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Biomedical subjects

H Hefter

Publications and source records attributed to H Hefter.

At least 73 records · Page 4Linked to original sources
PubMed1993

Persistent chorea after recurrent hypoglycemia. A case report.

In a 72-year-old woman with a 33-year history of diabetes mellitus bilateral chorea had occurred after a series of hypoglycemic comas at the age of 58. The choreiform movements remained untreated, persisted more than 10 years and inspite of intermittent exaggeration were tolerated by the patient. Except for the hyperkinetic movements, neurological examination of this patient was otherwise normal as was cranial computer tomography.

Aged↗
PubMed1992

Regional cerebral glucose consumption measured by positron emission tomography in patients with Wilson's disease.

Using positron emission tomography (PET), the regional cerebral metabolic rate of glucose consumption (rCMRGlc) was measured in 14 patients with Wilson's disease (WD) and 23 normal subjects. In WD patients, cerebellar, striatal and--to a lesser extent--cortical and thalamic rCMRGlc were significantly decreased compared with controls. Striatal rCMRGlc was significantly reduced in those 4 patients who had recently started decoppering therapy as compared with striatal rCMRGlc measured in those 10 patients with longer duration of medication. Caudate rCMRGlc correlated significantly with various signs of extrapyramidal dysfunction. Cerebellar, thalamic and cortical rCMRGlc correlated significantly with the severity of pyramidal signs. These data indicate that the PET measurement of rCMRGlc may be a useful tool to evaluate cerebral involvement in WD and to monitor the response to treatment.

Adult↗
PubMed1992

Impairment of temporal organization of speech in basal ganglia diseases.

Absolute and relative speech timing were examined in patients suffering from Parkinson's, Huntington's, and Wilson's disease. The task was to speak a standard sentence 10 times, first slowly, and then successively faster up to maximum rate. All patient groups had low maximal speech rates and showed decreased variability of speech rate. The duration of pauses between words was the same as in normals and the relative time structure of the test sentence was basically preserved. For comparison, two cases with nonfluent aphasia had even slower speech rates, large increases in pause duration, and major changes in relative speech timing. The results show the same type of alterations of the temporal organization of speech as those characteristic for rapid alternating limb movements in such patients. They support the view that the speech and skeletomotor systems share common neural control modes despite fundamental biomechanical differences. The common denominator between the speech and the skeletomotor disturbances in basal ganglia diseases may be the undamping and slowing of a fast central oscillator.

Adult↗
PubMed1992

Oscillatory activity in different motor subsystems in palatal myoclonus. A case report.

In a patient with palatal myoclonus the mutual interaction between voluntary movements and the myoclonic activity was analysed. Amplitude and frequency of myoclonic activity in hand muscles were modulated by flexions and extensions. A 1:1 relationship was found between EMG-bursts in hand muscles and palatal movements. A 1:2 relationship was found between eye and finger movements. Resetting of myoclonus in the abductor digiti minimi muscle occurred after cutaneous ulnar nerve stimulation. It is suggested that feedback plays an important role in the generation of the oscillatory activity in PM in addition to pacemaker activity in brainstem neurons.

Aged↗
PubMed1992

Improvement of motor performance of HIV-positive patients under AZT therapy.

We performed motor tests (most rapid alternating movements [MRAMs] of index fingers and most rapid contractions [MRCs] of voluntary isometric index finger extensions) in HIV-positive patients with (group 1) and without (group 2) AZT treatment over a 6-month period. Whereas MRAMs remained uninfluenced, MRCs showed a clear improvement in the treated group and a decline in the nontreated group, according to the T helper cell counts. MRCs were not only a sensitive test procedure for detecting subclinical lesions in HIV-positive patients, but also a reliable therapy control measurement.

HIV Seropositivity↗
PubMed1991

Wilson disease: clinical presentation, treatment, and survival.

OBJECTIVE: To evaluate the diagnostic features, clinical course, and overall long-term survival of patients with Wilson disease. DESIGN: Retrospective cohort study with a mean follow-up period of 14.2 years. SETTING: A university medical center and a community hospital. PATIENTS: Fifty-one consecutive patients with Wilson disease were evaluated between 1957 and 1989. INTERVENTIONS: Patients were treated with D-penicillamine (600 to 1800 mg/d). Two patients with end-stage liver disease had liver transplantation. MAIN RESULTS: Initial symptoms occurred at a mean age of 15.5 years. At diagnosis, the most common neurologic signs were dysarthria, tremor, writing difficulties, and ataxia followed by hypersalivation and headache. Somatic symptoms included abdominal pain, hepatomegaly, splenomegaly, cirrhosis of the liver, and thrombocytopenia. The mean serum concentrations of ceruloplasmin and copper were 44 mg/L and 4.7 mumol/L, respectively. The mean basal urinary copper excretion was 5.5 mumol/d, and the mean hepatic copper concentration was 19.6 mumol/g dry weight. Free serum copper concentration (mean, 2.7 mumol/L) was a reliable indicator of disease and was useful in assessing the effectiveness of therapy (values less than 1.6 mumol/L). Treatment with D-penicillamine improved most of the hematologic and neurologic abnormalities but had little effect on hepatomegaly and splenomegaly and did not reverse cirrhosis. Two patients died of fulminant hepatic failure during the observation period, whereas two others with end-stage liver disease had successful liver transplantation and remain asymptomatic. Long-term survival of patients with Wilson disease was similar to that of age- and sex-matched controls. CONCLUSION: Our results suggest that long-term treatment of patients with Wilson disease with D-penicillamine can relieve symptoms and improve prognosis.

Adolescent↗
PubMed1991

A premovement silent period does not occur prior to rapid changes of velocity during human limb movements.

Surface electromyographic activity (EMG) of the interosseous dorsalis I, the hand extensors and the biceps muscles was analysed in human young normal subjects for the occurrence of a precontraction (PCSP) or a premovement silent period (PMSP) before a sudden increase in tension or rapid change of position after a small isometric contraction or slow isotonic movement. Contrary to the results of Conrad et al. (Exp. Brain Res., 51 (1983) 310-313), in whose experiments the contraction condition changed from isometric to isotonic, a PCSP or PMSP could never be observed in our experiments, where contractions or movements first started at a low force level or with a low speed which had to be changed most rapidly after 2 s and where the recording condition remained strictly either isometric or isotonic. It is concluded that the central nervous system resets the motoneuronal pool activity when the central program is changed from the control of an isometric to the control of an isotonic contraction.

Adult↗
PubMed1991

[Antimicrobial susceptibility of organisms infecting the urinary tract in northern Israel].

Urinary tract infections (UTI), especially in women, are very frequent and require antimicrobial treatment. The choice of drug depends on the susceptibility of the infecting organism. In many cases treatment is begun before the identity and susceptibility of the organism/s cultured are known. Knowledge of the profile of the infecting organisms and their drug susceptibility can improve the treatment of community-acquired UTI. We recorded the distribution and susceptibility of 50,699 positive UTI cultures, during the years 1986-7, in 2 community laboratories in northern Israel serving a population of about 830,000. The sensitivity of organisms to ampicillin was only 32 in the Haifa region and 42 in the Afula region and, respectively, to cotrimoxazole 51 and 76, to cephalexin 60 and 77, to nitrofurantoin 83 and 89, and to nalidixic acid 80 and 92. The differences between the regions were statistically significant. As a result of this study, we suggest that ampicillin should not be used empirically in UTI in these these 2 regions, nor cotrimoxazole in the Haifa region. All women with uncomplicated UTI should be treated with nitrofurantoin or nalidixic acid, provided they are not sensitive to these drugs and tolerate them well. The use of new drugs such as the quinolones and amoxicillin/clavulanic acid must be controlled and monitored to avoid rapid development of resistant strains.

Anti-Bacterial Agents↗
PubMed1991

Does tremor pace repetitive voluntary motor behavior in Parkinson's disease?

In patients with Parkinson's disease and in normal subjects, the influence of tremor on repetitive voluntary movement was investigated in the index finger by comparing frequency of isometric force tremor with frequency of voluntary alternating isometric contractions. Tremor frequency, measured over the range from 0 to 70% maximum voluntary force, usually increased with force. The tremor frequency band was lower and more often overlapped with the upper voluntary frequency range in patients than in normal subjects. Normal subjects could accurately produce voluntary contractions at all cue frequencies from 1 to 5 Hz. Patients could produce auditory-paced frequencies of 1 and 2 Hz, but at higher cue frequencies, their voluntary contractions were often faster or slower than the cue. The faster or "hastened" voluntary frequencies were within the tremor frequency band, whereas the slowed voluntary frequencies were below it. Maximal voluntary frequency was often greater than the lowest but always less than the highest tremor frequency. It is concluded that parkinsonian tremor may pace voluntary repetitive movements to go faster than intended with the highest tremor frequency being an upper limit for voluntary frequency. Similar mechanisms may underlie the hastened repetitive vocal responses that were also observed in the parkinsonian patients.

Adult↗
PubMed1991

Two cases of cerebral toxoplasmosis in AIDS patients mimicking HIV-related dementia.

Two unusual cases of cerebral toxoplasmosis in AIDS patients are presented. Two homosexual males aged 33 and 52 years in CDC stage IV C1 complained of memory loss during the past 6 months, as well as weight loss and mild fever. They showed severe intellectual deterioration and discrete basal ganglia dysfunction. Motor performance and cognitive function as well as the conventional EEG findings were grossly abnormal. MRI was normal in the younger patient but showed signal-intensive zones in the basal ganglia and cortical atrophy in the older one. CSF and serological antibody tests were normal; immunological function was severely impaired. The patients were diagnosed as late "HIV-related dementia". Both deteriorated rapidly and died within a few weeks. Neuropathological examination revealed histologically severe Toxoplasma gondii encephalitis, involving the basal ganglia in particular. It is concluded that in AIDS patients with a severely impaired immune status cerebral opportunistic infection may present as dementia with mild basal ganglia impairment in the absence of other focal neurological signs or the characteristic radiological findings.

AIDS Dementia Complex↗
PubMed1991

Does orthotopic liver transplantation heal Wilson's disease? Clinical follow-up of two liver-transplanted patients.

Two patients with Wilson's disease (WD) underwent orthotopic liver transplantation, one for subacute liver failure and the other for severe oesophageal haemorrhage. After transplantation both patients fully recovered within five months, and copper metabolism returned to normal. Follow-up examinations were continued for 4 and 6 years. Clinical as well as electrophysiological testing in these two patients yielded better results than in most of 12 WD-patients being conventionally treated for a similar period or even longer.

Adult↗
PubMed1991

Electrophysiological characterization of the X-linked recessive bulbospinal neuronopathy (XRBSN).

Detailed electrophysiological analyses including nerve conduction velocity measurements of motor and sensory nerves, EMG recordings of a variety of muscles, evoked potentials, magnetic brain stimulation, electrophysiological testing of autonomic functions, tremor measurements and testing of voluntary movements were applied to three patients with X-linked recessive bulbospinal neuronopathy (XRBSN). All three patients presented with a slowly progressive anterior horn impairment, involvement of sensory nerves and posterior columns, but intact central descending motor pathways and an essential tremor responding to propranolol treatment. The spectrum of electrophysiological findings helps to diagnose XRBSN reliably even in sporadic cases.

Adult↗
PubMed1990

Normal striatal glucose consumption in two patients with benign hereditary chorea as measured by positron emission tomography.

Positron emission tomography (PET) with [18F]-2-fluoro-2-deoxy-D-glucose (FDG) was used to investigate the regional cerebral metabolic rate of glucose consumption (rCMRGlc) in two patients with benign hereditary chorea (BHC) and 21 normal subjects. Relative and absolute values of cerebellar, striatal, thalamic, and cortical rCMRGlc were within normal limits for both patients with BHC, indicating that the choreic movement disorder encountered in these two patients was not caused by a decrease of energy metabolism in the striatum such as that found regularly in most patients with other forms of chorea (e.g. Huntington's and Wilson's disease).

Adult↗