Authors: Flaherty, Alice W.; Rost, Natalia S.
Title: Massachusetts General Hospital Handbook of Neurology, The, 2nd Edition
Copyright 2007 Lippincott Williams & Wilkins
> Table of Contents > Child Neurology > Movement Disorders and Ataxia
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.
P.146
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.