND or atypical parkinsonian syndromes.
Eye movements and the eyes themselves are important to examine (e.g., supranuclear palsy or slowing of saccades suggests PSP; Kayser-Fleischer ring suggests Wilson’s disease).
Motor function: Look for extrapyramidal features suggesting Parkinson’s disease or other parkinsonian syndromes, weakness and fasciculations (seen in FTD-MND), myoclonus (Huntington’s disease (HD), Creutzfeldt-Jakob disease (CJD), CBD and advanced AD), chorea (HD), limb dystonia (CBD or Wilson’s disease), and asterixis (metabolic encephalopathy). The patient with the alien limb phenomenon, typically seen in CBD, complains that one arm is becoming increasingly useless and performs movements and actions “on its own.”
Gait: Gait ataxia may suggest cerebellar involvement (neurodegenerative disorders, alcohol-related cerebellar degeneration, MS, leukodystrophy, or CJD), a slow and unsteady ataxic gait may suggest NPH, and a shuffling gait with small steps suggests PD.
3.2 Cranial Nerves
With practice, a complete CN examination in a cooperative patient can be assessed in less than 90 s.
CN I is rarely tested; usually it is enough to simply ask if the patient can smell his partner’s perfume or the coffee in the morning. Otherwise, the patient can be examined using a selection of odors, testing one nostril at a time. Remember that ammonia and other irritants activate trigeminal fibers, not CN I.
Examination of CN II may begin with an ophthalmoscopy.2 The presence of spontaneous venous pulsations (SVPs) as revealed by ophthalmoscopy is a useful finding in a patient with headache and should be documented in the charts. SVPs occur at the optic disc where the retinal veins leave the orbit (the retinal veins are the darker, broader vessels; the arteries are lighter and thinner). With some practice, SVPs can be detected in nine of ten people. Video demonstrations of SVP can be found on youtoube.com. Venous engorgement and the lack of spontaneous pulsations are the earliest signs of raised intracranial pressure. Full papilledema usually takes a few days to develop and leads to blurring of optic margins, elevation of the optic disc, hemorrhages from congested retinal veins, and nerve fiber damage (cotton wool spots) (Case 3.2). Thus, the presence of SVP suggests that ICP is normal, at least at the time of examination. Although SVPs do not fully exclude an intracranial cause for the patient’s headache, their presence nevertheless allows the conclusion that headache is not due to raised ICP (e.g., as seen with sinus venous thrombosis, a cerebral tumor, or idiopathic intracranial hypertension). This is highly useful when evaluating a patient with headache.
Idiopathic intracranial hypertension and spontaneous CSF fistula. A 29-year-old female was diagnosed with IIH based on a history of headache, obesity, papilledema (a), increased CSF opening pressure, and lack of a mass lesion or CVST on an MRI of her brain. Although the MRI initially was reported as normal, it showed signs of increased intracranial pressure, such as vertical kinking of the optic nerves and flattening of the posterior sclera (b). She was treated with diuretics and acetazolamide, as well as given dietary advice. At a 2-year follow-up, her headaches had disappeared, and the fundus examination was normal. Treatment was discontinued. In the following years, she experienced continuous watery nasal discharge, consistent with CSF rhinorrhea, and two episodes of bacterial meningitis. CT and MRI revealed a left-sided nasal cavity meningocele (c, asterisk). Spontaneous CSF leaks have epidemiological and clinical features very similar to IIH. In one case series of spontaneous CSF leaks, 85% of patients were obese, 77% were women, and the CSF lumbar opening pressure increased following closure of CSF leaks. Increased ICP and constant pulsatile pressure lead to bone erosion. Spontaneous CSF leaks probably represent a variant of IIH, which is why after operative closure of a spontaneous CSF leak, patients must be carefully followed for signs of increased ICP to prevent recurrence of the leak (Adapted with permission from Kurtzhals et al. (2011))
Ophthalmoscopy may also reveal chronic optic neuropathy (small, pale, and atrophic optic disc), drusen (optic disc elevation due to mucoproteins and mucopolysaccharides without other signs of papilledema—usually a normal variant without any pathological value), diabetic and hypertensive changes (retinal hemorrhages, cotton wool spots, and atherosclerotic vascular changes), and optic neuritis (although with retrobulbar neuritis, inspection can be normal). Occasionally, one may see retinitis pigmentosa, cholesterol emboli and retinal infarction, retinal melanoma, and retinal toxoplasmosis.
If the history suggests loss of visual acuity, assess this using the classic Snellen chart. Optic correction (if needed) is important since the function of nervous tissue and not the lens apparatus should be assessed. If a Snellen chart is not available, a simple test of visual acuity is to let the patient read a newspaper using one eye at a time. Is he able to read the small text or only the big letters in the headings? If neither, can he count fingers from 20 in. away?
Testing the visual field is part of a standard neurological examination. For finger perimetry, the examiner uses his visual fields as a reference. If the patient’s history does not suggest any visual field loss, asking the patient if he can see finger movements in each quadrant of the eyes is usually sufficient. Each eye should be assessed independently by covering the other eye. Another more sensitive examination technique is to slowly move a finger or another object from each corner of the periphery toward the center of the patient’s visual field and to ask when the patient can see the object. Visual attention, however, is best tested using double confrontation. Show the patient both hands and ask him to point at the hand where the fingers are moving. Also, move both hands simultaneously at some time during the examination to assess for possible extinction. With complete visual neglect, there is extinction of visual stimuli of one side at all times; with visual hemineglect there is extinction only with double confrontation. Examination of visual fields and visual attention assesses not only CN II but the entire visual system and its cortical projections.
Color vision is normally only assessed when suspecting (chronic) optic atrophy, e.g., due to optic neuritis in MS. Since the color red is most significantly affected, the patient is shown a red object and asked to look at it first with one, then with the other eye. Is there desaturation of red with one of the eyes?
Optic atrophy leads also to an afferent pupillary defect, which can be tested for by applying the swinging-flashlight test. If the optic and oculomotor nerves and their parasympathetic connection (Edinger-Westphal nucleus) are intact, both pupils constrict and stay miotic when moving a flashlight quickly from one eye to the other. This is because of the consensual light response. With unilateral optic dysfunction, however, both pupils will constrict as light falls into the healthy eye, but they will dilate as light falls into the affected eye. The direct and indirect (consensual) pupillary reflexes can also be tested in a well-lit room by first covering one eye and then the other. This is especially useful in small children, who often do not like the glare of a pocket lamp.
Accommodation can be tested by asking the patient to fixate on a point on the wall and then to fixate on the examiner’s finger moving into the visual field just in front of the patient’s nose. Accommodation leads to constriction of the pupils and convergence of the eyes.
In contrast to afferent pupillary defects, efferent pupillary defects are due to CN III palsy, e.g., due to increased ICP, autoimmune mechanisms (Adie pupil), or infections such as neurosyphilis (Argyll-Robertson pupil). Drug intoxication is another cause, with atropine and sympathetic drugs leading to mydriasis and opioids to miosis.
It is important to actively look for Horner’s syndrome, because it is easily missed otherwise. Horner’s syndrome consists of miosis, ptosis, and anhidrosis. See Chap. 2 for a discussion of the relevant neuroanatomy. Anhidrosis is loss of sweating in the upper quadrant of the ipsilateral half of the face. It is tested with the back of the hand or—even better—with a plastic card moving over the forehead. With anhidrosis, the skin is smooth and dry, and the plastic card will not stick to the skin as much as on the unaffected side. When physical activity provokes facial flushing, the affected skin area remains pale. However, anhidrosis can be lacking when the sympathetic fibers traveling along the external carotid artery are spared. Miosis and pupillary efferent dysfunction are most easily assessed in a semi-dark room as miosis is most visible because the contralateral pupil dilates. When the light is turned on, the healthy pupil will constrict as well. (In contrast, mydriasis is most visible in a well-lit room.) Ptosis can be evaluated by comparing the border of the upper eyelids with the level of the pupils when the patient looks straight forward and upward.
Oculomotor function, mediated by CN III, IV, and VI, is assessed by evaluating gaze axis, range of eye movements, smooth pursuit, saccades, and, if present, nystagmus. The examiner moves a finger slowly to both sides and up and down in an H-like movement and asks the patient to follow it. The examiner’s finger should not be held too close to the patient’s eyes. Many patients will instinctively move their head slightly, which the examiner can avoid by fixating the patient’s head with the other hand. Oculomotor function is analyzed according to whether gaze is conjugated or divergent, whether pursuit is smooth or saccadic, and whether the range of movements is full or restricted (Case 3.3). If nystagmus other than end-gaze nystagmus is present, determine whether it is conjugate or unilateral, unidirectional or gaze changing, and horizontal or vertical, whether it occurs after a latent phase of a few seconds or immediately, whether it is positional or independent, and whether it is transient or constant. (The first adjective of each pair is consistent with vestibular nystagmus; the second suggests nystagmus of central origin.) For assessment of saccades, ask the patient to fixate on the examiner’s face. The examiner then holds up his hands and moves them suddenly and in a random manner. The patient has to look at the moving hand and then immediately at the examiner again. Are eye saccades precise, or does the patient need to perform corrections due to either overshoot or hypometric saccades?
Carotid-cavernous fistula. A 53-year-old previously healthy male presented with a 6-month history of watering of the right eye, double vision, and pulse-synchronous tinnitus. Examination revealed ptosis, ciliary injection, and restricted movements of the right eye (a). MRI showed exophthalmos, orbital edema, and a dilated orbital vein on the right side (b). A digital subtraction angiography was consistent with a low-flow carotid-cavernous fistula (asterisk) (c). The fistula was successfully embolized, and the symptoms disappeared (Digital subtraction angiography images courtesy of Markus Holtmannspötter, Department of Neuroradiology, Rigshospitalet, Copenhagen)
Skew deviation may be revealed by asking the patient to fixate on the examiner’s face, while the examiner covers one of the patient’s eyes with his hand. Skew deviation, as well as latent strabismus, is revealed by a corrective movement of the affected eye when the hand is removed (Chap. 2).
The VOR or oculovestibular reflex is a reflex eye movement that stabilizes the image on the retina during head movement by producing an immediate eye movement opposite to the movement of the head. Thereby, the image remains fixed in the center of the visual field. The head impulse test can be used for assessment of the VOR. Again, ask the patient to fixate on your face. Take the patient’s head in both hands and turn the head quickly 10–20° to one side. With vestibular failure, the eyes will follow the head movement for a fraction of a second until a compensatory saccade in the opposite direction allows the patient to fixate on you again. Take care not to miss this compensatory saccade as it is minute.3 With smooth pursuit, in contrast, VOR must be suppressed in order to allow a stable image on the retina. Ask the patient to extend both arms, to put his thumbs together, and to fixate them while you turn the patient en bloc. With correct VOR suppression, the gaze is fixed on the thumbs; with deficient VOR suppression (e.g., in a patient with MS), the patient’s gaze constantly lags behind, and compensatory saccades are necessary to catch up with the moving thumbs.
Oculomotor function is often impaired in neuromuscular junction disorders such as MG. For assessment of fatigability and ptosis, ask the patient to look upward for 60 s and look for occurrence of ptosis (Jolly’s test). When ptosis is subtle, it may be difficult to establish whether or not the Jolly’s test is positive. Ptosis is best detected when focusing on the decreasing gap between the upper eyelid and the upper border of the pupil. For other relevant bedside tests (tensilon test, ice-on-eyes test), consult the differential diagnosis of neuromuscular junction disorders in Chap. 4.
Facial sensation, mediated by CN V, is assessed by touching both halves of the face gently. There is usually no need to test the different qualities of facial sensation. Most sensory disturbances are obvious. One exception is sensory disturbance due to vestibular schwannoma, which initially is subtle and may sometimes only manifest as impaired corneal sensation leading to a decreased corneal reflex. Also, it is crucial not to miss complaints of circumoral sensory symptoms as these may point toward a brainstem lesion, as discussed in Chap. 2. The skin area innervated by V3 ends at the lower mandible; thus, sensory disturbances including areas below the mandible may suggest that these complaints are nonorganic in nature. Also, keep in mind that minor differences in pain and temperature sense between the right and left facial halves are often described by suggestible patients; again, such complaints are seldom due to an underlying organic disorder if the patient has not mentioned them spontaneously during history taking.
To test CN V motor function, have the patient bite his teeth together and palpate the masseter muscle bulks; one-sided atrophy suggests chronic damage to the motor nucleus or the peripheral axons, e.g., secondary to an ipsilateral skull base tumor.
CN V motor fibers constitute the efferent pathway of the jaw jerk. A hyperactive jaw jerk points toward bilateral UMN lesions such as in pseudobulbar palsy.
Frontal headache may be due to congestion of nasal sinuses and may increase with percussion of the bony area above the relevant sinus as well as with bending of the head forward. In the elderly, with new-onset headache and complaints of generalized muscle ache, weight loss, and/or pains in the jaw while chewing (jaw claudication), it is important to look for a prominent painful temporal artery suggestive of giant cell arteritis.
The most important question in a patient presenting with a facial palsy (CN VII) is, “Can you wrinkle your forehead?” This distinguishes central from peripheral facial palsy. In a patient with a subtle facial palsy, examine all three CN VII branches. Ask the patient to whistle, to show his teeth, and to repeat this maneuver rapidly, while you look for a slight palsy as revealed by one corner of the mouth lagging behind the other. Sometimes a mild facial palsy (whether central or peripheral in origin) is best detected during observation of spontaneous facial activity. An asymmetry of the nasolabial fissure may also occasionally reveal a facial palsy better than voluntary muscular activity. Facial reflexes are hyperactive with bilateral UMN impairment, e.g., due to multiple vascular lacunes (pseudobulbar palsy) and other neurodegenerative disorders. One of these reflexes is the so-called snout reflex (a frontal release sign), which leads to pouting or pursing of the lips elicited by a slight and constant pressure on the philtrum (essentially a variation of the tactile rooting response). Another example of a frontal release sign is the palmomental reflex, which consists of a twitch of the chin muscle elicited by stroking the thenar eminence and the palm. However, although it is held dear by many neurologists, the palmomental reflex lacks sensitivity, specificity, and localizing value; and therefore it should be considered obsolete (Schott and Rossor, 2016).
In a patient with a peripheral CN VII palsy (e.g., Bell’s palsy), ask about a loss of taste or an unusual metallic taste in the mouth. A useful but uncomfortable examination is the salt test. Ask the patient to protrude his tongue, fixate its tip with your hand (using gloves), and rub salt on the left and right side of the tongue. The patient with a peripheral CN VII palsy that also affects the chorda tympani will report loss of salt taste perception on the ipsilateral side of the tongue. Also test for hyperacusis by clapping your hands together in front of the patient’s ears. Consult Chap. 2 regarding localizing the lesion in peripheral facial palsy.
Lesions of the vestibular part of CN VIII may lead to vestibular ataxia and nystagmus, as discussed earlier. In vestibular damage, horizontal or torsional nystagmus occurs with a latency of a few seconds after position change and is habituating.
The Dix-Hallpike maneuver is used to diagnose the symptomatic side of the vestibular lesion. Youtube.com provides excellent demonstrations of the Dix-Hallpike maneuver. Have the patient sit upright and stand behind him, holding his head with both hands. Then rotate the patient’s head approximately 45° to one side. Afterward, help the patient to lie on his back with his head hanging over the edge of the examination table at roughly a 30° angle. When the Dix-Hallpike maneuver is positive, rotatory nystagmus occurs after a latency of 2–10 s, and the fast phase is toward the affected ear. If no nystagmus occurs, the maneuver is repeated with the head rotated to the other side.
The Epley maneuver, also demonstrated on youtube.com, can be used to cure benign paroxysmal positional vertigo. The first steps are identical to the Dix-Hallpike maneuver, but the patient remains in the position that provokes nystagmus for 1–2 min, after which the patients rotates his head 90° to the other side, remaining in this position for another 1–2 min while maintaining his head at a 30° angle. Then, have the patient roll over onto his side in the direction he is facing while keeping the head and neck in a fixed position relative to the body. Now the patient’s face should be pointing downward (toward the floor). Let the patient remain in this position for 1–2 min before slowly returning to an upright sitting position, where he should remain for up to 30 s. The entire procedure can be repeated once or twice.
The Unterberger stepping test can be useful in assessment of vestibular pathology. Ask the patient to walk in place for 60 s with his eyes closed. If the patient turns more than 45° to one side, this is pathological and suggests a labyrinthine lesion on that side. A similar test is the finger-pointing test, in which the patient lifts both arms up and down a few times, again with eyes closed, while trying to keep his fingers pointed in the same direction. Deviation suggests an ipsilateral vestibular lesion.
Sensorineural deafness (due to injury of the cochlear part of CN VIII) is usually obvious during normal speech. If not, rub your index finger and thumb together in front of the patient’s ears to reveal slight hearing impairment. The Weber and Rinne tests allow distinguishing sensorineural from conductive hearing impairment. During the Weber test, a vibrating tuning fork is placed in the middle of the patient’s head. If available, a 512 Hz tuning fork is preferable; otherwise, a common 256 Hz fork will do as well. With conductive hearing loss, the patient will hear the sound lateralized toward the affected ear, whereas the opposite is true with sensorineural hearing loss. Then, perform the Rinne test by placing the vibrating tuning fork on the patient’s mastoid. When the patient no longer hears the sound, hold the fork in front of the ipsilateral ear. Since air conduction is normally better than bone conduction, the patient should hear the sound again and at least twice as long as with bone conduction. For instance, with conductive hearing loss of the left ear, the Weber test will be lateralized to the left, and, since bone conduction is better than air conduction in this case, the Rinne test will be negative on the left side. In contrast, with left sensorineural hearing loss, the Weber test will be lateralized to the right, while the Rinne test will remain positive (showing that air conduction is still better than bone conduction).
CN IX and CN X are assessed together. In most cases, simply asking the patient to stick out his tongue and say “aaah” and watching whether or not the soft palate and uvula are lifted symmetrically are sufficient. If the patient is able to clear his throat, this suggests preserved diaphragmatic function and sufficient closure of the epiglottis. Chapter 2 discusses the gag reflex and the curtain phenomenon due to ipsilateral uvula paresis. Dysphagia can be assessed at the bedside by having the patient (carefully) drink a glass of water. Dysarthria is usually obvious when taking the history but can be formally tested for by having the patient say “p-p-p” (CN VII), “t-t-t” (CN XII), and “k-k-k” (CN IX/X). The examiner needs to decide whether dysarthria is spastic (high-pitched sounds, e.g., due to supranuclear pseudobulbar palsy), atactic (“eve-ry syl-la-ble-tends-to-be-pro-nounced-by-it-self,” encountered in cerebellar disease or MS), or of the LMN type (flabby and nasal speech, e.g., due to neuromuscular disorders such as ALS and MG).
CN XI is tested by asking the patient to press his head against the examiner’s hand (sternocleidomastoid muscle function) and by providing resistance as the patient lifts his shoulders (trapezius muscle function).
Chronic injury to CN XII leads to tongue atrophy and fasciculations. The latter are best seen when the patient relaxes his tongue in his mouth. Also ask the patient to stick out his tongue in order to look for possible deviation (to the paralyzed side). Assess tongue power and mobility by having the patient move his tongue quickly from one side to the other. Dysdiadochokinesia of the tongue is occasionally seen in extrapyramidal diseases and may occur early in the course of PD disease. General tongue weakness due to pseudobulbar or bulbar palsy is revealed by slow side-to-side movements or the patient may not be able to move his tongue at all.
3.3 Motor Function
The examination of motor function includes:
Formal power testing of defined muscle groups. Here the examiner systemically quantifies weakness by offering resistance to the patient’s movements
Observation of spontaneous and unrestrained motor movements. This is usually more sensitive for detection of slight central palsies
For formal power testing of defined muscle groups, the examiner needs to stabilize the proximal and distal limbs of the joint in question to make sure to test well-defined muscle groups, e.g., when testing power of elbow flexion and extension, hold the upper arm with the left hand and the forearm with the right (if the examiner is right-handed). Be sure to use the principle of leverage to your advantage. The more distally on the patient’s extremity force is applied, the better the chances to quantify power in even muscular patients. When testing for shoulder abduction, for example, press the forearm down instead of the upper arm.
Muscle power can be graded according to the British Medical Research Council (MRC) scale:
MRC 0: Lack of any muscle contraction
MRC 1: Visible muscle contraction without movement
MRC 2: Movement with elimination of gravity
MRC 3: Movement against gravity
MRC 4−: Movement against resistance, 25% of normal strength
MRC 4: Movement against resistance, 50% of normal strength
MRC 4+: Movement against resistance, 75% of normal strength
MRC 5: Normal strength
The MRC scale was established for quantification of peripheral palsies secondary to military injuries, but it can also be used, albeit somewhat less reliably, for quantification of other neuromuscular and central palsies.
The abovementioned formal power testing is often inferior to other techniques when it comes to detection of slight central palsies. Observation of asymmetries of unrestrained motor movements may reveal such palsies even in cases when formal power testing does not. Isotonic movements include hand and foot tapping, playing the piano, screwing in light bulbs, alternating fast supination and pronation of the hands, and hopping on one foot. Unilateral decrease of fine motor movement suggests a central palsy. Examine each extremity independently, because simultaneous examination tends to lead to synchronization, which may obscure asymmetric motor performance. Isometric techniques include the straight arm test and the flexed leg test.4 To perform a straight arm test, ask the patient to close his eyes, lift both arms over his head, and extend the elbow and finger joints with the hands supinated. Finger flexion, pronation of the wrist, elbow flexion, or lowering of the arm occurring on one side suggests a contralateral central lesion. Sometimes the patient may only complain that one arm feels heavier than the other, and if consistent with the history, this is the mildest sign of a central palsy. In contrast to the straight arm test, which is easily performed, the flexed leg test demands greater effort on the patient’s part and should therefore be reserved for cases in which the history suggests a mild central paresis of the lower extremity. The supine patient is asked to lift both legs by flexing 90° at the hips, the knees, and the ankles. Asymmetric weakness suggests a central palsy of the affected leg.
If MG or another neuromuscular junction syndrome is suspected, it is advisable to assess fatigability by counting the number of repetitive movements (e.g., knee bending, neck flexion) the patient is able to perform or by counting the seconds the patient is able to maintain certain postures (e.g., lifting his head off the pillow when lying in bed). These are also excellent ways to clinically monitor the effect of medical treatment. Furthermore, the Cogan lid twitch is a relatively specific sign for MG. It occurs due to transient improvement in eyelid strength after rest of the levator in downgaze, followed by droop in the primary position as the levator fatigues. Patients with ptosis and possible MG are asked to look straight ahead, down, and straight ahead again. The eyes are carefully assessed immediately after this movement for the presence of a brief upward twitch of the upper eyelid, indicating a positive test. Other bedside tests for MG include the tensilon and the ice-on-eyes tests, which will be explained in Chap. 4.
After these general considerations, let us review the practical part of the motor examination. Ideally, the examination should begin with the patient undressed and sitting at the bedside in a well-lit room. If the history suggests LMN impairment, check for possible atrophy and fasciculations. The latter may be very difficult to detect unless enough time is reserved to examine each extremity carefully. Fasciculations can sometimes be elicited by slightly tapping the muscle. Then, proceed with a straight arm test to gain an immediate impression of power in the upper extremities.
Subsequently, formal power testing in the upper extremities should be performed. The following movements may be tested routinely (“UMN” denotes movements especially affected by UMN lesions; key myotomes shown in italics):
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