A. Muratorio

Unbalanced progesterone and estradiol secretion in catamenial epilepsy

Ten women with a documented history of catamenial epilepsy underwent a hormonal study to evaluate hypophyseal-gonadal function. Baseline values of luteinizing hormone, follicle-stimulating hormone and prolactin were similar in catamenial seizure patients and in control groups throughout a complete menstrual cycle. Stimulated secretions of the same hypophyseal hormones in catamenial seizure patients overlapped those of the controls. The luteal secretion ratio of progesterone to estradiol was significantly reduced in catamenial seizure patients versus normal controls. In a subgroup of catamenial seizure patients on antiepileptic therapy, luteal progesterone levels were remarkably decreased compared to normal and epileptic controls. These results indicate that catamenial epilepsy is characterized by an imbalance in ovarian steroid secretion and emphasize the need for an endocrinological assessment in these patients.

Headache, Panic Disorder and Depression: Comorbidity or a Spectrum?

Past epidemiological and clinical research has identified depression as the most common psychiatric disorder associated with headache. The present study carried out in a neurology headache clinic showed that the major associations were with current anxiety disorders, especially panic and related conditions. These findings were particularly true of the subgroup of migraine with aura; in the relatively few patients with mood disorders, depression was nearly always comorbid with panic or other anxiety disorders. Past history of depression was mainly a characteristic of the tension headache group. These data are compatible with the hypothesis that migraine, especially that with aura, panic disorder and some forms of depressive illness are part of the same spectrum.

Clozapine in Huntington's chorea

Article abstract –In an open-label trial, we evaluated the efficacy of clozapine on abnormal involuntary movements in five patients with Huntingtons chorea. We administered clozapine at increasing doses of 25, 50, and 150 mg/d for 3 weeks. Subjective self-evaluation of all patients reported reduction of chorea and improvement of daily living activities. At the end of the trial, all patients requested to continue with clozapine. Objective evaluation with the Abnormal Involuntary Movements Scale demonstrated in all patients moderate-to-marked reduction of abnormal involuntary movements without any significant side effects; the improvement was dose-dependent and markedly decreased 1 week after drug withdrawal

Type 1 fiber abnormalities in skeletal muscle of patients with hypertrophic and dilated cardiomyopathy: Evidence of subclinical myogenic myopathy

Abnormalities of skeletal muscle have been described in patients with dilated and hypertrophic cardiomyopathy. Eleven patients with dilated and eight with hypertrophic cardiomyopathy without overt symptomatic skeletal myopathy underwent extensive neuromuscular studies. Quantitative electromyography showed abnormal reduction of motor unit potential duration, indicative of myogenic myopathy, in four patients (36%) with dilated and in three (37%) with hypertrophic cardiomyopathy. Values were 21% to 40% (mean 28%) lower than those in age-matched normal control subjects. The presence of normal nerve conduction velocities and of normal motor unit fiber density in all patients indicated lack of neurogenic abnormalities. Skeletal muscle biopsy was performed in five patients with dilated and in four with hypertrophic cardiomyopathy. In all nine patients light and electron microscopy showed central hyporeactive cores, selective atrophy and mitochondrial abnormalities of type 1 fibers but not of type 2 fibers. The degree of impairment of left ventricular function in patients with electromyographic abnormalities was similar to that of those without (percent fractional shortening at two-dimensional echocardiography 21 +/- 9 versus 25 +/- 10, ejection fraction at angiography 39 +/- 13% versus 42 +/- 13% and left ventricular end-diastolic pressure 21 +/- 6 versus 21 +/- 8 mm Hg) as well as symptom duration (9 +/- 4 versus 12 +/- 8 months). Thus, subclinical electromyographic alterations indicative of myogenic myopathy are frequent in patients with dilated and hypertrophic cardiomyopathy and are unrelated to the degree of impairment of left ventricular function. The concomitant histologic alterations, characterized by selective type 1 atrophy, are similar to those observed in congenital and idiopathic myopathies, but different from those described in secondary heart failure.

Muscle modifications in Parkinson's disease: myoelectric manifestations

The muscle changes occurring in Parkinsons disease (PD) may come about as a consequence of the modified pattern of motor unit activation and rigidity, which are characteristic of the disease. A tendency towards hypertrophy of type I fibers and, in some instances, atrophy of type II fibers has been observed. Fourteen patients affected by PD and 10 age-matched controls were studied in order to investigate these muscle changes. We indirectly evaluated muscle modifications by measuring muscle fiber conduction velocity (CV) and median frequency (MDF) of the power spectrum using automatic analysis of surface EMG. The tibialis anterior muscle was selected for the study of contractions electrically induced by 35 Hz pulse trains lasting 30 s; the myoelectric signal was detected using the 4-bar electrode technique described by Broman et al. (Broman, H., Bilotto, G. and De Luca, C.J. Myoelectric signal conduction velocity and spectral parameters: influence of force and time. J. Appl. Physiol., 1985, 58: 1428-1437). Muscle biopsy specimens were obtained in 4 PD patients by surgical excision at the site where the EMG recording electrode had been placed. The main difference observed between PD subjects and controls was the rate of change of MDF and CV during the course of stimulated contraction; patients with PD sustained a smaller fatigue related decrease in both parameters compared to controls. According to our histological data, this result can be explained by a type I fiber percentage which accounts for 79% of the myofiber population on average. As expected, the CV basal values correlated directly with type I fiber diameter. These data suggest that non-invasive surface EMG techniques are useful in assessing the modifications of muscle characteristics that are observed in PD patients and for analyzing some aspects of the peripheral fatigue in this disease.

Dream structure in Parkinson's patients.

Dream reports of patients with Parkinsons disease (PD) were analyzed to ascertain whether cognitive deficits associated with this nonfocal brain pathology influence dream structure or dream recall. Fifteen outpatients with idiopathic PD were sampled (diagnosed from 1 to 10 years and currently in stage II or III of Hoehn and Yarhs scale); all were without psychiatric symptoms or major medical illnesses and were currently being treated with L-dopa. After an adaptation night in the sleep laboratory, each patient spent a night in which he/she was awakened at least twice in rapid eye movement sleep and asked to report dream experience. Thirteen patients were able to report at least one dream. Overall frequency of dream recall (71.4%) was fully compatible with normative data for the elderly. Multiple regression analyses showed that both the length of the dream report as story and the organization of contents into coherent episodes (analyzed using Mandler and Johnsons story grammar) varied significantly in relation to level of cognitive functioning and, in part, of language comprehension, but not in relation to age, illness duration, and dose of L-dopa.

Platelet monoamine oxidase B activity in parkinsonian patients.

Monoamine oxidase B (MAO B) plays a pivotal role in N-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) induced Parkinsonism. An increased MAO B activity in platelets of patients with idiopathic Parkinsons disease (PD) is reported in this study. The possibility that high MAO B activity may represent a trait of vulnerability for PD by enhancing the neurotoxic effects of environmental compounds is discussed.

Platelet peripheral benzodiazepine receptors are decreased in Parkinson's disease

Peripheral benzodiazepine (BDZ) receptors are located in a variety of tissues, including platelets, in the nuclear and/or mitochondrial membranes. We studied the density of peripheral BDZ receptors in platelets of 10 de novo Parkinsons disease (PD) patients, 18 PD patients treated with a levodopa/carbidopa combination, and in 15 healthy subjects matched for sex and age. The binding assay was conducted using [3H]PK 11195, a specific ligand for peripheral BDZ receptors. A significant decrease in the density of [3H]PK 11195 binding sites has been observed in PD patients with respect to controls (p less than 0.01), but not between de novo and treated PD patients. No correlation has been found between the decrease in density of [3H]PK 11195 binding sites in platelets and either the duration or severity of PD. Peripheral BDZ receptors are implicated in the regulation of mitochondrial respiratory function. Thus, their decrease in PD might parallel the abnormalities in mitochondrial function recently found in this neurologic disease.

Prevalent cardiac involvement in dystrophin Becker type mutation.

Myocardial involvement is frequently present in Xp21-linked muscular dystrophy, due to a lack of dystrophin in cardiac fibres. We describe a 41-yr-old man affected by dilated cardiomyopathy with sporadic episodes of myoglobinuria induced by effort and increased levels of serum creatine kinase. Very mild signs of skeletal myopathy were clinically evident. His mother was affected by an indefinite cardiopathy and suddenly died when she was 36 yr old. Muscle biopsy of the patient showed a dystrophic process. Dystrophin analysis together with a genetic DMD locus study led us to diagnose Becker type muscular dystrophy, with truncated dystrophin and a gene deletion extending from exon 45 to 48. Prevalent cardiac involvement in a Becker type mutation of the dystrophin gene further confirms clinical variability of dystrophinopathies.

Naloxone partly counteracts apomorphine side effects

The effects of naloxone on side effects provoked by apomorphine (APO) administration in patients with parkinsonian syndrome have been studied. The group under study included eight patients with Parkinsons disease and four with parkinsonism who received 100 micrograms/kg s.c. APO acutely to test dopaminergic responsiveness. All patients were treated with 20 mg domperidone tablets t.i.d. and then for 2 consecutive days (in double blind fashion) were given a 2-hour i.v. saline infusion alone or with naloxone (8 mg) starting 30 min before APO administration. In both groups, naloxone delayed the appearance of sleepiness, and reduced the intensity of yawning, sleepiness, nausea, and vomiting as compared with saline. These findings indicate a potential usefulness of naloxone and other opioid antagonists in preventing acute APO side effects.