Evaluation of ataxia

subacute or chronic evolution of symptoms, history of alcohol dependence

gait ataxia with broad-based gait with nystagmus and dysarthria are infrequent

acute onset of symptoms, presence of risk factors, headache, nausea, vomiting, imbalance

poor standing and walking ability, neck stiffness

acute onset of symptoms, imbalance, speech and swallowing difficulties, numbness and weakness of limbs, obtundation

ataxia may be one-sided, cranial nerve palsies, direction-changing nystagmus, subtle skew deviation on "cover" test, paralysis, decreased consciousness

acute onset of symptoms, headache, nausea, vomiting, imbalance

poor standing and walking ability, neck stiffness

acute onset of symptoms, common in adults, relapsing and remitting course, many central nervous system structures affected (e.g., vision, spinal cord, eye movements)

optic nerve dysfunction, upper motor neuron signs (e.g., brisk tendon reflexes and Babinski sign), ocular palsy, sensory loss

acute onset of symptoms, confusion, oculomotor palsy, history of alcohol dependence or nutritional deficiencies

confusion, sixth-nerve palsies, nystagmus

acute onset of symptoms, common in children, ataxia in temporal relation with administration of cerebellotoxic drugs (e.g., phenytoin, carbamazepine, oxcarbazepine, vigabatrin, gabapentin, phenobarbital, ketamine, fluorouracil, cytarabine, intrathecal methotrexate, procarbazine, epothilones, vincristine, capecitabine, lithium, amiodarone, cyclosporine and bismuth salts)

nystagmus

gradual onset of symptoms, exposure to neurotoxic drug (e.g., platinum drugs, large doses of vitamin B6), imbalance, numbness

gait ataxia, loss of deep tendon reflexes, loss of sensation in limbs

imbalance; characterized by multiple tumors such as renal cell carcinoma, pheochromocytoma, retinal hemangioblastoma, and endolymphatic sac tumor

retinal lesions

history of recovery from hypoxic episode such as cardiac arrest or significant heat stroke

intention myoclonus

common in children, acute onset of symptoms, imbalance; due to either acute meningoencephalitis or postinfectious syndrome several weeks after a viral illness or immunization

gait ataxia, appendicular ataxia, dysarthria, titubation, nystagmus; mutism is rare

HIV positive, symptom evolution over a few months

cognitive impairment on screening questionnaires

family history (autosomal dominant), relatively rapid symptom evolution over months to 1 to 2 years

mild cognitive impairment on screening questionnaires, brisk reflexes, myoclonus

symptom evolution over a few months

cognitive impairment on screening questionnaires, myoclonus

symptom evolution over few days to weeks, headache, vomiting

asymmetric ataxia, signs of middle ear infection

ataxia is part of a broader picture of memory loss and seizures

gaze palsy, memory impairment, unusual oculomasticatory movement

rash (often on palms or soles), headache, meningism, blurred vision, photophobia, reduced color perception, seizures, or cognitive dysfunction, in late tertiary syphilis: personality change, gait impairment, incontinence

genital chancre or oral mucous patch, rash, uveitis, hyporeflexia, ataxia, anisocoria, Argyll Robertson pupils, cranial neuropathy, motor or sensory deficits, dementia, paranoia

subacute or chronic balance problems, headache, nausea

gait and limb ataxia, nystagmus, cranial nerve deficits such as facial palsy, upper motor neuron signs (hyperreflexia, increased tone)

gradual neurologic problems including pyramidal weakness

gait and limb ataxia, downbeat nystagmus, upper motor neuron signs (hyperreflexia, spasticity, weakness), lower cranial nerve palsies

subacute evolution of symptoms (weeks to months), numbness in hands and feet (often asymmetric), onset in upper limbs not uncommon; ask about weight loss, tobacco history

gait and limb ataxia, asymmetric or symmetric loss of position and vibration sense, loss of deep tendon reflexes

gradually progressive, often associated with other autoimmune disorders (e.g., diabetes, thyroid dysfunction), more common in women

may be associated with oculomotor problems, stiff lower limbs

neuropathy, may be associated with gastrointestinal symptoms related to celiac disease

gait and limb ataxia, peripheral neuropathy

rapid or subacute onset of symptoms, history of neuroblastoma

myoclonic jerks, chaotic eye movements of involuntary nature

rapidly progressive severe ataxia over a few weeks or months, nausea, vomiting, known triggering malignancy in some but in others ataxia occurs before diagnosis of cancer

severe ataxia of gait and limbs, dysarthria, nystagmus

acute onset of symptoms, imbalance, trouble moving eyes over a few days

oculomotor paralysis, absent tendon reflexes, may reveal or develop other signs of generalized paralysis including dysphagia and respiratory weakness

gradual evolution of symptoms, numbness and paresthesias in hands and legs (often asymmetric), dry eyes and mouth

gait and limb ataxia, loss of deep tendon reflexes, loss of vibration and position sense

numbness

gait and limb ataxia, loss of deep tendon reflexes, loss of vibration and position sense

symptoms of hypothyroidism (e.g., weight gain, fatigue, dry skin)

mild gait ataxia

symptoms of hypoparathyroidism (e.g., muscle twitches or spasms, cramps, numbness or tingling, confusion), recent history of neck surgery

tetany, Trousseau sign (carpal spasm with occlusion), cerebellar ataxia

neuropathy, often history of alcohol dependence

midline ataxia, gait ataxia

imbalance, sensory loss in lower limbs

loss of vibration and position sense, pyramidal signs, positive Romberg test

mean age at onset 10 to 15 years, may have onset in adult life, history of diabetes, visual and hearing dysfunction, restless leg syndrome, or hypertrophic cardiomyopathy

gait ataxia, scoliosis, scanning dysarthria, loss of vibration and position sense, loss of tendon reflexes, square wave jerks of eyes, later weakness of lower limbs and extensor plantars, some may have retained or brisk deep tendon reflexes, pes cavus may be seen

onset of postural instability early in first decade of life and then progressive ataxia

gait and limb ataxia; truncal instability; titubation; absent tendon reflexes; choreic movements; poor initiation of ocular saccades (oculomotor apraxia); telangiectasia over conjunctivae, ear lobes, and popliteal fossa

progressive ataxia starting at <10 years of age, occasionally later

oculomotor apraxia very common, chorea

progressive ataxia starting in childhood (mean 15 years of age), occasionally later

oculomotor apraxia, chorea

progressive ataxia from childhood, occasionally later

loss of tendon reflexes, head tremor, retinopathy

early childhood onset of intestinal malabsorption

loss of tendon reflexes and sensation

ataxia and spasticity starting in first decade

spasticity, muscle atrophy, myelinated fibers in optic nerve (not universal)

childhood onset, common in Finnish population

progressive cerebellar signs (e.g., ataxia, nystagmus), myoclonus, neuropathy, tremor

recurrent episodes of acute liver failure in early infancy

ataxia, peripheral neuropathy

childhood onset, cognitive changes

mild decline in IQ

childhood onset (rarely young adult onset), ataxia often overshadowed by other neurologic problems such as cognitive decline, seizures, and encephalopathy, ataxia is often intermittent

ataxia with many other neurologic signs depending on specific disease (e.g., tendon xanthoma in cerebrotendinous xanthomatosis)

age at onset is childhood to >60 years, but mean is early 30s; gradual progression of symptoms, incoordination, speech problems

nystagmus, dysarthria, gait problems, upper motor neuron signs, such as spasticity and brisk tendon reflexes, hypermetric saccades, peripheral neuropathy

mean onset age is early 30s; positive family history, progressive ataxia, speech problems

dysarthria, early occurrence of very slow saccades, tendon reflexes often completely absent, parkinsonism, and myoclonia

mean onset age is early 30s; progressive ataxia, diplopia, stiffness

dysarthria; nystagmus; upper motor neuron signs such as spasticity, brisk tendon reflexes, and Babinski sign; extrapyramidal signs may dominate in some patients (akinetic-rigid syndrome, dystonia); slow saccades later in course with ophthalmoparesis; older-onset patients have ataxia and peripheral neuropathy

slow progression of ataxia

nystagmus (downbeat sometimes), gait and limb ataxia, dysarthria, upper motor neuron signs such as brisk deep tendon reflexes

mean onset age is 40s; slowly progressive ataxia with relatively normal lifespan, diplopia and other visual complaints

nystagmus (often downbeat), gait and limb ataxia, abnormal pursuit and inaccurate saccadic eye movements, saccade velocity relatively normal, noncerebellar signs minimal and often incidental

age at onset very variable, from childhood to older adult

prominent spasticity, maculopathy with depigmentation, low visual acuity; childhood onset can result in more rapid course with added cognitive changes and seizures

upper limb incoordination, family history may be negative

gait and limb ataxia, nystagmus, dysarthria, some upper motor neuron signs, such as brisk tendon reflexes, decreased vibratory sense, cognitive deficits

seizures

nystagmus, gait and limb ataxia, dysarthria, seizures, and cognitive dysfunction

progressive symptoms

gait and limb ataxia, nystagmus, occasional upper motor neuron signs such as brisk deep tendon reflexes, dystonia

cognitive and psychiatric symptoms, tremor

postural tremor, extrapyramidal signs, rigidity, dementia, action tremor, and parkinsonian features

childhood or adult onset, very slow progression, cognitive subnormality in some

mild intellectual disability (although not universal)

adult onset of symptoms

myoclonus, facial myokymia, tremor, mild sensory loss, and cognitive impairment

slowly progressive ataxia

ataxia and head tremor

imbalance, cognitive decline, psychiatric symptoms, positive family history more common, although there may be sporadic cases

ataxia and rigidity, dementia, dystonia

progressive ataxia

ataxia and nystagmus, hyporeflexia, dysarthria, sensorimotor neuropathy

progressive ataxia

ataxia and cognitive impairment, myoclonus (rapid jerking movement of muscle), tremor, hyperreflexia

progressive ataxia often beginning with speech problem

gait and limb ataxia, dysarthria, spasmodic dysphonia; diagnosis based on clinical findings and neuroimaging

progressive ataxia

ataxia and rigidity, bradykinesia, hyporeflexia, cognitive impairment

progressive ataxia

ataxia

progressive ataxia

ataxia and sensory neuropathy

progressive ataxia

isolated ataxia

progressive ataxia

facial dyskinesia, early onset tremor, sensory neuropathy, developmental delay

progressive ataxia in young adulthood

gait and limb ataxia, dysarthria, hyperreflexia, nystagmus, ophthalmoparesis, ptosis

progressive ataxia

pure ataxia (no other signs apart from ataxia)

progressive ataxia

pure ataxia (no other signs apart from ataxia)

progressive ataxia, hearing loss

ataxia and sensorineural hearing loss

progressive ataxia, infertility due to azoospermia in men

ataxia and cognitive impairment

progressive ataxia, skin changes in childhood

ataxia and hyporeflexia, skin changes that disappear in adulthood (e.g., papulosquamous, ichthyosiform plaques on the limbs)

progressive ataxia

ataxia and pseudobulbar palsy, tremor, hyperreflexia, torticollis

progressive ataxia after age 50 years

truncal ataxia, ataxic dysarthria, hyperreflexia, lower motor neuron disease (both lower motor neuron and cerebellar disease)

progressive ataxia

ataxia and abnormal vertical eye movements

progressive ataxia, starting in adulthood

ataxia and sensory axonal neuropathy

ataxia starting in adulthood

ataxia and spasticity and brisk reflexes (upper motor neuron signs)

cognitive decline, myoclonic epilepsy, dementia superficially resembling Huntington disease

chorea

onset in childhood, brief episodes (minutes) of imbalance

skeletal muscle myokymia may be seen in between episodes

brief episodes of ataxia (hours), nausea, vomiting, migraine-like headache, diplopia with onset usually in teen years

episodic dysarthria and nystagmus; some may have nystagmus and mild gait difficulties between episodes

usually occurs in older males, often family history of fragile-X intellectual impairment in a grandson, rarely in a female with a fragile-X syndrome in a son, family history may not be present, imbalance

tremor, autonomic deficits, dysarthria

coexistent myopathy, hearing loss, cardiomyopathy

retinal pigmentary degeneration, hearing loss, muscle weakness

manifestations vary according to age: clumsiness, speech or developmental delay, cataplexy (in late infantile form); seizures, cataplexy (in juvenile form); dementia (in adult form)

gait ataxia (in late infantile form); dystonia (in adult form); myoclonus or myoclonic tremor, abnormalities in voluntary saccadic eye movements, cerebellar signs, vertical supranuclear gaze palsy, hepatosplenomegaly (in all forms)