Thalamotomy in generalized dystonia.

A prospective long-term semiquantitative evaluation of the results of ventral intermediate-posterior ventral oral nucleus thalamotomy on the different aspects of dystonia was made in 29 patients with secondary disease, 12 with nonfamilial, eight with (non-Jewish) familial, and seven with atypical DMD. The effect of disease progression, even in secondary patients, on surgical outcome was reviewed. Thalamotomy resulted in a long-term improvement in limb function of more than 25% to 50% in 23% of the patients, over 50% in 34% of patients, but midline features responded poorly. Manual dexterity was little changed in secondary cases because of underlying paralysis but improved 38% in cases of DMD. Involvement of neck and trunk, of three to four limbs, and progressive disease prognosticated for a poorer result, but phasic and tonic, familial, and nonfamilial dystonia respond equally well and age at surgery made no difference. Significant complications in 29 secondary cases included one death 31 days postoperative, one case of worsened hemiparesis, two cases of worsened dysarthria, two cases of worsened locomotion, one case of hydrocephalus requiring shunting, and one case of need for permanent tracheotomy. In 27 cases of typical and atypical DMD, there were two instances of hemiparesis, two of significant speech deterioration, three of hand ataxia, one of postoperative seizures, and one of hydrocephalus requiring shunting for an overall significant morbidity rate of 21%. The limiting factor in treating secondary dystonia is the underlying spastic paralysis but that in DMD is the relentless postoperative progression. The overall results of this study are remarkably similar to those of other published series: a quarter of the patients improved by 25% to 50%, a quarter to a third by more than 50%. The analysis of effect on specific features of the disease may be useful in the future for predicting outcome in a particular patient.

Primary intracerebral Hodgkin's lymphoma.

The case is reported of a 51 year old man with primary intracerebral Hodgkin's lymphoma treated by surgical excision, intrathecal chemotherapy and whole-brain irradiation. One year later the patient had no evidence of Hodgkin's lymphoma intracranially or elsewhere. The possible histogenesis of this rare condition is discussed and a brief review of the literature is presented.

Aggressive oncocytic neuroendocrine tumour ('oncocytic paraganglioma') of the cauda equina.

An oncocytic neuroendocrine tumour ('oncocytic paraganglioma') of the cauda equina is reported. The tumour was predominantly intradural, with extension into and destruction of surrounding vertebral bone. The tumour had an organoid pattern, and the tumour cells had abundant non-argyrophilic eosinophilic cytoplasm. Immunocytochemical stains for neurone-specific enolase, S-100 protein, keratin and carcinoembryonic antigen were positive, but stains for glial fibrillary acidic protein were negative. On ultrastructural examination, there were numerous mitochondria and scattered 200 nm dense-core membrane-bound granules, that rarely clustered in small aggregates. Intermediate filaments were focally arranged in long compact bundles. The histogenesis of tumours reported as cauda equina paragangliomas is discussed.

Carotid ultrasonic arteriography combined with real time spectral analysis: a comparison with angiography.

In a one year period, 400 patients were examined by carotid ultrasonic arteriography combined with real time sound spectral analysis. Contrast arteriography was performed on 112 patients. The noninvasive examinations and contrast arteriograms of these patients were reviewed by two independent observers, who were unaware of the findings of the other diagnostic modality. There were 190 vessels available for comparison. The internal carotid artery was classified as normal, less than 50% stenosis, greater than or equal to 50% stenosis, or occluded. The specificity of these non-invasive tests was 79%. The sensitivity in correctly identifying stenoses as less than 50% or greater than or equal to 50 was 85% and 91% respectively. The sensitivity in identifying occluded vessels was 90%. The positive and negative predictive values were 87% and 92% respectively. Noninvasive evaluation correctly identified six lesions misdiagnosed by arteriography. We found pulsed Doppler arteriography combined with real time spectral analysis to be an accurate, direct, noninvasive method of assessing the extracranial carotid arteries.