PEDBASE.org - The Pediatric Database - Detailed information of BENIGN HEREDITARY CHOREA

BENIGN HEREDITARY CHOREA

DEFINITION:

A rare movement disorder characterized by continuous choreic movements.

EPIDEMIOLOGY:

incidence: rare
age of onset:
- early childhood; can present within the 1st year of life
risk factors:
- familial - autosomal recessive, autosomal dominant, and X-linked forms have been identified
- chrom.#: ?
- gene: ?

PATHOGENESIS:

1. Background

also called Benign Familial Chorea

CLINICAL FEATURES:

1. Choreic Manifestations

chorea usually symmetric and distal
continuous and not episodic or paroxysmal
usually not progressive but can either improve or worsen during adolescence
most children have only chorea but can be associated with:
- delayed motor development
- postural and/or intention tremor
- normal intelligence
- dysarthria
- hypotonia
- athetosis

INVESTIGATIONS:

all tests (CT, MRI, EEG, etc) are normal

MANAGEMENT:

see "Choreic Disorders"
Psychological and Pharmological therapy

Pediatric Database - BENIGN HEREDITARY CHOREA

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