Distinguished features between Huntington’s disease, benign hereditary chorea (BHC), dentatorubralpallidoluysian atrophy (DRPLA), and neuroacanthocytosis
| Features | Huntington’s disease | BHC | DRPLA | Neuroacanthocytosis |
|---|---|---|---|---|
| Onset | 4th decade | Early childhood | <20 years, or >40 years | 3rd decade |
| Genetics | Autosomal dominant | Autosomal dominant | Autosomal dominant | Autosomal recessive, dominant, or X-linked |
| Natural history | Progressive, mean disease duration 17 years | Non- progressive, normal life expectancy | Progressive | Progressive, mean disease duration 15 years |
| Seizures | May be present | None | Present in juvenile type | Common |
| Dystonia | Later in disease course | None | Present | Present, esp. in orofacial region |
| Myoclonus | Later in disease course | None | Common in juvenile type | Present |
| Ataxia | Later in disease course | None | Common (100%) | May be present |
| Cognitive deficits | Invariably | None | Invariably | Minimal |
| Psychiatric symptoms | Common and prominent | None | Present | Present |
| Others | Motor impersistence, dysarthria | None | Mental retardation (100%) | Sensorimotor neuropathy, acanthocytes, raised creatine kinase |