Movement Disorders and Ataxia | The Massachusetts General Hospital Handbook of Neurology

Authors: Flaherty, Alice W.; Rost, Natalia S.

Title: Massachusetts General Hospital Handbook of Neurology, The, 2nd Edition

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Movement Disorders and Ataxia

A. See also

Adult Movement Disorders and Ataxia, p. 74.

B. Ataxia

Acute or intermittent:
- Cerebellitis: Usually postviral, especially varicella and EBV. CSF shows mild lymphocytosis. Usually resolves completely.
- Intoxication: DPH, lead, alcohol, thallium .
- Occult neuroblastoma: Usually with opsoclonus-myoclonus. Thought to be paraneoplastic.
- Metabolic disorders: Including maple syrup urine dz, Hartnup's dz, pyruvate decarboxylase deficiency, arginosuccinic aciduria, hypothyroidism.
- Paroxysmal disorders: Seizure, migraine, benign positional vertigo, familial episodic ataxia.
- Other: Guillain-Barr syndrome, posterior fossa hemorrhage or stroke, multiple sclerosis.
Progressive ataxia:
- Congenital malformations: Aplasias, Dandy-Walker, Chiari.
- Hereditary degeneration:
  - Spinocerebellar ataxias: Besides ataxia, there are often neuropathies, absent DTRs, weakness, and other organ system involvement, depending on the syndrome; See Table 18.
  - Ataxia-telangiectasia syndrome: A defect in DNA repair causing ataxia, telangiectasias, infections, and tumors.
  - Abetalipoproteinemia (Bassen-Kornzweig syndrome): Treat with vitamin E.
  - Other: Leukodystrophy, mitochondrial dz .
- Posterior fossa tumors: See HA, vomiting (especially if early AM, not preceded by nausea), cranial nerve abnormalities, papilledema, meningismus.
- Vitamin deficiencies: E.g., E, B₁₂.

C. Chorea

Causes include Sydenham's dz, Wilson's dz, Huntington's dz, hyperthyroidism, vasculitis, basal ganglia tumors or strokes, estrogens, pregnancy, kernicterus, pantothenate kinase associated neurodegeneration (PKAN, n e Hallervorden-Spatz dz; see eponym rant, p. 28.)

Sydenham's chorea: Poststreptococcal, in association with rheumatic fever, often after other sx have resolved. Often with emotional lability and OCD. Check antistreptolysin O titer. Pt should get prophylactic penicillin until adulthood because 1/3 will develop valvular dz.
Wilson's dz: Autosomal recessive, copper builds up in liver and basal ganglia.
- H&P: Usually childhood onset. Cirrhosis, corneal Kayser-Fleischer rings, personality changes, and extrapyramidal sx including tremor, ataxia, dysarthria, and dystonia.
- Tests: Low serum ceruloplasmin, high urine copper, abnormal LFTs, subcortical and brainstem changes on CT or MRI.
- Rx: D-penicillamine 250 500 mg PO qid. Pyridoxine supplements. Side effects include transient decline of neurological function, allergic sensitivity, nephrotic syndrome, a lupus-like syndrome, low platelets, myasthenia, and Goodpasture's syndrome.

D. Dystonia

See also causes of chorea, above, and adult dystonia, p. 76.

Drug effect: E.g., from neuroleptic or antiemetic.
Idiopathic torsion dystonia (dystonia musculorum deformans): Onset usually age 5-15, with action dystonia of one foot that then spreads. Often mistaken for hysteria. Often autosomal dominant, from the DYT-1 gene. Rx: high-dose anticholinergics or deep brain stimulation.
Dopa-responsive dystonia: Autosomal dominant, incomplete penetrance. Worsens throughout day, improves with sleep. Responds to levodopa.

E. Tics

Common in normal 6- to 9-year-olds. Tourette's syndrome is motor and vocal tics for more than 1 yr without interruption; often with ADHD, OCD, echolalia, coprolalia (uncommon, strong family history). For motor tics, consider clonidine or pimozide. For OCD, use high-dose fluoxetine.