Vasoregulatory activity in patients with Huntington's chorea

Vasoregulatory activity has been assessed in 11 patients with Huntington's chorea and 13 control subjects by intra-arterial pressure studies. There was no significant difference in the resting mean blood pressure or its response to the Valsalva manoeuvre in the 2 groups. However, with rapid tilting of subjects into a 60° head-up position there was a fall in mean blood pressure which was significantly greater in patients with Huntington's chorea than in control subjects (p < 0.005). The significance of this defect in postural vasoregulatory mechanisms is considered. Our observations are also discussed in the light of the recent suggestion by others that the caudate nuclei have an important role in modulating vasomotor activity.     

The cerebrospinal fluid choline levels in patients with Huntington's chorea

Summary Lumbar cerebrospinal fluid (CSF) choline (CH) levels were measured in patients with Huntington's chorea (n = 14). This group was found not to differ significantly from normal controls (n = 13). The values for lumbar CSF Ch levels in the normal subjects were comparable with previously reported values. Of the choreic patients, seven were put on haloperidol treatment (4–6 mg daily). The CSF choline level remained unchanged with this treatment after 20 days. CSF cholinesterase activity was measured in the control and choreic group. The results were not significantly different.     

Neurophysiological study of facial chorea in patients with Huntington's disease.

OBJECTIVE: Choreic movements of patients with Huntington's disease (HD) may result from an abnormal control of sensory inputs. In order to further examine the pathophysiology of facial choreic movements (FCM), we carried out a neurophysiological study, including prepulse inhibition of the blink reflex (BR), in HD patients with and without FCM. METHODS: The study was conducted in 20 genetically proven HD patients with Unified Huntington Disease Rating Scale (UHDRS) scores of FCM ranging between 0 and 3, and in 12 age-matched healthy volunteers who served as control subjects. We counted the number of spontaneous blinks, recorded the electromyographic activity underlying FCM, and analyzed latency, amplitude, and duration of the BR responses to electrical and auditory stimuli. Prepulse inhibition was studied by comparing the responses to test trials with those to control trials. In control trials BRs were obtained to either a single supraorbital nerve electrical stimulus (EBR) or to a 90dB auditory stimulus (ABR). In test trials, the same stimuli were preceded by the prepulse, which was either a weak acoustic tone or a weak electrical stimulus to the third finger, delivered 30-150 ms before. RESULTS: Spontaneous blinking rate was abnormally low in 3 patients, and abnormally high in 9 patients. Mean duration of the BR was longer in patients than in control subjects. In prepulse trials, the percentage inhibition of the BR was abnormally reduced in 15 patients to at least one sensory modality, and significantly correlated with the score of FCM. CONCLUSIONS: Our results suggest that the severity of FCM in patients with HD might be an expression of a disturbance in motor control partly related to an abnormal processing of sensory inputs. Such abnormality involves circuits used in prepulse inhibition of the BR.     

Iatrogenic parkinsonism in Huntington's chorea

This case report describes a 63 year old woman with a nineteen year history of Huntington's Chorea who had been successfully treated with tetrabenazine for 9 years. During a hospital admission for an unrelated medical illness, she was given two doses of chlorpromazine 25 mg for a vascular headache and within hours became mute, extremely rigid, and unable to move or swallow. The consultation psychiatrist confirmed the diagnosis of severe drug-induced parkinsonism by intramuscular injection of benztropine mesylate which resulted in a dramatic immediate improvement. Both chlorpromazine and tetrabenazine were discontinued and the patient's severe parkinsonian symptoms completely abated on oral benztropine mesylate. Tetrabenazine alone, was restarted after 48 hours to control her Huntington's Chorea and there was no recurrence of parkinsonism. (Past history revealed the patient to have tolerated the same doses of chlorpromazine and tetrabenazine when given separately.) Previous literature reports concurrent safe use of antipsychotics with tetrabenazine, but this case report would suggest caution, and early discontinuance in the event of parkinsonism.     

Kluver-Bucy syndrome in Huntington's chorea

In this paper the first case of Kluver-Bucy syndrome (KBS) in Huntington's chorea is reported. The patient, a 46-year-old man with advanced Huntington's disease, displayed prosopagnosia, oral tendencies, emotional changes, hypersexual behavior, and hyperphagia associated with severe dementia. Haloperidol in moderate doses controlled both the KBS and the chorea, suggesting a possible role for the dopaminergic system in the pathogenesis of KBS in Huntington's disease. The presence of profound dementia in our patient supports the previous assertion that human cases of KBS are invariably associated with severe cognitive dysfunction. Since KBS was established as an entity, a great deal of attention has been directed to its neuroanatomical basis. However, due to the multidetermined nature of human behavior, the role of physiological, psychological, and environmental factors should also be taken into consideration with regard to the pathogenesis of this syndrome.     

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.     

Acute treatment of Huntington's chorea with lisuride

The authors studied the effects of lisuride hydrogen maleate (lisuride) on the hyperkinesias of 11 patients suffering from Huntington's chorea (HC). In all patients, acute injection of 150 micrograms of the drug induced a marked temporary improvement of the abnormal involuntary movements; the favourable drug-effect was more pronounced in the patients with a less severe degree of hyperkinesia. The antichoreic activity of the drug was prevented by pretreatment with haloperidol (2 mg) or sulpiride (400 mg), both injected intramuscularly 30 min before lisuride administration. The authors suggest the improvement of the motor disturbance induced in HC by lisuride may be explained on the basis of its preferential action on a subset of brain dopaminergic receptor.