Successful outcome of episodes of status epilepticus after vagus nerve stimulation: a multicenter study.

BACKGROUND AND PURPOSE: Vagus nerve stimulation (VNS) has been reported to be a safe and effective treatment for drug-resistant epilepsy. The aim of this study is to describe the effect of VNS in patients with a history of repeated episodes of status epilepticus (SE) before implantation. METHODS: From a total of 83 adult patients with drug-resistant epilepsy who had VNS implanted in four tertiary centers in Spain between 2000 and 2010, eight had a previous history of repeated episodes of SE. We performed a retrospective observational study analyzing the outcome of seizures and episodes of SE after implantation. Stimulation was started at the usual settings, and intensity increased according to clinical response and tolerability. RESULTS: Regarding the eight patients with a history of SE, the mean age at time of VNS implantation was 25.1 [14-40] years. Duration of epilepsy until the implantation was 21.7 [7-39.5] years, and they had been treated with a mean of 12 antiepileptic drugs [10-16]. Mean follow-up since implantation was 4.15 [2-7.5] years. Average seizure frequency decreased from 46 to 8.2 per month. Interestingly, four of the eight patients remained free of new episodes of SE after implantation, and in two additional patients, the frequency decreased by >75%. Adverse effects were mild or moderate in intensity and included mainly coughing and dysphonia. CONCLUSION: In those patients with refractory epilepsy and history of SE who are not surgical candidates, VNS is a safe and effective method to reduce seizure frequency and episodes of SE.

A novel mutation (K317M) in the MAPT gene causes FTDP and motor neuron disease.

BACKGROUND: Frontotemporal dementia with parkinsonism is often linked to chromosome 17 and is related to mutations in the MAPT gene. In some families the genetic basis is still unknown. The authors report two pedigrees with FTDP-17 harboring a novel mutation (K317M) in exon 11 in the MAPT gene. METHODS: The authors identified two apparently unrelated pedigrees with an autosomal dominant neurodegenerative condition. Thirteen patients were examined and eight autopsies were performed. RESULTS: Mean age at onset was 48 years. Mean disease duration was 6 years. Dysarthria often heralded the disease. All cases had parkinsonism and pyramidalism and half of them had amyotrophy. Behavioral or personality changes were not a prominent feature. Cognitive decline appeared late in the evolution. Neuropathologically, a massive degeneration of the substantia nigra without Lewy bodies was a constant finding. A variable degree of frontotemporal atrophy was found. Corticospinal tract degeneration and anterior horn neuron loss were present in six of seven autopsies in which the spinal cord was examined. An extensive deposition of abnormal tau protein in a mixed pattern (neuronal, glial) was observed. Pick's bodies were not seen. Biochemical analysis of tau revealed two bands of 64 and 68 kDa. CONCLUSION: Genetic analysis revealed the same novel mutation (K317M) in exon 11 of the MAPT gene in both pedigrees. A common haplotype between members of the two pedigrees suggests that they belong to the same family.

[Insomnia and hallucinations caused by vascular lesions of the pontine]. / Insomnio y alucinaciones tras lesiones vasculares del tegmento protuberancial en el hombre.

Small ischemic vascular lesions or hemorrhages provide a pathological model useful for the study of sleep disturbances in human due to lesions in the pontine tegmentum. We present 7 patients, 4 men and 3 women, with severe insomnia and hallucinations due to small vascular lesions of the pontine tegmentum. Nocturnal-sleep and/or 24-h polygraphic studies were done on all patients, as were neurophysiological and image studies; pathological studies were performed in one patient. Insomnia affected both non-REM and REM sleep, appeared in the acute phase and tended to improve with time. The patients tolerated insomnia with no serious effects on general health. Insomnia was not affected by administration of L-tryptophan with or without carbidopa. Hallucinations were mainly visual, but were also auditory in 2 cases; they were unrelated to the occurrence of normal or dissociated REM sleep. Imaging studies, and autopsy in 1 case, revealed damage to the pontis centralis caudalis and pontis centralis oralis nuclei, which appeared to be responsible for insomnia. The relationship between visual and/or auditory hallucinations and sleep disturbances remain speculative.

[Use of magnetic resonance in the diagnosis of neuro-cryptococcosis in the acquired immunodeficiency syndrome: study of 4 patients]. / Aportación de la resonancia magnética al diagnóstico de la neurocriptococosis en el síndrome de inmunodeficiencia adquirida: estudio de 4 enfermos.

Neuro-cryptococcosis is a common opportunistic infection in AIDS or HIV infected patients. From a series of 10 neuro-cryptococcosis the four of them studied by magnetic resonance (MR) are reported. In AIDS patients a high suspicion of opportunistic infection of the CNS is needed as exemplified by two of the four patients who only presented cephalalgia. The other two patients suffered additional symptoms and signs of meningeal and CNS involvement, such as nuchal rigidity, cranial nerve palsies, papilloedema, gait ataxia and dismetria. Diagnosis was achieved (confirmed) by a positive culture, serology or indian ink test in CSF. CT scan did not contribute to the diagnosis and management of the patients. In contrast MR, showed in three of them a peculiar pattern of small, confluent, high-signal lesions, roughly symmetrically placed in the basal ganglia and the internal capsule. They probably correspond to the dilated Virchow-Robin spaces through which torulae migrate from the subarachnoid space.

[Multifocal encephalopathy as the principle manifestation of an occult cancer]. / Encefalopatía multifocal como principal manifestación de un cáncer oculto.

We present a patient with an occult adenocarcinoma which manifested clinically as a confusional syndrome due to multiple cerebral infarctions associated to pericarditis and immunological abnormalities. Neurological involvement was secondary to nonbacterial thromboembolic endocarditis (NBTE) which did not provoke cardiac murmurs nor was detected in type B echocardiogram. Malignant cells were not observed in the pericardial effusion or in the pericardial biopsy (1.5 x 1 cm). The clinical picture mimicked an atypical lupous syndrome. The positive diagnosis was established on necropsy. The cardiovascular complications are not frequent in cancer. NBTE usually shows up clinically as a neurologic syndrome due to multiple cerebral infarctions: the normality of complementary exams and the lack of demonstration of an underlying disease do not rule out its diagnosis. When suspecting an NBTE treatment with heparin should be promptly started.