Differentials
Common
Vestibular neuritis/labyrinthitis
History
often preceded by upper respiratory tract infection; acute-onset true vertigo, nausea, vomiting; aggravated by head movement, gradually subsides in several days, mild dizziness and balance difficulty may persist; sensorineural hearing loss in the affected ear in labyrinthitis
Exam
peripheral vestibular nystagmus, a positive head impulse test to the side of peripheral vestibular loss, post-headshake nystagmus, deficits of coordination and gait
1st investigation
- audiometry:
unilateral sensorineural hearing loss in cases of labyrinthitis
- caloric electronystagmography:
caloric responses from the affected ear are reduced compared with the normal ear
More - rotary chair testing:
vestibulo-ocular reflex (VOR) gain may be normal or reduced at lower frequencies of rotation in poorly compensated cases; VOR phase lead and shortening of the VOR time constant to 6 to 12 seconds (normal >20 seconds)
More
Other investigations
- microbiological culture and sensitivity of middle ear effusions if present:
positive if infectious cause
More - serum fluorescent treponemal antibodies:
raised antibodies are positive for syphilis
More - serum, joint fluid, or cerebrospinal fluid antibodies to Borrelia detected by enzyme-linked immunosorbent assay (ELISA):
raised antibodies to Borrelia on ELISA to detect Lyme disease
More - CT temporal bones:
fluid in the mastoid cells or a mass in the middle ear cavity if cholesteatoma is present
More - MRI head:
usually negative in vestibular disease but may show increased signal along the vestibular nerve or the labyrinths on the affected side; abnormal signal in the brainstem or cerebellum may indicate associated stroke and labyrinthine infarction
More
Meniere disease
History
vertigo (at least two spontaneous episodes lasting 20 minutes to 12 hours within a single attack); fluctuating and unilateral aural symptoms (i.e., tinnitus, aural fullness and hearing loss)[8]
Exam
sensorineural hearing loss on Rinne or Weber testing, peripheral vestibular nystagmus is present during the attacks
1st investigation
- audiometry:
unilateral sensorineural hearing loss; usually low-frequency hearing loss is present in early stages and during or before attacks; as disease progresses, middle and high frequencies are affected
More
Other investigations
- electrocochleography:
abnormally large summating potential amplitude relative to the action potential amplitude
- vestibular evoked myogenic potentials (VEMP):
increased amplitude in early disease; attenuated or absent in later stages
More
Vitamin B1 (thiamine) deficiency
History
history of chronic alcoholism, anorexia nervosa, hyperemesis gravidarum, gastrointestinal surgery, systemic malignancy, renal dialysis, or infections such as AIDS; imbalance and ataxic gait; nystagmus, abnormal ocular motility, cognitive impairment
Exam
bilateral or unilateral peripheral vestibular hypofunction features ataxic gait, positive head impulse to both sides, poor dynamic visual acuity, sway on Romberg testing; Wernicke encephalopathy features ataxic gait, nystagmus (usually horizontal gaze evoked to both sides), ocular motility disturbance, cognitive impairment
1st investigation
- erythrocyte thiamine pyrophosphate:
low
Other investigations
- vestibular testing:
reduced caloric responses; reduced vestibulo-ocular reflex (VOR) gain, VOR phase lead, and shortening of the VOR time constant indicating unilateral or bilateral peripheral vestibular hypofunction
- MRI brain:
bilateral increased T2 signal in the paraventricular regions of the thalamus, hypothalamus, mamillary bodies, periaqueductal region, fourth ventricle floor, and midline cerebellum
More
Ototoxic vestibulopathy
History
progressive ataxic gait and oscillopsia shortly following exposure to ototoxic medication; may be accompanied by progressive bilateral hearing loss; balance usually worsens when walking in dim light or with eyes closed
Exam
poor dynamic visual acuity, bilateral positive head impulse tests, ataxic gait, poor tandem walk, sway on Romberg testing
1st investigation
- audiometry:
sensorineural hearing loss may be associated with vestibulopathy from aminoglycoside ototoxicity
Other investigations
- caloric electronystagmography:
Bilaterally reduced or absent caloric responses
More - rotary chair testing:
very low vestibulo-ocular reflex (VOR) gain, marked VOR phase lead, and marked shortening of the VOR time constant to <6 seconds
- blood aminoglycoside levels:
elevated aminoglycoside blood levels or a history of them may be indicative but ototoxicity can also occur with therapeutic levels
Benign paroxysmal positional vertigo (canalithiasis)
History
recurring, brief vertigo precipitated by specific positional change such as tilting the head, lying down, or rolling over in bed, with no associated auditory or neurologic symptoms
Exam
Dix-Hallpike maneuver resulting in vertigo with torsional, vertical nystagmus after a latency of several seconds (a positive result) confirms benign paroxysmal positional vertigo (BPPV) involving the posterior semicircular canal on the tested side; if the Dix-Hallpike maneuver is negative then perform the supine roll test to assess for lateral semicircular canal BPPV[11]
1st investigation
- clinical diagnosis:
vertigo with the appropriate position-provoked nystagmus response on positional maneuvers
Other investigations
Hemorrhagic stroke
History
history of hypertension; acute-onset ataxia and dizziness, headache, vision loss, diplopia, dysarthria, dysphagia, sensory symptoms, and weakness involving the limbs; may progress to altered level of consciousness
Exam
altered level of consciousness, ocular motility disturbance, nystagmus, pupillary abnormalities such as Horner syndrome, dysarthric speech, abnormal gag reflex, sensory loss, pyramidal weakness. Central nrevous system (CNS) findings on HINTS exam (normal vestibular-ocular reflex, direction-changing gaze-evoked nystagmus and vertical correction on alternate eye cover test)
1st investigation
- CT brain:
intracranial hemorrhage
More
Other investigations
Ischemic stroke
History
history of vascular disease; acute-onset ataxia, dizziness, vision loss, diplopia, dysarthria, dysphagia, sensory symptoms, weakness involving the limbs; may progress to altered level of consciousness
Exam
altered level of consciousness, ocular motility disturbance, nystagmus, pupillary abnormalities such as Horner syndrome, dysarthric speech, abnormal gag reflex, sensory loss, pyramidal weakness. Central CNS findings on HINTS exam (normal vestibular-ocular reflex, direction-changing gaze-evoked nystagmus and vertical correction on alternate eye cover test)
1st investigation
- MRI brain:
evidence of infarction
More
Other investigations
- angiography (conventional computed tomography angiography or magnetic resonance angiography):
vertebrobasilar artery stenosis or dissections
Alcohol-related cerebellar degeneration
History
chronic history of alcohol abuse (>10 years); usually gradual onset of imbalance and impaired gait, although sudden onset or acute worsening may occur; tremor, dysarthria, and loss of coordination of arms in a minority of cases only
Exam
signs of midline cerebellar dysfunction: abnormal stance, ataxic gait, inability to perform tandem walk, poor heel-shin testing, mildly abnormal finger-nose testing; postural tremor of arms and dysarthria in a minority of cases; nystagmus, abnormal ocular motility, and cognitive impairment if co-existing Wernicke encephalopathy; vertigo is not a feature
1st investigation
- CT brain:
may show cerebellar hemisphere and vermis atrophy
- MRI brain:
may show cerebellar hemisphere and vermis atrophy
Other investigations
Vestibular migraine
History
diagnostic criteria are based on a history of migraine with at least 5 episodes fulfilling the following criteria: vestibular symptoms of moderate or severe intensity, lasting between 5 minutes and 72 hours, a temporal association between vestibular and migraine symptoms during at least half of the episodes, and exclusion of other causes
Exam
young and middle-aged patients with vertigo, headache, nausea, and low-velocity, sustained, positional nystagmus that dissipates as the patient becomes asymptomatic is highly suggestive of vestibular migraine; spontaneous and head shake nystagmus is seen in some episodes of vestibular migraine
1st investigation
- clinical diagnosis:
low-velocity, sustained nystagmus on positional testing when symptoms are present
More
Other investigations
Idiopathic Parkinson disease
History
falls, slowing of movements, difficulty initiating movements, difficulty standing up from a chair or turning while walking, resting tremor
Exam
bradykinesia, slow shuffling gait, flexed posture, decrease arm swing, postural instability, Myerson sign, mask facies, hypophonic voice, rigid tone with cogwheeling, pill rolling tremor, hypometric saccades; repetitive finger movements are slow and with reduced amplitude
1st investigation
- no initial test:
clinical diagnosis
- dopaminergic agent trial:
improvement in symptoms
More
Other investigations
- functional neuroimaging:
assess integrity of nigrostriatal projections in Parkinson disease
More
Peripheral neuropathy
History
numbness in the lower extremities, paresthesiae, dysesthesias, lower extremity weakness, difficulty with gait, falls, may have history of diabetes mellitus
Exam
loss of proprioception and sensation in the lower extremities, sensory ataxia, lower extremity weakness, difficulty with gait, Rhomberg positive
1st investigation
- nerve conduction studies/electromyography (EMG):
demyelinating neuropathies: dispersion of the compound muscle action potentials, marked slowing of conduction velocity, conduction block, delay in F waves, absent H reflex; axonal neuropathies: compound muscle action potentials with low amplitude, mild slowing of conduction velocity, EMG may show fibrillation potentials and increased insertional activity
More
Other investigations
- chest x-ray:
in selected cases to exclude infection/malignancy
- fasting blood glucose:
may be raised in diabetic peripheral neuropathy
- serum autoantibodies (antinuclear antibodies or antineutrophil cytoplasmic antibodies):
in selected cases to exclude collagen vascular disease
- serum vitamin B12 and folate levels:
in selected cases to exclude subacute combined degeneration of the spinal cord and folate deficiency
- genetic testing for Charcot-Marie-Tooth disease type 1:
in selected cases to exclude hereditary peripheral neuropathy
Uncommon
Vestibular schwannoma (acoustic neuroma)
History
unilateral hearing loss, dizziness, balance difficulty; with larger tumors there may be sensory symptoms over the face or facial weakness on the affected side; may occur sporadically or in bilateral cases as part of von Recklinghausen neurofibromatosis
Exam
sensorineural hearing loss on the affected side, chronic unilateral peripheral vestibular loss, a positive head-impulse test, post-headshake nystagmus, poor dynamic visual acuity; nonspecific deficits in balance, gait; tandem walk; Romberg sway
1st investigation
Other investigations
- caloric electronystagmography:
caloric responses from the affected ear are reduced compared with the normal ear
More - rotary chair testing:
vestibulo-ocular reflex (VOR) gain may be normal or reduced at lower frequencies of rotation in poorly compensated cases; VOR phase lead and shortening of the VOR time constant to 6-12 seconds (normal >20 seconds)
More - surgical excision:
histopathologic diagnosis of the tumor
More
Sequential bilateral vestibular neuritis
History
remote episode of vertigo with complete resolution of symptoms followed by a more recent episode of dizziness, resulting in persisting disequilibrium and imbalance; the first episode results in peripheral vestibular loss that is compensated leading to resolution of symptoms, whereas the second episode results in peripheral vestibular loss involving the fellow ear; oscillopsia or blurring of vision with head movement may result from a deficiency vestibulo-ocular reflex
Exam
poor dynamic visual acuity, bilateral positive head impulse tests, ataxic gait, poor tandem walk, sway on Romberg testing
1st investigation
- caloric electronystagmography:
Bilaterally reduced caloric responses
More - rotary chair testing:
very low VOR gain, marked VOR phase lead, and marked shortening of the VOR time constant to <6 seconds
Other investigations
- serum fluorescent treponemal antibodies:
raised antibodies are positive for syphilis
More - serum, joint fluid, or cerebrospinal fluid antibodies to Borrelia detected by enzyme-linked immunosorbent assay (ELISA):
raised antibodies to Borrelia on ELISA to detect Lyme disease
More - MRI brain:
exclude brainstem/cerebellar infarcts
More
Idiopathic bilateral vestibular hypofunction
History
progressive ataxic gait and oscillopsia accompanied by progressive bilateral hearing loss; balance usually worsens when walking in dim light or with eyes closed; no history of prior inner ear disease such as vestibular neuritis to explain the peripheral vestibular loss
Exam
poor dynamic visual acuity, bilateral positive head impulse tests, ataxic gait, poor tandem walk, sway on Romberg testing
1st investigation
- audiometry:
may show co-existing bilateral sensorineural hearing loss
- caloric electronystagmography:
Bilaterally reduced caloric responses
More - rotary chair testing:
very low vestibulo-ocular reflex (VOR) gain, marked VOR phase lead, and marked shortening of the VOR time constant to <6 seconds
Other investigations
- MRI brain:
exclude bilateral vestibular schwannomas
- serum fluorescent treponemal antibodies:
raised antibodies are positive for syphilis
More - serum, joint fluid, or cerebrospinal fluid antibodies to Borrelia detected by enzyme-linked immunosorbent assay (ELISA):
raised antibodies to Borrelia on ELISA to detect Lyme disease
More
Congenital bilateral vestibular hypofunction
History
ataxic gait and oscillopsia that may be accompanied by congenital bilateral hearing loss; balance usually worsens when walking in dim light or with eyes closed; symptoms and signs are milder than in acquired cases of bilateral peripheral vestibular loss; may present as “always having been a little off balance
Exam
poor dynamic visual acuity, bilateral positive head impulse tests, gait may be normal, poor tandem walk, sway on Romberg testing
1st investigation
- audiometry:
may show co-existing bilateral sensorineural hearing loss
- caloric electronystagmography:
Bilaterally reduced caloric responses
More - rotary chair testing:
very low vestibulo-ocular reflex (VOR) gain, marked VOR phase lead, and marked shortening of the VOR time constant to <6 seconds
Other investigations
- MRI brain:
exclude bilateral vestibular schwannomas
- serum fluorescent treponemal antibodies:
raised antibodies are positive for syphilis
More - serum, joint fluid, or cerebrospinal fluid antibodies to Borrelia detected by enzyme-linked immunosorbent assay (ELISA):
raised antibodies to Borrelia on ELISA to detect Lyme disease
More
Degenerative (age-related) inner ear disease
History
chronic slowly progressive ataxic gait and oscillopsia, which may be accompanied by progressive bilateral hearing loss (presbycusis); balance usually worsens when walking in dim light or with eyes closed
Exam
poor dynamic visual acuity, bilateral positive head impulse tests, ataxic gait, poor tandem walk, sway on Romberg testing; may rarely be accompanied by signs of cerebellar dysfunction[19]
1st investigation
- audiometry:
usually shows associated bilateral high frequency sloping sensorineural hearing loss typical of presbycusis
- caloric electronystagmography:
Bilaterally reduced caloric responses
More - rotary chair testing:
very low vestibulo-ocular reflex (VOR) gain, marked VOR phase lead, and marked shortening of the VOR time constant to <6 seconds
Other investigations
- MRI brain:
exclude bilateral vestibular schwannomas
- serum fluorescent treponemal antibodies:
raised antibodies are positive for syphilis
More - serum, joint fluid, or cerebrospinal fluid antibodies to Borrelia detected by enzyme-linked immunosorbent assay (ELISA):
raised antibodies to Borrelia on ELISA to detect Lyme disease
More
Autoimmune inner ear disease
History
progressive bilateral sensorineural hearing loss and dizziness that may be similar to bilateral simultaneous Meniere disease, corneal involvement (eye pain and photophobia), and systemic symptoms resulting from vasculitis; may occur with Cogan syndrome and may be associated with another autoimmune disease
Exam
bilateral hearing loss, peripheral vestibular nystagmus, slit lamp exam is essential to exclude interstitial keratitis (Cogan syndrome)
1st investigation
- audiometry:
rapidly progressive fluctuating bilateral sensorineural hearing loss
- CBC:
may show anemia
- erythrocyte sedimentation rate and CRP:
elevated
- antinuclear antibodies, rheumatoid factor, antineutrophil cytoplasmic antibodies, C1Q immune complex:
elevated
- vestibular testing:
bilateral peripheral vestibular loss
Other investigations
- serum fluorescent treponemal antibodies:
exclude infectious cause
- serum, joint fluid, or cerebrospinal fluid antibodies to Borrelia detected by enzyme-linked immunosorbent assay (ELISA):
raised antibodies to Borrelia on ELISA to detect Lyme disease
More - MRI brain:
exclude bilateral vestibular schwannomas
- antithyroid (thyroglobulin and microsomal) antibodies:
elevated in cases associated with thyroid disease
- anti-HSP70 antibodies:
may be elevated
More - antigliadin and antiendomysial antibodies:
elevated in celiac disease
Superior canal dehiscence (SCD)
History
brief dizziness precipitated by coughing, sneezing, straining, or sudden loud noise; autophony; feeling that the affected ear is blocked
Exam
dizziness and nystagmus can be precipitated by fistula test, tragal compression, Valsalva, or loud noise (Tullio phenomenon)
1st investigation
Other investigations
- audiometry:
bone conduction scores are better than air conduction (conductive hyperacusis)
More - tympanometry:
may induce dizziness
More - fistula test:
Valsalva or the fistula test of the affected ear precipitate brief dizziness associated with nystagmus
More - MRI temporal bones:
may exclude other unilateral diseases affecting the middle ear such as tumor
More
Meningitis
History
bacterial meningitis presents acutely with fever, altered mental state, and headache, dizziness, balance difficulty, and hearing loss and may follow syphilis, Lyme disease, or fungal infection
Exam
fever, nuchal rigidity, and altered mental status; poor dynamic visual acuity, positive head impulse tests; unsteady gait, poor tandem walk, sway on Romberg testing
1st investigation
- cerebrospinal fluid (CSF) examination:
raised CSF pressure; lymphocytosis or polymorphonuclear leukocytes; raised protein in bacterial infection; specific pathogen isolated
More
Other investigations
- caloric electronystagmography:
caloric responses may be reduced in one or both ears
- rotary chair testing:
low vestibulo-ocular reflex (VOR) gain, VOR phase lead, and shortening of the time constant with peripheral vestibular loss
- audiometry:
sensorineural hearing loss may be present
Mal de debarquement syndrome
History
persisting disequilibrium with onset after an ocean cruise or other prolonged travel;[22] disequilibrium is most severe when sitting still or lying down
Exam
examination is normal or shows nonspecific deficits in balance (e.g., difficulty with tandem walk)
1st investigation
- no testing required:
clinical diagnosis
Other investigations
Spinocerebellar ataxia
History
usually begins in childhood; progressive ataxia, motor difficulty, tremor, cognitive decline, vision loss, cognitive dysfunction, seizures, myoclonus, dysarthria, sensory symptoms, vomiting, and gastrointestinal pain depending on the genetic type
Exam
nystagmus, gaze-evoked nystagmus, slow saccades, upper motor neuron signs, macular degeneration, tremor, myoclonus, and cognitive impairment[18]
1st investigation
- genetic testing:
mutation detected
More
Other investigations
Multiple sclerosis (MS)
History
variable presentation: multiple episodes separated by space (i.e., neurological symptoms result from lesions in different central nervous system sites) and time; painful unilateral visual loss, sensory disturbances, progressive limb weakness, gait difficulty, ataxia, loss of balance, paroxysmal vertigo
Exam
coexisting signs of brainstem/cerebellar dysfunction or of myelopathy, which suggests a multifocal disease
1st investigation
- MRI brain and spinal cord:
hyperintensities in the periventricular white matter; areas of demyelination in the spinal cord
More
Other investigations
- lumbar puncture with cerebrospinal fluid examination:
glucose and protein should be normal; cell count may be slightly elevated but never above 50/microliter; oligoclonal bands and elevated CSF IgG usually present
More
Cerebellitis/brainstem encephalitis
History
headache, acute vertigo, ataxia, nausea, vomiting; may present as a post-infectious disorder, as a paraneoplastic syndrome, or as part of an inflammatory disorder[16]
Exam
gaze-evoked nystagmus, downbeat nystagmus, rebound nystagmus, saccadic dysmetria, ocular flutter, dysarthria/scanning speech, limb dysmetria, wide-based ataxic gait, signs of raised intracranial pressure
1st investigation
- cerebrospinal fluid (CSF) protein, glucose, and white cell count:
mildly elevated protein (60 to 80 mg/dL), normal glucose, and a moderate mononuclear cell leukocytosis (up to 1000 leukocytes/microL)
More - CSF virology:
findings from CSF cultures for enteroviruses, mumps, and certain arboviruses may be positive
More
Other investigations
- CSF immunoglobulin detection by enzyme-linked immunosorbent assay:
raised IgM detection early in viral infection with West Nile virus and IgG in dengue
More - CT brain:
edema, hemorrhages, low-density viral lesions, hydrocephalus, and herniation
- MRI brain:
encephalitis results in cerebral edema, which can readily be seen on MRI as increased signal
More - electroencephalogram:
may show diffuse activity in some of the viral encephalitides
Wernicke encephalopathy
History
bilateral vestibulopathy, nystagmus, ophthalmoparesis, ataxia, change in mental status, history of alcohol abuse or of protracted vomiting (e.g., hyperemesis gravidarum)
Exam
confusion, memory difficulty, confabulation, ophthalmoplegia, nystagmus, positive head impulse tests, ataxic gait, poor tandem walk, sway on Romberg testing
1st investigation
- MRI brain:
increased density of the mammillary bodies and periaqueductal grey matter
More
Posterior fossa tumor
History
disequilibrium, ataxic gait, limb ataxia, dysarthria, oscillopsia, headache
Exam
gaze-evoked nystagmus, downbeat nystagmus, rebound nystagmus, saccadic dysmetria, ocular flutter, dysarthria/scanning speech, limb dysmetria, wide-based ataxic gait, sway on Romberg testing
1st investigation
- MRI brain:
tumor visualized
Other investigations
- brain biopsy/surgical excision:
brain smear, biopsy, or surgical excision allows for histopathologic diagnosis
Chiari malformation
History
balance difficulty and positional dizziness
Exam
downbeat nystagmus, most prominent on lateral gaze; positional testing may precipitate dizziness with downbeat nystagmus
1st investigation
- MRI brain/spine:
craniocervical junction lesion
Other investigations
Central pontine myelinosis
History
occurs in the setting of rapid correction of hyponatremia; acute paraplegia or quadriplegia associated with diplopia, dysarthria, and dysphagia; may progress to locked-in syndrome
Exam
abnormal ocular motility especially involving horizontal eye movements, progressive paralysis, dysarthric speech, loss of consciousness
1st investigation
- MRI brain:
abnormal signal in the pons on T2 images
Other investigations
Paraneoplastic syndrome
History
symptoms may be rapid or slowly progressive, ataxia with loss of coordination of limbs and dysarthria; commonly associated malignancies include small cell lung cancer, breast cancer, ovarian cancer, uterine cancer, and Hodgkin lymphoma
Exam
vestibulo-cerebellar dysfunction resulting in progressive ataxic gait, marked truncal ataxia, vertical nystagmus, and dysarthria
1st investigation
- anti-Yo antibodies:
positive in cerebellar degeneration
- anti-Ri antibodies:
positive in cerebellar ataxias
- anti-Ma antibodies:
positive in cerebellar or brainstem dysfunction
Other investigations
Vitamin E deficiency
History
progressive gait difficulty, ataxia associated with weakness and visual field loss, dementia, cardiac arrhythmias; consider in people with cystic fibrosis, gastrointestinal disease, and abetalipoproteinemia
Exam
ataxia, hyporeflexia, visual field constriction, upgaze palsy, limb dysmetria, muscle weakness, diminished proprioception, pigmentary retinopathy
1st investigation
- serum alpha-tocopherol level:
decreased
Other investigations
Celiac disease
History
variable gastrointestinal symptoms (diarrhea, bloating, abdominal discomfort, weight loss), fatigue; neurological complications of celiac disease include ataxia, peripheral neuropathy, dementia, and seizures
Exam
anemia, dermatitis herpetiformis (pruritic papulovesicular lesions over the extensor surfaces of the arms and legs), ataxia, hyporeflexia, limb dysmetria, muscle weakness, diminished proprioception
1st investigation
- immunoglobulin A-tissue transglutaminase titer:
elevated
- endomysial antibody:
elevated
Other investigations
- small bowel biopsy and histologic examination:
villous atrophy on histology
Primary cerebellar degeneration
History
typically late-onset chronic progressive ataxic gait with earlier onset in familial cases; may, rarely, be accompanied by bilateral peripheral vestibular hypofunction
Exam
wide-based ataxic gait, downbeat nystagmus, gaze-evoked nystagmus, saccadic pursuit, dysarthria, limb dysmetria, and dysdiadochokinesia, bilateral head impulse tests with poor dynamic visual acuity if co-existing bilateral peripheral vestibular loss
1st investigation
- CT or MRI brain:
may show cerebellar atrophy
More
Other investigations
- serum methylmalonic acid and homocysteine:
elevated levels
More - anti-Yo antibodies:
positive in cerebellar degeneration
More - anti-Ri antibodies:
positive in cerebellar ataxias
More - anti-Ma antibodies:
positive in cerebellar or brainstem dysfunction
More - serum alpha-tocopherol:
decreased level
More - serum HIV enzyme-linked immunosorbent assay:
positive in HIV infection
More
Secondary parkinsonism
History
acute or subacute onset, secondary to stroke or drug, focal weakness, sensory symptoms, cognitive decline
Exam
bradykinesia, slow shuffling gait, flexed posture, decrease arm swing, postural instability, Myerson sign, mask facies, hypophonic voice, rigid tone with cogwheeling, pill rolling tremor, hypometric saccades; repetitive finger movements are slow and with reduced amplitude
1st investigation
- CT or MRI brain:
may detect lesion or infarct
More
Other investigations
Normal pressure hydrocephalus
History
progressive cognitive decline with gait difficulty and urinary incontinence
Exam
gait may be ataxic or may have magnetic gait, cognitive decline, parkinsonian features
1st investigation
- MRI brain:
enlargement of ventricles associated with transependymal edema
Other investigations
Corticobasal degeneration
History
progressive gait difficulty associated with motor dysfunction and alien hand syndrome
Exam
may have signs of parkinsonism associated with dystonia, myoclonus, alien hand syndrome, and cognitive decline including involvement of language function
1st investigation
- MRI brain:
asymmetric frontoparietal atrophy
Other investigations
Progressive supranuclear palsy
History
progressive gait difficulty with speech and swallowing affected; parkinsonism with early falls
Exam
vertical gaze palsy, pseudobulbar palsy, early postural instability
1st investigation
- MRI brain:
sagittal views may show midbrain atrophy resulting in morphology resembling a hummingbird; midbrain/pons ratio may be reduced
Other investigations
Multiple system atrophy (MSA; striatonigral degeneration, Shy-Drager syndrome)
History
rigidity, bradykinesia, tremor, gait difficulty, poor response to levodopa, erectile dysfunction, urinary symptoms, coordination difficulty; postural hypotension is seen in Shy-Drager syndrome
Exam
rigidity, bradykinesias, hypophonic voice, slow shuffling gait, tremor, myoclonus, orthostatic hypotension, ocular dysmetria, gaze evoked nystagmus, saccadic pursuit, ataxic gait; limb ataxia from cerebellar involvement
1st investigation
- no testing required:
clinical diagnosis
More
Other investigations
Spinal cord trauma
History
acute limb weakness, sensory loss, and sphincter dysfunction following trauma
Exam
upper motor neuron signs (corticospinal tract lesions), lower motor neuron signs (cervical or lumbar spine lesions): muscle atrophy, muscle fasciculations, hyporeflexia, muscle weakness; sensory signs: loss of joint position sense (posterior column lesions), loss of temperature and pinprick sensation (spinothalamic tract lesions), loss of reflexes
1st investigation
Other investigations
Guillain-Barre syndrome (GBS)
History
acute progressive balance difficulty, ascending weakness and areflexia, numbness and paresthesias of extremities, ataxic gait, limb weakness, constipation, bladder symptoms, orthostatic lightheadedness; may have diplopia with the Miller Fisher variant; history of upper respiratory infection or diarrhea preceding onset of symptoms supports diagnosis of GBS
Exam
muscle atrophy and fasciculations, muscle weakness, hyporeflexia or areflexia, ataxia, ophthalmoplegia, pupillary dilation with the Miller Fisher variant
1st investigation
- nerve conduction studies/electromyography (EMG):
demyelinating neuropathies: dispersion of the compound muscle action potentials, marked slowing of conduction velocity, conduction block, delay in F waves, absent H reflex; axonal neuropathies: compound muscle action potentials with low amplitude, mild slowing of conduction velocity, EMG may show fibrillation potentials and increased insertional activity; Miller Fisher syndrome: absent or reduced sensory responses[60]
More
Spinal spondylosis
History
weakness, numbness, paresthesias, gait and coordination difficulty, neck pain and stiffness
Exam
limited range of motion of neck, signs of myelopathy, pyramidal distribution weakness, spastic tone, hyperreflexia, Hoffmann sign, extensor plantar responses, loss of joint position sense in the lower extremities, unsteady gait
1st investigation
- MRI cervical spine:
spondylosis and spinal stenosis with myelopathy (abnormal cord signal)
Other investigations
Spinal cord tumor
History
progressive myelopathy that may be associated with back pain
Exam
signs of myelopathy and or radiculopathy
1st investigation
- MRI spine:
mass and spinal cord involvement
Other investigations
- chest x-ray:
lung mass if lung cancer is the primary
- mammogram:
breast mass if breast cancer is the primary
- prostate-specific antigen:
elevated if prostate cancer is the primary
Neuromyelitis optica (NMO)
History
history of MS, ascending sensory symptoms to trunk, sphincter disturbance, vision loss from optic neuritis (ON), diplopia, dysarthria and coordination difficulty due to cerebellar involvement, fatigue and cognitive difficulty
Exam
spastic muscle tone, hyperreflexia, pyramidal distribution weakness, extensor plantar responses, sensory loss, sway on Romberg testing, loss of anal sphincter tone, vision loss, cerebellar dysfunction, internuclear ophthalmoplegia, nystagmus, monocular vision loss
1st investigation
- MRI brain:
swelling of optic nerve; enhancement in optic nerve; white matter lesions in patients with MS or at risk of MS
Subacute combined degeneration of the cord
History
gait difficulty associated with weakness and numbness of limbs, Lhermitte symptom from posterior column involvement in the cervical cord, optic neuropathy, cognitive decline associated with pernicious anemia, gastric bypass surgery, nutritional deficiency, Crohn disease, Whipple disease, Zollinger-Ellison syndrome
Exam
pyramidal weakness, hyperreflexia, extensor plantar responses, loss of proprioception in limbs
1st investigation
- serum methylmalonic acid and homocysteine levels:
elevated levels are diagnostic of vitamin B12 deficiency[66]
Other investigations
- blood film:
macrocytic anemia and hypersegmented neutrophils
- serum vitamin B12:
usually reduced but may be normal in deficiency states
Syphilis, tabes dorsalis
History
history of syphilis infection, lancinating pain, or gastric crisis
Exam
Argyll Robertson pupils
1st investigation
- serum fluorescent treponemal antibody absorption (FTA-ABS) and microhemagglutination treponema pallidum (MHA-TP):
reactive
More
Other investigations
- cerebrospinal fluid (CSF) cytology:
may be normal or show mild lymphocytic pleocytosis (10 to 50 cells/microL) with increased protein (45 to 75 mg/dL or higher)
- CSF serology:
CSF VDRL may be nonreactive in up to 25% of cases; CSF FTA-ABS is reactive in neurosyphilis
More
Tuberculosis of the spine (Pott disease)
History
myelopathy with back pain, history of tuberculosis (TB) infection
Exam
may have signs of myelopathy (upper motor neuron and sensory loss to sensory level on trunk)
1st investigation
- chest x-ray:
consolidation, pulmonary infiltrates, mediastinal or hilar lymphadenopathy, upper zone fibrosis
More - MRI spine:
compressive spinal lesions
- sputum acid-fast bacilli smear and culture:
presence of acid-fast bacilli (Ziehl-Neelsen stain) in specimen; testing of three specimens (minimum 8 hours apart, including an early morning specimen) is recommended in many countries; consult local guidance[69]
More - acid-fast bacilli smear and culture of extrapulmonary biopsy specimen:
positive
More - nucleic acid amplification tests (NAAT):
positive for M tuberculosis
More
Other investigations
- lateral flow urine lipoarabinomannan (LF-LAM) assay:
positive
More
HIV infectious myelopathy
History
history of HIV infection; progressive spastic weakness with sensory symptoms and sphincter dysfunction
Exam
weakness, sensory deficits, sphincter dysfunction, signs of myelopathy without distinct sensory level
1st investigation
- serum HIV enzyme-linked immunosorbent assay:
positive in HIV infection
Other investigations
- MRI spine:
non-enhancing high signal areas present on multiple contiguous slices, which can involve posterior columns or be diffuse
Human T-lymphotropic virus (HTLV)-1 infectious myelopathy
History
progressive spastic weakness with sensory symptoms and sphincter dysfunction; affects adults from equatorial areas of the world; myelopathy may be associated with uveitis, arthritis, polymyositis, keratoconjunctivitis, and pulmonary lymphocytic alveolitis
Exam
uveitis, keratoconjunctivitis, joint swelling
1st investigation
- cerebrospinal fluid (CSF) polymerase chain reaction for HTLV-1 antigen:
positive
More
Other investigations
Systemic lupus erythematosus (SLE) inflammatory myelopathy
History
history of SLE, malar rash, vasculitis, myocarditis
Exam
spastic paraparesis, sphincter disturbance, and sensory loss (acute transverse myelitis)
1st investigation
- antinuclear antibodies (ANA):
positive
More
Other investigations
- anti-dsDNA and anti-Smith antibodies:
elevated titre
More - MRI spine:
may demonstrate intramedullary spinal lesions
Anxiety disorder
History
anxiety, dizziness or faintness, palpitations, sweating, trembling or shaking, fear of dying, paresthesiae, chills or hot flushes, breathlessness or choking sensation
Exam
hyperventilation, rapid pulse rate, exam otherwise normal
1st investigation
Other investigations
- ECG:
normal
- chest x-ray:
normal
Drug toxicity
History
dizziness, faintness, deafness, nausea, history of medication for anxiety, epilepsy, ischemic heart disease, hypertension, malignancy
Exam
common drugs include aminoglycosides, methotrexate, cisplatin, tranquilizers, barbiturates, antihistamines, antihypertensives, diuretics, amiodarone
1st investigation
Other investigations
- urine drug toxicity screen:
raised levels of drugs and their metabolites
- blood drug toxicity screen:
raised levels of drugs and their metabolites
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