Status epilepticus in acute ischemic stroke.

Stroke is one of the most frequent causes of acute symptomatic status epilepticus. The aim of this study was to investigate the electroclinical features of status epilepticus in acute ischemic stroke. Nine consecutively admitted patients with status epilepticus during ischemic stroke were examined: five of them had convulsive unilateral or generalized status epilepticus for from 24 hours to 9 days after a large hemispheric infarction, always associated with EEG epileptiform abnormalities; the remaining four had focal motor status epilepticus during the first 24 hours after a small cortical or subcortical infarction, and showed no clear EEG changes. Status epilepticus in acute ischemic stroke may have two distinct electroclinical patterns of different prognostic significance.

The effect of space location on neglect depends on the nature of the task.

It has been often reported that in patients with visual neglect line bisection is more accurate in the right than in the left hemispace. However, no data are available on the effect of hemispace on reading errors associated with neglect. We examined a 62-year-old man who presented with severe left neglect following a large infarction in the right cerebral hemisphere. The patient was asked to read 180 words aloud and to bisect 90 lines. Stimuli were presented in three different spatial locations: across the centre, to the right or to the left of the body midline. Line bisection was significantly more accurate in the right hemispace compared with the centre, or the left hemispace. In contrast, reading was significantly more accurate with words presented on the left side than on the centre or right side. This is the first time that such dissociation has been reported. We hypothesize that the dissociation depends on the nature of the stimuli and on the different cognitive demands of the tasks.

Neuroleptic malignant syndrome in an AIDS patient: clinical and pathological findings.

Neuroleptic malignant syndrome (NMS) has been recently described following therapy with non strictly neuroleptic drugs that alter dopaminergic function, such as sulpiride and metoclopramide, and might occur more easily in patients with functional or organic brain disorders. We observed an AIDS patient who suffered from NMS following treatment with clotiapine for insomnia and agitation. Two months later, he presented with a similar syndrome following antiemetic treatment with alizapride. On both occasions, the symptoms completely regressed after the administration of dopaminergic and muscle relaxant drugs. The patient died of pneumonia one month after the last episode. The present paper describes the clinical and pathological findings.

Veralipride-induced tardive dystonia in a patient with bipolar psychosis.

Veralipride is a benzamide derivative effective in the treatment of menopausal syndrome. Despite its antidopaminergic action, extrapyramidal side effects seem to be very uncommon. We observed a patient with bipolar psychosis, who developed segmental dystonia after taking the drug. The disorder slowly receded in 14 months, but reappeared during neuroleptic therapy a few months later. To the best of our knowledge, this is the first case of veralipride-induced tardive dystonia, and the affective disorder might have predisposed our patient to the development of this tardive side effect.

Lyme disease. First reported case in Sicily.

Recently many cases of Lyme disease have been described in Italy. The patients come prevalently from Friuli Venezia Giulia and the eastern Ligurian Coast. Isolated cases have been diagnosed in other parts of northern Italy and in the central regions. We describe the first Sicilian case, concluding that Lyme borreliosis may well be widely distributed in our country.

Orthostatic tremor: essential and symptomatic cases.

We studied clinically and electrophysiologically 8 patients affected by orthostatic tremor (OT), which is an unusual movement disorder consisting of shaking movements of the legs and trunk in the standing position. We failed to find any cause in 6 of the 8 cases. In 2 patients OT was clearly secondary to neurologic disease: hydrocephalus due to non-tumoral aqueduct stenosis and chronic relapsing polyradiculoneuropathy. The findings obtained suggest a relationship between OT and essential tremor (ET).

Drug-induced tremor of the tongue.

Two cases are reported in which a transient tremor of the tongue followed treatment with thioridazine and flunarizine. The finding of this kyperkinesia as the only extrapyramidal side-effect of drug therapy is uncommon.

Recurrent familial brachial plexus palsies as the only clinical expression of 'tomaculous' neuropathy.

Two familial cases of recurrent brachial plexus are described and similar episodes were noticed in other members of the family. Electrophysiological investigations found impaired motor and sensory nerve conduction velocity in affected and nonaffected members. Tomaculous neuropathy was found at biopsy of peripheral nerve in more than 40% of dissected fibers. In addition, two affected members showed a reduced interpupillary distance, i.e. the most common dysmorphic feature found in the hereditary neuralgic amyotrophy (HNA). A literature review showed only two other instances of recurrent familial brachial plexus palsies as the only manifestation of tomaculous neuropathy (hereditary neuropathy with liability to pressure palsy, HNPP). The dysmorphic feature found in our cases in addition to the clinical, electrophysiological and anatomical data support the hypothesis that these cases of HNPP and the HNA may represent the same disease.

Familial hypertrophy of masticatory muscles.

Three familial cases of idiopathic hypertrophy of the masticatory muscles are reported. The muscular enlargement was noted in late childhood or early adult life, was bilateral, insidious, painless and non-progressive. Electrophysiological and laboratory findings were normal in one case. CT examination, performed in two cases, showed homogeneous bilateral enlargement of masseter muscle bulk consistent with true hypertrophy. Two patients also showed congenital malformations. A review of the literature showed that this disorder is very rare.

Different clinical features of essential tremor: a 200-patient study.

Two hundred consecutive patients affected by essential tremor (ET), referred to the Movement Disorders Center of the University of Bologna between January 1977 and December 1981, were studied to identify the different clinical features of this disorder, and its associated neurological signs. In this series, 169 patients suffered from typical essential tremor (TET); in the other 31 cases uncommon clinical features were detected: 25 presented postural, action and rest tremor (continuous tremor: CT); 5 had orthostatic tremor (OT) and 1 was affected by writing tremor (WT). Buccolinguofacial dyskinesias (BLFD) were associated with ET in 19 cases; 2 presented dystonia, and in 6 mild extrapyramidal signs were detected. Uncommon types of tremor: ET plus BLFD and ET associated with extrapyramidal signs had an higher incidence in older people. No patient developed Parkinson's disease (PD) during the follow-up period (2-5 years).

Congenital muscular dystrophy with central nervous system involvement: case report.

A case of a mediterranean boy with congenital muscular dystrophy (CMD) and central nervous system (CNS) involvement with mild intellectual impairment and seizures is reported. Muscular dystrophy involved both skeletal and mimic muscles, and histological findings were consistent with a congenital dystrophy. EEG recordings showed generalized and localized paroxysmal activities. CT scan demonstrated low-density periventricular areas. Ophthalmoplegia was also observed. A literature review disclosed that in some western cases of CMD plus CNS involvement, cranial muscles other than mimic muscles may be involved.

X-linked bulbo-spinal neuronopathy of late onset.

Two cases of X-linked bulbo-spinal neuronopathy (BSN) of late onset are presented. An extensive review of the literature shows that, in addition to the signs of a chronic degeneration of brainstem and spinal neurons, other clinical features may also be present at the onset of the disease, or develop during its course. Some clinical, electrophysiological and anatomical data support the hypothesis that BSN is not purely a motoneuronopathy but also concerns sensory nerve.

Essential tremor and buccolinguofacial dyskinesias.

Essential tremor (ET) has been described in association with major dyskinesias, such as dystonia musculorum deformans, torticollis, and dystonic writer's cramp. In a series of 104 patients affected by ET, the incidence of buccolinguofacial dyskinesias (BLFD) has been investigated, including a comparison with another dyskinesia involving the facial muscles--mimic muscle tremor. The association of ET and BLFD may be casual; however, on the other hand, it might suggest the existence of a subgroup of patients suffering from a non-specific sign of motor control impairment, such as ET, in which other non-specific disorders of voluntary movement, such as BLFD, can gradually develop, particularly late in life.