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Benign hereditary chorea--entity or syndrome?

A Schrag¹, N P Quinn, K P Bhatia

¹Department of Neurology, Queen Square, London, UK.

Movement Disorders : Official Journal of the Movement Disorder Society

|April 7, 2000

Summary

The diagnosis of benign hereditary chorea (BHC) is increasingly questioned due to reclassifications. This study suggests BHC is a syndrome needing further research, not a distinct diagnosis.

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Area of Science:

Neurology
Genetics
Pediatrics

Background:

Benign hereditary chorea (BHC) is a rare childhood-onset disorder.
Its existence is questioned as many patients are later diagnosed with other conditions.

Purpose of the Study:

To re-evaluate the diagnostic validity of benign hereditary chorea.
To investigate if BHC represents a distinct genetic entity.

Main Methods:

Reviewed 42 published families with BHC and contacted authors for follow-up.
Evaluated 11 new families and 4 sporadic cases with suspected BHC through videotaping.
Analyzed existing literature and clinical data for atypical features.

Main Results:

Diagnosis of BHC was changed in 9 out of 11 reviewed families from literature.

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BHC was not unequivocally diagnosed in any of the 11 new familial or sporadic cases.

Atypical features were common, and consensus on diagnosis was often not reached.

Conclusions:

Benign hereditary chorea is likely a syndrome, not a distinct diagnosis.
Further research is required to determine if a specific genetic abnormality underlies BHC.
The concept of BHC as a singular entity with a single gene defect remains unproven.