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Middle-age Down's syndrome patients develop dementia with antomicopathological changes that are characteristic for Alzheimer's disease. Cerebral amyolid angiopathy (CAA) most commonly manifests itself as a lobar intracerebral haemorrhage that tends to recur. Multiple haemorrhages may occur simultaneously. CAA is frequently associated with Alzheimer's disease, however the relationship between CCA and Down's syndrome is poorly know. Although there are established clinical criteria for the diagnosis of CAA related to lobar intracerebral haemorrhage, definitive CAA needs anatomicopathologial confirmation. This paper presents the case of a male with Down's syndrome and recurrent lobar intracerebral haemorrhage. An autopsy showed lesions in leptomeningeal and cortical arteries which are characteristic of CAA in addition to anatomicopathological changes of Alzheimer s disease that were mostly seen in entorhinal cortex and hippocampus. The paper also discusses the pathological association between beta-amyloid protein, apolipoprotein alleles, CAA, Alzheimer's disease and Down's syndrome.

We present a case of encephalitis caused by Herpes Simplex Virus in a 57 year old woman. The acute picture was suggestive of viral infection without associated neurological symptoms. Due to the posterior appearance of neurological focality, cerebral spinal fluid (CSF) was analyzed. It showed pleocytosis and lymphocytosis, inflammatory process data, and serological test with positivity for Simple Herpes Virus (SHV) subtypes I and II. During admission, other complementary tests were performed: EEG, CT, MRI, cerebral perfusion SPECT; the later supplied significant data regarding anatomical neuroimaging (CT, MRI) in regards to bihemispheral extension of the encephalic condition. Furthermore, after clinical discharge, persistent metabolic abnormality was demonstrated in temporal cortex, responsible for concomitant mixed aphasia.

Objective: To analyze the experience in daily clinical practice of interferon-beta (IFN-beta) treatment in relapsing-remitting (RR) and secondary progressive (SP) multiple sclerosis (MS) in Galicia (Spain)., Patients and Methods: Patients with RR-MS and SP-MS treated with IFN-beta1a and 1b between 1995 and December/2000, analyzing demographic and clinical data., Results: 313 patients were included, with a mean age of 38.2 years. A total of 296 patients (94.6%) were clinically defined MS and 17 (5.4%) were laboratory supported (Poser criteria); 84.6% of the patients were RR and 15.4% were SP. The mean duration of the disease prior to treatment was 7.06 years. Betaferon was used in 52.4% patients (115 RR-MS and 47 SP-MS), Avonex in 26% and Rebif in 21.6%. Relapse rate was reduced in 68.8% for the RR-MS for Betaferon-treated patients, 73.3% for Avonex treated and 35.7% for Rebif-treated patients. Betaferon reduced relapse rate in 50% for SP-MS. The global EDSS remained stable during IFN-beta treatment. During treatment, 33% of Betaferon, 60.5% of Avonex and 54.5% of Rebif-treated patients remained relapse-free. Treatment was suspended in 12.9% of Betaferon, 6.2% of Avonex, and 3% Rebif-treated patients. The most frequent causes of treatment suspension were increase in disability and in relapse count., Conclusions: The present study supports the benefits of IFN-beta treatment in RR MS and SP MS in daily clinical practice, with reduction in relapses count and incapacity, good over-all tolerance and low incidence of serious adverse side-effects.

Introduction: Marchiafava-Bignami disease, cerebral pellagra and alcoholic cerebellar degeneration are a group of diseases included in the alcoholic encephalopathies, although they may also be caused by metabolic or nutritional disorders. The isolated appearance of these diseases usually permits diagnosis during the life of the patient, based on the neuro-radiological findings. However, their combination leads to complex form, with variable neurological expression, which means that precise diagnosis may often be post mortem., Clinical Case: We present a malnourished alcoholic patient with neurological features compatible with alcoholic encephalopathy. The post mortem findings showed lesions typical of alcoholic cerebellar degeneration, cerebral pellagra and Marchiafava-Bignami disease.

Progressive general paralysis, tabes dorsalis and meningovascular syphilis are different manifestations of tertiary syphilis. The clinical picture of meningovascular syphilis is of a subacute or chronic meningeal syndrome. It may be associated with focal neurological signs of cerebral arteritis. Inflammation of the leptomeninges may impede circulation of the cerebrovascular fluid at different levels, giving rise to noncommunicating, or exceptionally to communicating hydrocephalus. Diagnosis of this depends on the clinical signs and neuro-imaging changes. We present the case of a man with meningovascular syphilis who developed clinical signs of normotensive hydrocephalus.

We describe the case of a 61 years-old-woman with lupus erythematosus who presented with neurological symptoms, namely dementia, focality and crisis. The neurological presentation of lupus is evaluated clinically, being similar to the central manifestations of patients with systemic onset. The marked cognitive affectation with relation to other cases of lupus with a neurological onset is emphasized. MR has been shown to be a sensitive means of detection of corticosubcortical lesions of neurolupus, not specific to this condition, as well as to their response to immunosuppressors. The aetiopathogenesis of some of the manifestations of cerebral lupus and the clinical response to corticosteroids and cyclophosphamide is reviewed.

Granulomatous angiitis of the central nervous system is an uncommon condition characterized by vascular wall necrosis, inflammatory exudate and development of giant cells in medium and small size vessels. The pathogenesis of this disease remains unknown, but it has been associated with immune complexes, mechanical factors and infection by the varicella-zoster virus. We report a young patient who presented with herpes zoster involving the VII cranial nerve and contralateral hemiplegia. Subsequently, pontine infarct and fatal subarachnoid hemorrhage developed. The pathological study showed granulomatous angiitis of basilar artery.

The clinical onset of cerebral venous thrombosis (CVT) is very variable and the causes that may lead to its development are also quite numerous. Although characteristic CT diagnostic signs have been described, in most cases only nonspecific findings are present and angiography is required to confirm the diagnosis. The prognosis is variable. Classically, the mortality is high when the deep venous system is involved. We report a male with CVT involving Galen's vein and its major tributary vessels, who had a favorable outcome with heparin therapy and drugs for cerebral edema. We discuss the etiologic factors in this patient, the characteristic neuroradiologic findings, and their evolution during the course of therapy.