Causes of cerebellar ataxia

Ataxia of the cerebellar (etiologic classification)

I. Dysgenesis of the cerebellum

1. Hypoplasia
2. Dandy Walker malformation
3. Arnold-Chiari malformation

II. Hereditary and degenerative diseases

1. Diseases of accumulation: lipidosis, glycogen exchange disorders, leukoencephalopathy (Balsen-Kornzweig abetalipoproteinemia, Rephsum's disease, Tay-Sachs disease, Nyman-Pick disease, metochromatic leukodystrophy, ceric lipofuscinosis, sialidosis, Lafore's disease)
2. Metabolic disorders of amino acids, mitochondrial enzyme deficiency and other metabolic disorders (insufficiency of transcarbamylase, arginine succinase, arginase, Hartnup disease, Leigh disease, pyruvate dehydrogenase insufficiency, mitochondrial myopathy).
3. Chromosome disorders (Gippel-Lindau disease, ataxia-telangiectasia)
4. Multiple systemic atrophy
5. Wilson-Konovalov's disease
6. Autosomal recessive (Friedreich's ataxia, early-onset ataxia), autosomal dominant (dentato-rubro-pallid-Lewis atrophy, Machado-Joseph disease, episodic ataxia of type 1 and type 2) and X-linked recessive spinocerebellar ataxia.

III. Acquired metabolic and nutritional disorders

1. Toxins
2. Insufficiency of nutrition and syndromes associated with alcoholism (alcoholic cerebellar degeneration, vitamin E deficiency)

IV. Infections

1. Viral infections (subacute sclerosing panencephalitis, chickenpox, measles, parainfluenza infections, herpes simplex, postinfection disseminated encephalomyelitis, mumps, cytomegalovirus infection)
2. Non-viral infections (toxoplasmosis, mycoplasmic infection, legionnaires' disease)
3. Prion diseases (Creutzfeldt-Jakob disease, Gerstman-Streusler disease)

V. Vascular diseases

1. Hemorrhagic stroke
2. Ischemic stroke

VI. Tumors

1. Primary tumors (astrocytoma, medulloblastoma, neurinia, meniigoma)
2. Metastatic swelling
3. Paraneoplastic lesion of the cerebellum

VII. Demyelinating diseases

1. Central nervous system (multiple sclerosis)
2. Peripheral Nervous System (Miller Fisher Syndrome)

VIII. Basilar migraine

IX. Iatrogenic (drug) ataxia

1. Diphenine
2. Carbamazepine
3. Barbiturates
4. Lithium
5. Piperazine
6. Other

Cerebellar ataxia (some clinical disease markers)

Impaired consciousness:

Hemorrhage or cerebral infarction; intoxication; anonzodorosis status epilepticus.

Impaired mental function:

Atactic cerebral palsy; congenital ataxia; some hereditary ataxia with early onset; pigmentary xeroderma.

Dementia:

Hydrocephalus; some "degenerative" ataxia; Gerstman-Streusler syndrome; Creutzfeldt-Jakob disease.

Atrophy of the optic nerve:

Multiple sclerosis; Friedreich's ataxia; other hereditary ataxia; alcoholism.

Retinopathy:

Some hereditary ataxias; mitochondrial encephalopathy.

Apraxin eye movements (ocular motor apraxia): ataxia-telangiectasia.

Supranuclear ophthalmoplegia:

Autosomal dominant ataxia; idiopathic ataxia with late onset; hexosaminidase deficiency; Nyman-Pick disease (type C).

Interernal ophthalmoplegia:

Multiple sclerosis; Wernicke's encephalopathy (rarely); "Degenerative" ataxia.

Ptosis, paralysis of the external muscles of the eye:

Mitochondrial encephalomyopathy; encephalopathy Wernicke; Lee's disease.

Paralysis III, IV and VI of the cranial nerves:

Heart attack; hemorrhage; multiple sclerosis; volumetric process in the posterior cranial fossa.

Ocular flutter, optoclonus:

Viral Cerebellitis; paraneoplastic syndromes.

Nystagmus, "beating down":

Processes in the region of the large occipital foramen; "Degenerative" ataxia.

Extrapyramidal syndromes (dystonia, chorea, rigidity of muscles):

Wilson-Konovalov's disease; dominant-inherited and sporadic ataxia with late onset; ataxia-telangiectasia.

Myoclonus:

Mitochondrial encephalomyopathy; multiple insufficiency of carboxylase; ceroid lipofuscinoses; sialidosis; Ramsay Hunt's syndrome; unconvulsive status epilepticus; some autosomal dominant ataxia.

Hyporeflexia or areflexia, often with a decrease in pro-conception and vibratory feelings:

Friedreich's Ataxia; other hereditary or "degenerative" ataxia; alcoholic cerebellar degeneration; insufficiency of vitamin E; hypothyroidism; ataxia-telangiectasia; pigment xeroderma; leukodystrophy; Miller Fisher syndrome.

Deafness:

Some hereditary ataxias; mitochondrial encephalomyelopathy.

Fragile, brittle hair:

Lack of arginine succinase (hereditary autosomal recessive disease), manifested by a delay in physical and mental development; epilepsy; ataxia; impaired liver function; brittle and growing hair; increased excretion of arginine-succinic acid.

Hair loss:

Intoxication with thallium; hypothyroidism; adrenoleukeloneelopathy.

Low border of hair:

Malformation in the area of the craniovertebral junction and the large occipital foramen.

Skin changes:

Teleangiectasia, especially in the conjunctiva, nose, ears, flexor surfaces of the extremities (ataxia-telangiectasia); sensitivity to light, skin tumors (pigmented xeroderma); pellagrope-like rash (Hartnup disease); dry skin (hypothyroidism, Refsum's disease, cocaine syndrome); pigmentation (adrenoleukomyeloneuropathy).

Changes in the eye area:

Teleangiectasia; the Kaiser-Fleischer ring (Wilson-Konovalov's disease); Retinal angioma (Gippel-Lindau disease accompanied by cerebellar hemangiomas); cataracts (congenital rubella, cholesterol, Sjogren-Larson syndrome, mitochondrial encephalomyopathy); aniridia (Zhilepsy syndrome, manifested by congenital absence of iris, delayed mental development and cerebellar ataxia).

Fever:

Fever can increase the manifestations of intermittent metabolic ataxia; fever may be a manifestation of cerebellar abscess, viral cerebellitis, cysticarcosis.

Vomiting:

Hemorrhage or cerebral infarction; acute demyelination; volumetric process in the posterior cranial fossa; Intermittent metabolic ataxia.

Hepatosplenomegaly:

Niman-Pick's disease, type C; Wilson-Konovalov's disease; alcoholism; some metabolic ataxia of childhood.

Heart disease (cardiomyopathy, conduction disorders): Friedreich's ataxia.

Low growth:

Mitochondrial encephalomyopathy, ataxia-telangiectasia, cocaine syndrome, Sjogren-Larsen syndrome.

Hypogonadism:

Recessive ataxia with hypogonadism; mitochondrial encephalomyopathy; ataxia-telangiectasia; adrenoleukemieloneuropathy; Sjögren-Larsen syndrome.

Deformations of the skeleton:

Friedreich's Ataxia; Sjogren-Larsen's syndrome and other cerebellar degenerations with early onset; hereditary sensory and motor neuropathies.

Immune Deficiency:

Ataxia-telangiectasia; multiple carboxylase deficiency.

Disturbance of nutrition:

Insufficiency of vitamin E; alcoholism.

[1], [2], [3], [4], [5], [6]