[Peripheral nervous system involvement in HCV-related mixed cryoglobulinemia] / Interessamento del sistema nervoso periferico nella crioglobulinemia mista HCV-correlata.

In HCV-related mixed cryoglobulinemia (MC) a peripheral neuropathy (PN) may occur. To evaluate the prevalence and the characteristics of PN, 133 consecutive patients with HCV-MC (117 type II, 16 type III) were studied. Neurologic evaluation was performed according to the guidelines of Italian Group for the Study of Cryoglobulinemias, using a neurological disability score and a neurological symptom score. In 52/133 patients an electrophysiologic study (ENG) of ulnar, peroneal and sural nerves was performed. For 27/52 patients ENG data registered at different times (interval 12-96 months) were available. In 11 patients a sural nerve biopsy was obtained. An overt PN, mostly as sensory asymmetrical or symmetrical nerve impairment, was found in 107/133 patients (80.4%). ENG abnormalities-reduction or absence of sensory and sometimes of motor action potential, normal or slightly impaired nerve conduction velocity, consistent with axonal damage- were detected in 48/52 patients (92.3%). In 26 out of the 27 patients observed at different times an evolution of PN was found. Nerve biopsies showed a prevalent axonal damage, swollen endothelial cells in epi- and perineurial vessels and scarce mononuclear perivascular infiltrates. No leukocytoclastic vasculitis was observed. Immunoglobulins and complement in sub-perineurial vessel wall were detected. CONCLUSIONS: In HCV-MC a PN is frequent. It is mostly a sensory and progressively worsening axonopathy. Different mechanisms may be involved in the pathogenesis of this disorder and a direct role of HCV cannot be excluded.

Gender and peripheral neuropathy in chronic alcoholism: a clinical-electroneurographic study.

In some alcohol-related pathologies of chronic alcoholism women are more vulnerable than men. A consecutive sample of 62 chronic alcoholics was studied, 18 females and 44 males, aged between 28 and 69 years to assess the incidence and distribution of peripheral neuropathy with regard to gender. All patients underwent clinical and neurological observations, laboratory tests, and electroneurography. Total lifetime dose of ethanol (TLDE) and other risk factors for neuropathy (disease duration, age, nutritional status) were calculated and correlated to sural nerve sensory-evoked potential (SEP) amplitude. In 42 patients (67.7%), we observed the presence of clinical and/or infraclinical neuropathy, mostly axonal, in 29 males (65.9%) and 13 females (72.2%). In women, compared to men, TLDE and disease duration were significantly inversely correlated to sural nerve SEP amplitude, i.e. in women, SEP amplitude is significantly reduced in relation to TLDE and disease duration increase. These data indicate a higher sensitivity of females towards the toxic effects of ethanol, other than malnutrition, on peripheral nerve fibres.

Progressive rubella panencephalitis. Follow-up EEG study of a case.

Progressive rubella panencephalitis is a very rare slow virus disease of the nervous system. The authors present a case, concerning a young man, aged 20 years, died 11 months after the onset of the disease. The following peculiarities of the case are emphasized: 1) the clinical symptomatology and the evolution (myoclonus, lack of cerebellar impairment) could suggest the diagnosis of SSPE; 2) the EEG recordings showed epileptiform abnormalities, long latency diffuse periodic complexes and--during interferon therapy and simultaneously with a temporary clinical improvement--the appearance of short latency anterior periodic complexes.

Carpal tunnel syndrome: a clinical electrophysiological study of 84 cases.

A retrospective study of 84 patients with carpal tunnel syndrome (CTS), for a total of 118 hands, was performed in order to check clinical aspects of the disease including age of onset, sex, side affected, other associated pathological conditions, and to assess the existence of correlation between the clinical picture and electrophysiological parameters. All patients were subjected to careful clinical observation and electromyographic and electroneurographic examinations. Our study confirms that the female sex is by far predominant, the most interesting ages being the 5th and 6th decades of life and the right side is more affected than the left. None of the patients had a family history of CTS. The sensory pathology is far more frequent than the motor one and the percentage of hands with sensory and motor deficit increases with the duration of the disease. As far as the electrophysiological data is concerned, the most important and also the earliest alterations fundamentally concern the distal motor latency and distal sensory conduction velocity of the median nerve and the amplitude of its sensory action potential (SAP). This study leads us to point out a significant correlation in CTS between electrophysiological (distal motor latency and distal sensory conduction velocity of the median nerve, amplitude of its SAP) and clinical parameters (symptomatology and duration of the disease).

[Clinico-developmental aspects in 44 cases of polymyositis/dermatomyositis]. / Aspetti clinico-evolutivi in 44 casi di polimiosite/dermatomiosite.

A retrospective study, paying particular attention to the clinical and evolutive aspects of the disease, was performed on 44 subjects affected by polymyositis/dermatomyositis (PM/DM) and hospitalized at the Institute of Medical Clinics of the 1st School of Medicine of Naples University. On the basis of the different clinical pictures, the cases were classified into the following groups: primary PM (4); primary DM (19); DM/PM associated with malignancy (6); childhood DM/PM (3); PM/DM associated with connective tissue disorders (12). Diagnosis was established in terms of the following criteria: a) symmetrical and mostly proximal bilateral muscle weakness (100%); b) elevation of serum enzymes (86.3%); c) electromyographic findings of myopathy sometimes with fibrillation potentials, increased insertional irritability and pseudo-myotonic discharges (93.1%); d) muscle biopsy changes compatible with a clinical form of polymyositis (83.3% out of 30 cases); e) dermatological manifestations including particularly pink or lilac edema-erythema over the periorbital areas, wine-red maculae, Gottron's sign, "poikiloderma vasculare atrophicans", telangiectasias and skin vasculitis (86.3%). An involvement of the extraneural apparatus and organs was present in 40 patients; the most damaged was the osteoarticular apparatus, followed by esophagus, lung, heart and kidney; such pathology was rarely present in the childhood form. A follow-up of the disease has been performed in 36 cases and the therapy consisted fundamentally of high dose corticosteroids (mostly prednisone), associated, in a minority of cases, with methotrexate. A clinical improvement was observed in most cases and a remission of the disease in part of the latter. However, a worsening of the illness was noticed only in the patients suffering from PM/DM associated with malignancy, and mortality rate was 11.1% in all.

Perceived parental rearing behaviour and psychopathology in epileptic patients: a controlled study.

Memories of parental rearing behaviour were assessed by the EMBU in 61 epileptics and 151 healthy controls. The occurrence of the first crisis during the childhood was an inclusion criterion for patients. Epileptics, as compared with controls, rated their fathers and mothers as less stimulating, their fathers as less performance oriented and affectionate, and their mothers as more tolerant. Moreover, the score on the subscale 'favouring subject' for both fathers and mothers was higher in epileptics. As patients with and without interictal psychopathological features were compared, the scores on the subscales 'overprotective' and 'favouring subject' for mothers and 'abusive' and 'depriving' for fathers were higher in the former subgroup, whereas that on the subscale 'performance oriented' for fathers was higher in the latter. No significant difference was observed among patients suffering from the various subtypes of epilepsy. These results are consistent with the idea that parents of epileptics tend to encourage passivity in their children, have low expectations as regards their ability to operate effectively, and treat them in a more indulgent way because of their disability. Furthermore, they are in line with the reported association between maternal overprotectiveness and problem behaviour in epileptics.

[Further considerations on epilepsy in the follow-up of patients subjected to surgery for intracranial pathology]. / Ulteriori considerazioni sull'epilessia nel follow-up di soggetti operati per patologia intracranica.

A retrospective study has been carried out on a pool of 210 pts. suffering from various intracranial lesions (tumours, abscesses, vascular malformations, hydrocephalus), submitted to neurosurgical operation. The main evidences of our investigation are: both early and tardive seizures are observed only related to supratentorial pathology, mainly to tumours; pts. with seizures before the operation present a major incidence of postoperative epilepsy; and pts. with early seizures have more often also late epilepsy. The Authors relate this evidence to a factor of individual predisposition; pharmacological prophylaxis can be really effective in reducing postoperative epilepsy.

Continuous muscle fiber activity associated with denervation atrophy. Clinical presentation, electrophysiological and pharmacological investigations, histological and histochemical examinations of muscle biopsy.

We describe a case presenting continuous muscle fiber activity syndrome, which was slightly reduced by the blocking of nerve conduction and abolished by ischemia. Clinical, electromyographical and bioptical evidence of marked abnormalities of peripheral motoneurons were observed. Association with central motoneuron lesions was suspected due to the observation of initial pyramidal signs. Therapy with diphenylhydantoin and/or carbamazepine resulted only in discrete amelioration of the syndrome. This case, which presented similarities as well as differences to the cases described by Isaacs and to those reported by other authors after 1961, stimulates a critical discussion of the already raised problem of the clinical heterogeneity of continuous muscle fiber activity syndrome.

H-reflex behavior in glue (n-hexane) neuropathy.

The H-reflex response, and in particular the excitability cycle of spinal motor-neurones, were studied in a group of patients suffering from glue (n-hexane) neuropathy. Motor and sensitive nerve conduction, EMG, and clinical features were also considered. The results, showing an increased excitability of alpha-motorneurones, give evidence of spinal involvement in these patients.