Acta neurologica最新文献

A rare case of spontaneous cure of an internal carotid artery aneurysm is reported; the progressive resolution of the spasm and the disappearance of the aneurysm have been confirmed by serial angiograms respectively performed two weeks, two months and four months after the hemorrhage. The other 14 reported cases of angiographically documented spontaneous cure of intracranial aneurysms are reviewed and the possible causes of spontaneous aneurysmal thrombosis, such as retarded cerebral circulation, arterial spasm, size of the neck, thrombosis of the feeding artery and antifibrinolytic therapy, are discussed.

Heterotopic ossifications are a well known but serious complication in patients suffering severe head injury. Their prevalence varies between 10% and 20%. The current concept of surgery recommends removal after "maturation" of the new developed bone because of a suspected higher rate of recurrence. The observations of early operations in 6 patients and 9 joints led us to the conclusion that the disadvantages of long lasting joint stiffness and the according complications outweigh the danger of recurrence. In our patients recurrence was observed in one subject and even there recurrence was most probably the result of wound infection and concomitant osteomyelitis.

The early onset sensory motor hereditary neuropathy (HSMN) can be divided into two forms: the early onset type (HSMN type III or Dejerine-Sottas) and the congenital hypomyelinating neuropathy (CHN). In both cases, abnormalities of myelination are present in peripheral nerves. Symptoms include hypotonia, weakness, hypotrophy, and areflexia. Skeletal changes may be present. In CHN symptoms may be present at birth and are rapidly progressive. Many authors actually consider the two forms different. The diagnosis is based only on clinical and neuropathological criteria. Here we report a case with a typical phenotype of HSMN type III but with peripheral nerve bioptic findings suggesting a CHN.

Circulating IC levels were assayed serially in 12 G.B.S. patients treated with PE and an attempt was made to predict the outcome of treatment, from the levels of circulating IC. It was found that there was no significant correlation between the levels of circulating IC and the outcome of treatment with PE, in G.B.S. patients.

A 17-year-old girl with acute schizophrenic symptoms who after the administration of 9 mg P.O. of nemonapride, developed a neuroleptic malignant syndrome, a rare but serious adverse effect of neuroleptic medication, is reported. She recovered after the administration of dantrolene. This side effect induced by nemonapride has not been reported previously. Nemonapride is a highly selective dopamine D-2 antagonist, equivalent to haloperidol. It is supposed that nemonapride can induce the neuroleptic malignant syndrome similar to haloperidol.

We retrospectively evaluated results obtained from azathioprine (AZA) treatment on a selected sample of 40 patients affected by autoimmune myasthenia gravis (MG). Patients received AZA as a single immunosuppressive drug for at least 2 years. Twenty out of 40 patients received also a one-month course of cyclophosphamide (CP) before starting AZA. All patients started immunosuppressive treatment out of myasthenic crisis. After 3, 12 and 24 months of AZA treatment, 82.5%, 92.5% and 97.5% of the patients respectively showed improvement in functional state, disappearance of bulbar involvement, or both. The impressive percentage of short-term positive results did not seem influenced by pre-treatment by CP. Side effects included only minor and transitory gastrointestinal symptoms and reversible cytopenia. Although the patient population was either particularly suitable for AZA treatment or candidate to a better response, our data suggest that AZA might also have good short term effects in a subgroup of MG patients.

The authors describe the case of a patient in whom painful tonic spasms developed as a consequence of a small infarct involving the lateral aspect of the putamen, the external capsule, and the subcortical white matter of the posterior insular region. Possible pathogenetic mechanisms explaining painful tonic spasms are discussed.

We describe a 6-year-old child who presented the phenotype of cri-du-chat disease. The study of her caryotype confirmed an interstitial deletion of the short arm of chromosome 5. The neurological examination showed mental retardation, behavioral disturbances and features of cerebellar and cortico-spinal impairment. The MRI scan of the brain showed hypoplasia of the vermis associated with dysgenesia of the corpus callosum. This is the first report of vermian hypoplasia in cri-du-chat disease. We suggest that the most likely pathogenesis of this malformation is a midline dysraphia.

A patient with unusually prolonged epileptic aphemic attacks following a surgical lesion in the left frontal lobe is described. Clinical, electroencephalographic and neuropsychological findings are reported. True aphemia is relatively rare and the described case affords the opportunity of emphasizing that a relatively rare disorder of language as aphemia might also occur with true epileptic attacks.