Huntington's chorea and tryptophan

Daily use of 7 g l-tryptophan and 70 mg pyridoxine for 14 days led to no deterioration of objectively measured manual motor skills in a small group of patients with Huntington''s chorea.     

A progressive case of ataxia in a family of Huntington's chorea]

One case of ataxic, pyramidal and demential syndrome associated with retinitis pigmentosa occurred in a family with Huntington's disease (36 patients among 7 generations). The disease started at 15 years of age. Ataxia was transiently attenuated by isoniazid. The patient died of the disease at 26 years of age.     

Huntington's chorea and cerebellar atrophy. Apropos of amanatomo-clinical case]

The authors report the clinical and pathological findings in a 46 years old woman with Huntington's chorea and cerebello-olivar atrophy. Eight previously reported cerebellar atrophies in Huntington's chorea with pathological examination are reviewed. The primary or secondary nature of the cerebellar lesions is discussed.     

A case of Huntington's chorea with unilateral ectopic gray matter

The authors report a single case of Huntington's chorea associated with a unilateral focus of ectopic gray matter. The patient's symptoms began at age 45 and included typical involuntary jerking movements of all extremities and face. Mental deterioration may have proceeded the choreiform movements. The family history was positive for Huntington's chorea. Pneumoencephalogram showed atrophy of the caudate nuclei bilaterally early in the disease. The patient improved transiently with haloperidol therapy. The major pathologic features included mild generalized cerebral atrophy with marked atrophy of the caudate nuclei and putamen. Within the white matter of the left frontal lobe, there were irregular nodules of ectopic gray matter with an overall diameter of 2 cm. The rarity of either unilateral ectopia or Huntington's chorea alone, makes it impossible to judge if the two lesions might be linked by a common pathologic mechanism. The significance such a linkage might hold is discussed in light of several currently postulate pathologic mechanisms.     

Involuntary movements in chorea-acanthocytosis: a comparison with Huntington's chorea

The involuntary movements in three patients with chorea-acanthocytosis (CA) and three with Huntington's chorea (HC) were studied by polygraphic recordings and jerk-locked averaging technique. Choreic movements in patients with CA were suppressed by mental calculation, voluntary muscle contraction, and instruction to stop them, whereas movements in HC were generally enhanced by these tasks. Backward averaging of the EEG time-locked to the choreic movement of an extremity or the trunk demonstrated a cortical slow negativity before the movement, similar to the Bereitschaftspotential (“readiness potential”), in cases of CA but not those of HC. Involuntary movements in CA appear to differ from those in HC and might be generated at least in part by a mechanism similar to that of voluntary movement.