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Ataxia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor in Chief: M.Umer Tariq [2]; Raviteja Guddeti, M.B.B.S. [3] Apeksha Gupta, MBBS[4]

Synonyms and keywords: Unsteady gait; ataxy; staggering gait; impaired coordination; lack of coordination; incoordination; incoordination of muscle movement

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Ataxia (from Greek α- [used as a negative prefix] + -τάξις [order], meaning “lack of order”) is a neurological sign and symptom consisting of gross incoordination of muscle movements [1]. Ataxia is an aspecific clinical manifestation implying dysfunction of parts of the nervous system that coordinate movement, such as the cerebellum Several possible causes exist for these patterns of neurological dysfunction. The term “dystaxia” is rarely used as a synonym.

Ataxia results from the involvement of cerebellar structures, or from a combination of cerebellar and extra-cerebellar lesions, especially the brainstem [2]. Ataxia can be of several types:

  • Sporadic ataxias. Ataxias of this type usually begin in adulthood and have no known family history.
  • Hereditary ataxias. These ataxias are caused by a defect in a gene that is present from the start of a person’s life and can be either dominantly inherited or recessively inherited. Recessive disorders commonly cause symptoms to begin in childhood rather than in adulthood.

Risk Factors

  • Viral infections, such as chickenpox , Coxsackie virus, Epstein-Barr, or HIV
  • Bacterial infections such as Lyme disease
  • Exposure to certain toxins, such as lead , mercury , thallium, alcohol , and organophosphates found in insecticides
  • Cerebellar hemorrhage, abscess, blood clot, or obstruction of an artery
  • Para-neoplastic syndromes—occurs when the immune system attacks the cerebellum in the area of a cancer
  • Certain vaccinations.

Medical Therapy

There is no specific treatment for ataxia as such, altough there may be for the underlying cause [3]. The disability of ataxia may be reduced by physical therapy, including exercises, along with leg braces or shoe splints. If foot alignment has been affected then a cane or walker is often used in the effort to prevent falls [4]

References

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  1. ↑ Mariotti C, Fancellu R, Di Donato S (May 2005). “An overview of the patient with ataxia”. J. Neurol. 252 (5): 511–8. doi:10.1007/s00415-005-0814-z. PMID 15895274.
  2. ↑ Ashizawa T, Xia G (August 2016). “Ataxia”. Continuum (Minneap Minn). 22 (4 Movement Disorders): 1208–26. doi:10.1212/CON.0000000000000362. PMC 5567218. PMID 27495205.
  3. ↑ Klockgether T, Paulson H (May 2011). “Milestones in ataxia”. Mov. Disord. 26 (6): 1134–41. doi:10.1002/mds.23559. PMC 3105349. PMID 21626557.
  4. ↑ Akbar U, Ashizawa T (February 2015). “Ataxia”. Neurol Clin. 33 (1): 225–48. doi:10.1016/j.ncl.2014.09.004. PMC 4251489. PMID 25432731.
Classification

Classification

According to current etiology-based classifications, the ataxias can be subdivided into six major groups: autosomal dominant spinocerebellar ataxias (SCA), autosomal recessive ataxias, congenital ataxias, mitochondrial ataxias, X-linked cerebellar ataxias and sporadic ataxias [1].

  • Sporadic ataxias. Ataxias of this type usually begin in adulthood and have no known family history.
  • Hereditary ataxias. These ataxias are caused by a defect in a gene that is present from the start of a person’s life and can be either dominantly inherited or recessively inherited. Recessive disorders commonly cause symptoms to begin in childhood rather than in adulthood.

Classification

Cerebellar Ataxia

The term cerebellar ataxia is employed to indicate ataxia due to dysfunction of the cerebellum. This causes a variety of elementary neurological deficits, such as antagonist hypotonia, asynergy, dysmetria, dyschronometria, and dysdiadochokinesia. How and where these abnormalities manifest depend on which cerebellar structures are lesioned, and whether the lesion is bilateral or unilateral.

  • Vestibulo-cerebellar dysfunction presents with postural instability, in which the person tends to separate the feet on standing to gain a wider base, and avoid oscillations (especially posterior-anterior ones); instability is therefore worsened when standing with the feet together (irrespective of whether the eyes are open or closed: this is a negative Romberg’s test).
  • Spino-cerebellar dysfunction presents with a wide-based “drunken sailor” gait, characterized by uncertain start and stop, lateral deviations, and unequal steps.
  • Cerebro-cerebellar dysfunction presents with disturbances in carrying out voluntary movements, including intention tremor (coarse trembling, accentuated over the execution of voluntary movements, possibly involving the head and eyes as well as the limbs and torso), peculiar writing abnormalities (large, unequal letters, irregular underlining), and a peculiar pattern of dysarthria (slurred speech, sometimes characterized by explosive variations in voice intensity despite a regular rhythm).

Sensory Ataxia

  • The term sensory ataxia is employed to indicate ataxia due to loss of proprioception (sensitivity to joint and body part position), which generally depends on dysfunction of the dorsal columns of the spinal cord, since they carry proprioceptive information up to the brain; in some cases, the cause may instead be dysfunction of the various brain parts that receive that information, including the cerebellum, thalamus, and parietal lobes.
  • Sensory ataxia presents with an unsteady “stomping” gait with heavy heel strikes, as well as postural instability that is characteristically worsened when the lack of proprioceptive input cannot be compensated by visual input, such as in poorly lit environments.
  • Doctors can evidence this during physical examination by having the patient stand with his / her feet together and eyes shut, which will cause the patient’s instability to markedly worsen, producing wide oscillations and possibly a fall (this is called a positive Romberg’s test).
  • Worsening of the finger-pointing test with the eyes closed is another feature of sensory ataxia. Also, when the patient is standing with arms and hands extended toward the examiner, if the eyes are closed, the patient’s finger will tend to “fall down” and be restored to the horizontal extended position by sudden extensor contractions (“ataxic hand”).

Vestibular Ataxia

  • The term vestibular ataxia is employed to indicate ataxia due to dysfunction of the vestibular system, which in acute and unilateral cases is associated with prominent vertigo, nausea and vomiting.
  • In slow-onset, chronic bilateral cases of vestibular dysfunction, these characteristic manifestations may be absent, and dysequilibrium may be the sole presentation.

References

  1. ↑ Teive HA, Ashizawa T (August 2015). “Primary and secondary ataxias”. Curr. Opin. Neurol. 28 (4): 413–22. doi:10.1097/WCO.0000000000000227. PMC 6516769 Check |pmc= value (help). PMID 26132530.

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Pathophysiology

Pathophysiology

Ataxia can have both motor and sensory components. [1], and not all patients with ataxia have disease pathology in the cerebellum. The first symptom for patients with ataxia is usually gait imbalance. Patients experience difficulty in climbing stairs and often hold on to near by objects or railings. Double vision is present in the initial stages, often seen when patients turn their heads quickly. Blurry vision is also common. Patients experience Slurred speech which makes some words difficult to be understood. Also, they can face loss of hand dexterity resulting in handwriting problems and difficulty performing delicate movements [2]


Most common experiences symptoms are :

  • Uncoordinated movements of the limbs or trunk
  • Clumsiness with daily activities
  • Difficulty walking
  • Speech disturbances with slurred speech and changes in tone, pitch, and volume
  • Visual complaints
  • Abnormal eye movements
  • Headache
  • Nausea and vomiting
  • Lightheadedness
  • Changes in mental state, such as personality or behavioral changes
  • Chaotic eye movements
  • Difficulty swallowing

References

  1. ↑ Diener HC, Dichgans J (1992). “Pathophysiology of cerebellar ataxia”. Mov. Disord. 7 (2): 95–109. doi:10.1002/mds.870070202. PMID 1584245.
  2. ↑ Kuo SH (August 2019). “Ataxia”. Continuum (Minneap Minn). 25 (4): 1036–1054. doi:10.1212/CON.0000000000000753. PMC 7339377 Check |pmc= value (help). PMID 31356292.


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Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Causes

Common Causes[1]

Causes by Organ System

Cardiovascular Arteriosclerosis, Circulation disorders in area of brain stem, Morgagni’s Syndrome
Chemical / poisoning 2,4,6-Trichlorophenol, Acrylamide, Aftershave, Amitraz, Aniline, Antifreeze, Apple seed poisoning, Arizona Bark Scorpion poisoning, Benzene, Blue-ringed octopus poisoning, Buckeye poisoning, Carbon monoxide poisoning, Death Camas poisoning, English Ivy poisoning, Ethotoin, Galantamine hydrobromide, Gasoline, Lantana poisoning, Lead, Mayapple poisoning, Mercury, Mescal poisoning, Minamata disease, Mistletoe poisoning, Thallium poisoning, Tolterodine toxicity, Toxic mushrooms — Anticholinergic, Toxic mushrooms — Monomethylhydrazine, Toxic mushrooms — Psychedelic, Varnish makers’ and painters’ Naptha, Xylene
Dermatologic Erythrokeratodermia with ataxia, Tranebjaerg-Svejgaard syndrome, DeSanctis-Cacchione syndrome, Xeroderma pigmentosum
Drug Side Effect Agalsidase beta, Alprazolam, Amobarbital sodium, Artemether and lumefantrin, Atroine, Amiodarone, Barbituates, Benzodiazepines, Carbamazepine, Chlordiazepoxide, Cidofovir, Cytarabine, Eslicarbazepine acetate, Estazolam, Ethosuximide, Flurazepam hydrochloride, Flurbiprofen, Flucytosine, Gabapentin, Hydroxychloroquine, Lacosamide, Lamotrigine, Levetiracetam, Loxapine, lomustine, Metronidazole, Morphine, Meprobamate, Nabilone, Nelarabine, Olanzapine,oxazepam Oxcarbazepine, Perampanel, Pergolide, Perphenazine, Phenytoin, Polymyxin B, Primidone, Rufinamide, Secobarbital sodium, Sertraline, Spironolactone, Topiramate
Ear Nose Throat Acoustic neurinoma, Benign Paroxysmal Positional Vertigo, Reardon-Wilson-Cavanagh syndrome, Richards-Rundle syndrome, Susac syndrome, Treft-Sanborn-Carey syndrome
Endocrine Adult-onset adreno leukodystrophy, Diabetic neuropathy, Functioning pancreatic endocrine tumor, Furukawa-Takagi-Nakao syndrome, Hypogonadism, Hypothyroidism, Insulinoma, Metastatic insulinoma, Morgagni’s Syndrome
Environmental Acute altitude sickness
Gastroenterologic Bassen-Kornzweig syndrome, Celiac disease, Cirrhosis, Dykes-Markes-Harper syndrome, Hepatocerebral degeneration, Wilson’s Disease
Genetic Alpers Syndrome, Angleman syndrome, Bangstad syndrome, Bassen-Kornzweig syndrome, Bhaskar-Jagannathan syndrome, Cerebellar ataxia, Cockayne syndrome, Dravet syndrome, Familial isolated deficiency of vitamin E, Familial periodic ataxia, Feline spongiform encephalopathy, Friedreich’s ataxia, Griscelli disease, Hallervorden-Spatz disease, Hereditary spastic paraparesis, Krabbe leukodystrophy, Richards-Rundle syndrome, Roussy-Levy syndrome, Rubinstein-Taybi syndrome, Sialidosis type 1 and 3, Tranebjaerg-Svejgaard syndrome, Xeroderma pigmentosum, X-linked sideroblastic anaemia
Hematologic Acanthocytosis, Aceruloplasminemia, Macrocytic anemia, Pernicious anemia, Revesz Debuse syndrome, X-linked sideroblastic anaemia
Iatrogenic No underlying causes
Infectious Disease Brain abscess, Cerebellar abscess, Chickenpox, Diphtheria, Encephalitis, HIV infection, Measles, Quaternary syphilis, Ramsay-Hunt syndrome, Smallpox, Tabes dorsalis
Musculoskeletal / Ortho Dinno-Shearer-Weisskopf syndrome, Furukawa-Takagi-Nakao syndrome, Myopathy
Neurologic Acute Disseminated Encephalomyelitis, Alexander Syndrome, Alpers Syndrome, Arachnoid Cysts, Arnold-Chiari Malformation, ARTS syndrome, Ataxia deafness reardon type, Ataxia tapetoretinal degeneration, Ataxia telangiectasia / Louis-Bar syndrome, Ataxia-oculomotor apraxia syndrome, Basal ganglia calcification, Basilar artery migraine, Vertebro-basilar artery ischemia, Brain abscess, Brain cancer, Brain cyst, Brain injury, Brainstem or cortical lesions, Central nervous system lymphoma, primary, Cerebellar abscess, Cerebellar ataxia, Cerebellar cortex atrophy, Cerebellar degeneration, Cerebellar haemorrhage, Cerebellar heredoataxia, Cerebellar hypoplasia, Cerebellar infarct, Cerebellar mass, Cerebral Amyloid Angiopathy, Familial, Cerebral hemorrhage, Cerebral infarction, Cerebral palsy, Cerebrorenodigital syndrome, Cerebrovascular accident, Ceroid lipofuscinosis, Cervical myelopathy, Chorea familial benign, Chronic progressive traumatic encephalopathy, Cockayne syndrome, Combarros Calleja Leno syndrome, Corneal cerebellar syndrome, Creutzfeldt-Jakob disease, Cutler-Bass-Romshe syndrome, Dandy-Walker Syndrome, Dementia pugilis, Demyelinating disorder, DeSanctis-Cacchione syndrome, Dinno-Shearer-Weisskopf syndrome, Dravet syndrome, Dykes-Markes-Harper syndrome, Dysequilibrium syndrome, Encephalitis, Encephalocele frontal, Epiphyseal tumor, Episodic ataxia, Erythrokeratodermia with ataxia, Feline spongiform encephalopathy, Fisher’s Syndrome, Friedreich’s ataxia, Frontal lobe lesion, GALOP Syndrome (gait disorder, autoantibodies, late age onset, polyneuropathy), Glioma, Granulomatous Angiitis of the central nervous system, Guillain-Barré syndrome, Hallervorden-Spatz disease, Heidenhain syndrome, Hepatocerebral degeneration, Hydrocephalus, Infantile polymyoclonus, Inherited neuropathies, Joubert syndrome, Juvenile pilocytic astrocytoma, Kearns-Sayre Syndrome, Kuru, Lhermitte-McAlpine syndrome, Machado-Joseph Disease, Macrogyria, pseudobulbar palsy and mental retardation, Mann Syndrome, Marinesco-Sjogren-like syndrome (MSLS), Marinescu-Garland Syndrome, Medulloblastoma, Meinecke syndrome, Meningoencephalocele, Mental retardation progressive spasticity, X-linked, Mental retardation, X-linked, with cerebellar hypoplasia and distinctive facial appearance, Metachromatic Leukodystrophy, Miller Fisher Syndrome, Mitochondrial encephalomyopathy, Mononeuropathy/radiculopathy affecting the lower extremities, Multiple sclerosis, Myelopathy, Myoclonus, Neuronal intranuclear hyaline inclusion disease, Normal pressure hydrocephalus, Olivopontocerebellar atrophy, Opsoclonus myoclonus syndrome, Parietal lobe lesion, Parkinson’s disease, Partington X-linked mental retardation syndrome, Pelizaeus-Merzbacher disease, Peripheral neuropathy, Polyneuritis, Polyneuropathy, Post-infective polyradiculopathy, Posterior column ataxia with retinitis pigmentosa, Posterior fossa tumor, Progressive Multifocal Leukoencephalopathy, Reardon-Wilson-Cavanagh syndrome, Rett syndrome, Rhombencephalosynapsis, Roussy-Levy syndrome, Schroer-Hammer-Mauldin syndrome, Segawa syndrome, autosomal recessive, Shy-Drager syndrome, Spastic paraplegia, Spinal cord compression (and pressure on dorsal nerve roots), Spinal cord tumor, Spinocerebellar ataxia, Spinocerebellar degenerescence, book type, Stroke, Susac syndrome, Tabes dorsalis, Telencephalic leukoencephalopathy, Thalamic syndrome, Thoracic dysplasia — hydrocephalus syndrome, Tranebjaerg-Svejgaard syndrome, Transient ischemic attack (TIA), Transverse myelitis, Vascular dementia, Vertebral Artery Dissection, Vertebrobasilar dolichoectasia, Vertigo, benign paroxysmal, Westphal’s Disease, Wilson’s Disease
Nutritional / Metabolic Abetalipoproteinemia, Alpha-ketoglutarate dehydrogenase deficiency, Aminoaciduria, Argininosuccinase lyase deficiency – late onset, Arginosuccinate synthetase deficiency, Ataxia with Vitamin E Deficiency, Biotinidase deficiency, Ceruloplasmin deficiency, Cholestanol storage disease, Coenzyme Q 10 (CoQ10), deficiency, Complex 2 mitochondrial respiratory chain deficiency, Congenital Disorders of Glycosylation, Cystinuria — lysinuria, Fabry’s Disease, Gangliosidosis GM1 type 3, Gaucher disease type 2, Glutathione synthase deficiency, Glycine encephalopathy, atypical mild form, GM2-gangliosidoses, Hartnup Disease , Hexosaminidase deficiency, Homozygous hypobetalipoproteinemia, Hydroxyacyl-coa dehydrogenase, type 2, deficiency, Infantile sialic acid storage disorder, Lactic acidosis congenital infantile, Lipoamide dehydrogenase deficiency, Maple syrup urine disease, Mevalonic aciduria, Mitochondrial cytopathy, Niemann-Pick disease, Pellagra-like syndrome, Phosphoribosylpyrophosphate synthetase superactivity, Purine nucleoside phosphorylase deficiency, Pyruvate dehydrogenase deficiency, Refsum Disease, Selective vitamin E deficiency, Sialidosis type 1 and 3, SSADH deficiency (succinic semialdehyde dehydrogenase deficiency), Thiamine deficiency, Triglyceride storage disease with impaired long-chain fatty acid oxidation, Vitamin B12 deficieny, Wernicke-Korsakoff psychosis, Zinc deficiency
Obstetric/Gynecologic No underlying causes
Oncologic Acoustic neurinoma, Brain cancer, Bronchogenic carcinoma, Central nervous system lymphoma, primary, Epiphyseal tumor, Functioning pancreatic endocrine tumor, Glioma, Insulinoma, Juvenile pilocytic astrocytoma, Medulloblastoma, Metastatic insulinoma, Paraneoplastic syndromes, Posterior fossa tumor, Spinal cord tumor
Opthalmologic Combarros Calleja Leno syndrome, Corneal cerebellar syndrome, Fisher’s Syndrome, Marinesco-Sjogren-like syndrome (MSLS), Posterior column ataxia with retinitis pigmentosa, Refsum Disease, Revesz Debuse syndrome, Schroer-Hammer-Mauldin syndrome, Susac syndrome, Treft-Sanborn-Carey syndrome
Overdose / Toxicity Lithium toxicity, Phenytoin toxicity
Psychiatric Hysteria
Pulmonary Bronchogenic carcinoma, Prolonged anoxia
Renal / Electrolyte Cerebrorenodigital syndrome, Cutler-Bass-Romshe syndrome
Rheum / Immune / Allergy ARTS syndrome, Griscelli disease, Fisher’s Syndrome
Sexual No underlying causes
Trauma Brainstem or cortical lesions, Frontal lobelesion, Mann Syndrome, Parietal lobelesion, Head trauma
Urologic No underlying causes
Miscellaneous Alcohol intoxication, Amyloidosis, oculoleptomeningeal, Arteriosclerosis, Bonnemann-Meinecke-Reich syndrome, Chitayat-Moore-Del Bigio syndrome, Hemangioblastoma, Hyperammonemia, sporadic ataxia, Tick paralysis, Transthyretin amyloidosis

Causes by Alphabetical Order


References

  1. ↑ Klockgether T, Paulson H (May 2011). “Milestones in ataxia”. Mov. Disord. 26 (6): 1134–41. doi:10.1002/mds.23559. PMC 3105349. PMID 21626557.
  2. ↑ Chardon L, Sassolas A, Dingeon B; et al. (2009). “Identification of two novel mutations and long-term follow-up in abetalipoproteinemia: a report of four cases”. Eur. J. Pediatr. 168 (8): 983–9. doi:10.1007/s00431-008-0888-6. PMID 19066957. Unknown parameter |month= ignored (help)
  3. ↑ Pagon RA, Bird TD, Dolan CR, Stephens K, Adam MP, Miyajima H. PMID 20301666. Missing or empty |title= (help)
  4. ↑ Oliver SJ, Sanders SJ, Williams CJ; et al. (2012). “Physiological and psychological illness symptoms at high altitude and their relationship with acute mountain sickness: a prospective cohort study”. J Travel Med. 19 (4): 210–9. doi:10.1111/j.1708-8305.2012.00609.x. PMID 22776381. Unknown parameter |month= ignored (help)
  5. ↑ Bonnefont JP, Chretien D, Rustin P; et al. (1992). “Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis”. J. Pediatr. 121 (2): 255–8. PMID 1640293. Unknown parameter |month= ignored (help)
  6. ↑ Pagon RA, Bird TD, Dolan CR; et al. PMID 20301738. Missing or empty |title= (help)
  7. ↑ Müller KI, Bekkelund SI (2011). “Epilepsy in a patient with ataxia caused by vitamin E deficiency”. BMJ Case Rep. 2011. doi:10.1136/bcr.01.2011.3728. PMID 22696689.
  8. ↑ Henschen F (1976). “[Morgagni’s syndrome]”. Virchows Arch A Pathol Anat Histol (in German). 370 (1): 1–11. PMID 818785. Unknown parameter |month= ignored (help)
  9. ↑ Kitsiou-Tzeli S, Konstantinidou A, Sofocleous C; et al. (2012). “Familial Pelizaeus-Merzbacher disease caused by a 320.6-kb Xq22.2 duplication and the pathological findings of a male fetus”. Birth Defects Res. Part A Clin. Mol. Teratol. 94 (6): 494–8. doi:10.1002/bdra.23015. PMID 22511562. Unknown parameter |month= ignored (help)
  10. ↑ Franceschi M, Parmigiani F, Zamproni P, Cairoli G, Canal N (1984). “Richards-Rundle syndrome, cochleovestibular dysfunction and neurofibromatosis in a family”. J. Neurol. 231 (1): 11–3. PMID 6425460.


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Differentiating Ataxia from other Diseases

Differentiating Ataxia from other Diseases

TYPE CAUSE PROGRESSION IMAGING FEATURES and OTHER TESTS SYMPTOMS
Acute cerebellitis
  • Primary infectious, postinfectious or postvaccination disorder [1].
  • Epstein-Barr virus, influenza A and B, mumps, varicella-zoster virus, coxsackie virus, rotavirus, echovirus, Mycoplasma pneumoniae and immunization
  • Postinfectious cerebellitis typically occurs between one and six weeks after varicella or measles, but also can follow Epstein-Barr or other viral infections and vaccinations in teenagers and young adults.
 From self-limited to fatal, depending on the amount of cerebellar swelling
  • Normal or abnormal brain magnetic resonance imaging (MRI) at onset
  • Bilateral hemispheric cerebellar swelling with cortical and white matter T2 hyperintensities; leptomenigeal enhancement may be present.
  • CSF: Elevated protein and leukocytes, with lymphocytic predominance, normal glucose
  • Blood/CSF: Antibodies anti- HSV, EBV, VZV, mumps, rubella, Lyme disease
  • Trunk and limbs ataxia, fever, abnormal eye movements, dysarthria, headache, nausea, vomiting and decreased level of consciousness
Bacterial infection
  • Mycoplasma pneumoniae, Listeria monocytogenes
  • Usually sudden and progressive
  • Lumbar puncture for examination of the cerebrospinal fluid (CSF) and microbiologic testing
  • Fever
  • instability when walking
  • changes in coordination that primarily affect the trunk or head and not the limbs
  • nodding or other unusual head movements
  • unusual eye movements, such as involuntarily darting from side to side
  • slow or slurred speech
  • changes in mood, behavior, or personality
  • headaches
  • nausea or vomiting
Acquired immunodeficiency syndromes
  • Related to M. pneumonia, Epstein-Barr virus, herpes simplex virus, and toxoplasmosis
 Subacute ataxia which progress in months
  • Positive serologic test for HIV
  • Cerebellar atrophy
Chronic alcohol use/Alcoholic cerebellar degeneration
  • Toxic effects on the central and peripheral nervous systems
  • Direct toxic alcoholic effect on the Purkinje cells
 Rapid progression (weeks or months)
  • Vermis atrophy
  • Severe ataxia of gait and lower limbs with relatively mild involvement of the upper limbs.
  • Speech and ocular motility are usually preserved
Antibiotic-induced acute ataxia
  • Interaction of polymyxins with neurons has been associated with the occurrence of several neurotoxic events
 Weeks after initiation
  • Brain MRI abnormalities
  • Characteristic reversible MRI signal abnormalities in the cerebellar dentate nuclei, dorsal brainstem, or splenium of the corpus callosum
  • Non-specific EEG abnormalities
  • Ataxia may also occur in isolation or combined with dizziness, generalized muscle weakness, partial deafness, visual disturbances, vertigo, confusion, hallucinations, seizures, and neuromuscular blockade
Toxic ingestions
  • Alcohol, benzodiazepines, or other anticonvulsant drugs or exposure to environmental toxins such as mercury or lead
  • Usually sudden and progressive
  • Toxicology testing
  • Elevated plasma levels of substances like lithium, and phenytoin
  • Other imaging unremarkable; Cerebellar atrophy in late stages
  • Additional findings that suggest occult ingestion (eg, depressed consciousness)
Atypical infections
  • Progressive multifocal leukoencephalopathy
  • Caused by reactivation of the JC virus in immunocompromised hosts.
  • Progressive and multifocal
  • Magnetic resonance imaging (MRI) reveals a multifocal process limited to the white matter
  • Presents with subacute neurologic deficits like altered mental status, motor deficits (hemiparesis or monoparesis), limb ataxia, gait ataxia, and visual symptoms such as hemianopia and diplopia
Brain tumors
  • Nonmalignant and malignant tumors of the brain and spinal cord.
  • Usually progressive over weeks to months
  • MRI
  • Biopsy
  • Surgery
  • Symptoms and signs of tumor local invasion,
  • Adjacent structures compression,
  • Raised intracranial pressure
Stroke
  • Ischemic stroke (part of the brain loses blood flow)
  • Hemorrhagic stroke (bleeding occurs within the brain)
  • Risk factors
    • high blood pressure (hypertension),
    • high cholesterol,
    • diabetes, and
    • smoking.
  • Progressive or Sudden
  • CT Scan: look for bleeding or masses in the brain.
  • CT perfusion scan: see how much brain is at risk to check brain blood supply (perfusion).
  • MRI of the brain
 Symptoms of ataxia with
  • numbness,
  • weakness,
  • tingling, or
  • vision loss or changes.
  • Confusion,
  • Changes in the level of consciousness,
  • Trouble speaking,
  • Trouble understanding speech, vertigo,
  • Balance problems
Vestibular neuritis
  • Problem in the inner ear or the brain.
  • Inflammation of the vestibular nerve caused by a virus
  • Symptoms may come and go over short periods of time, or last for longer periods of time
  • Hearing examination,
  • Blood tests,
  • Electronystagmo-gram
  • Imaging studies of the head and brain
  • Dizziness or vertigo
  • Falling or a feeling as if you are going to fall
  • Lightheadedness, fainting, or a floating sensation
  • Blurred vision
  • Confusion or disorientation
  • Nausea and vomiting
  • Diarrhea
  • Changes in blood pressure and heart rate
  • Fear
  • Anxiety
  • Panic

References

  1. ↑ Marsden JF (2018). “Cerebellar ataxia”. Handb Clin Neurol. 159: 261–281. doi:10.1016/B978-0-444-63916-5.00017-3. PMID 30482319.


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Epidemiology and Demographics

Epidemiology and Demographics

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References

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Risk Factors

Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Various Risk Factors for ataxia are :

  • Viral infections, such as chickenpox , Coxsackie virus, Epstein-Barr, or HIV
  • Bacterial infections such as Lyme disease
  • Exposure to certain toxins, such as lead , mercury , thallium, alcohol , and organophosphates found in insecticides
  • Cerebellar hemorrhage, abscess, blood clot, or obstruction of an artery
  • Paraneoplastic syndromes—occurs when the immune system attacks the cerebellum in the area of a cancer
  • Certain vaccinations

Those who have a family history of inherited ataxia (e.g Friedreich’s ataxia) may be at higher risk for developing ataxia. Genetic testing may be recommended in these cases.


Certain factors increase the chance of recurrent acute cerebellar ataxia:

  • Stroke
  • Malformation of the cerebellu
  • Multiple sclerosis
  • Migraine or vertigo
  • Genetic or metabolic disorders
  • Brain tumor
  • Alcohol use disorder
  • Certain medications

References

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Natural History, Complications and Prognosis

Natural History, Complications and Prognosis

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References

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Diagnosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | X Ray | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

[1]

Treatment

Treatment

The ataxia that occurs in children can often can go away in a few months without any treatment. In cases where an underlying cause is identified, the cause will be treated.

In some cases, you may have continuing and disabling symptoms. Treatment includes:

  • Corticosteroids
  • IV immune globulin
  • Plasma exchange therapy
  • Medications to improve muscle coordination

Occupational or physical therapy may also be needed. Changes to diet and nutritional supplements may also help.


Template:WikiDoc Sources

  1. ↑ de Silva R, Greenfield J, Cook A, Bonney H, Vallortigara J, Hunt B, Giunti P (February 2019). “Guidelines on the diagnosis and management of the progressive ataxias”. Orphanet J Rare Dis. 14 (1): 51. doi:10.1186/s13023-019-1013-9. PMC 6381619. PMID 30786918.

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