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Introduction: Multiple sclerosis (MS) is one of the most common diseases of the central nervous system (CNS) in young adults. MS is the most common disorder of the central nervous system in young people living in temperate climate regions. Although a few references to possible cases of the disease come from the xiii century, its scientific observation and systematic study only started in the late xix century., Development: Robert Carswell e Jean Cruveilhier were the first investigators to document the pathological lesions while the clinical picture was first studied by Charcot. In spite of a huge number of infectious agents has been proposed for the etiology of MS and a genetic susceptibility trait recently defined, the ultimate cause of the disease remains to be determined. The development of diagnostic criteria sets, clinical disability scales and image methods in the latter half of the last century has provided investigators with useful research tools allowing unprecedented advances. In the last 30 years ACTH and corticosteroids have been employed as treatment for MS relapses. Starting in 1993 a new class of drugs called disease modifying agents, such as interferon beta and more recently glatiramer acetate, was introduced with encouraging results., Conclusions: MS is postulated to be a cell mediated autoimmune disease directed against CNS myelin components and characterized by inflammation and chronic demyelination. This paper is a review of the principal most significant events in the search for knowledge of the disease in the world.

Objectives: To assess the epidemiologic characteristics of amyotrophic lateral sclerosis (ALS) in Brazil in 1998., Method: Structured Clinical Report Forms (CRFs) sent to 2,505 Brazilian neurologists from January to September 1998 to be filled with demographic and clinical data regarding any ALS patient seen at any time during that year., Results: Five hundred and forty CRFs were returned by 168 neurologists. Data on 443 patients meeting the criteria of probable or definite ALS according to El Escorial definition were analysed: 63 probable (14.2%) and 380 definite (85. 8%). Two hundred and fifty-nine (58.5%) of the patients were male, mean age of onset was 52. Spinal onset occurred in 306 patients (69%); bulbar onset in 82 (18.5%), and both in 52 (11.7%). Twenty-six (5.9%) had a family history of ALS. Two hundred and fifty-nine (58.6%) were seen by private practitioners, and 178 (40. 2%) at a hospital clinic. Age-ajusted incidence shows a peak incidence at the 65-74 years old range., Conclusions: The disease's characteristics are similar to those described in international studies, except for age of onset (Brazilian patients are younger). This difference is not confirmed when figures are age-adjusted.

Five patients with a tumefactive lesion were clinically followed from 1992 to 1993. Four patients were female; age ranged from 32 to 57 years, the duration of symptoms varied from 3 days to 3 years. Neurological examination disclosed dementia in two patients, aphasia in three, hemiparesis in four, hemihypoaesthesia in three, optical neuritis in two, tetraparesis with sensitive level and neurogenic bladder in one. MRI disclosed lesions with a hypersignal on images assessed at T2 and hyposignal at T1, and gadolinium heterogeneous enhancement; these lesions were located in the: a) temporooccipital region bilaterally and brain stem, b) frontoparietal white matter, c) basal ganglia, bilateral white matter and brain stem, d) left parietal region, e) cervical spinal cord, with enlargement of this region. Cerebral biopsy was performed in three patients; acute and subacute demyelinating disease was diagnosed by histological examination. Two patients had an evolutive diagnosis; exclusion of other pathologies and clinical and radiological improvement after corticotherapy, pointed to an inflammatory disease.

Creutzfeldt-Jakob disease (CJD) is a transmissible disease of the nervous system causatively related to the presence of an abnormal prion protein, with dementia, myoclonic jerks, and periodic EEG activity. Fourteen patients (7 females and 7 males) ranging from 26 to 76 years of age (median 59 years) were evaluated between 1974 and 1995 at the Neurologic Clinic of São Paulo University School of Medicine. The average duration of the disease was 12 months (3.5-34 months). Early clinical findings were: behaviour changes in 7 patients, dementia in 4, visual disturbances in 4, vertigo in 2, tremor in 9, and dystonia in one. Advanced symptoms were dementia and myoclonus in all patients. Pyramidal tract dysfunction was found in 6, cerebellar ataxia in 2, seizures in 3, nystagmus and vertigo in 4, and peripheral nervous system involvement in 2. Atypical clinical forms were found in 5 patients. Periodic EEG activity was found in 10 patients. Cerebrospinal fluid evaluation showed pleocytosis in 1 patient, higher protein content in 2, and higher gamma globulin level in 2. In 10 patients anatomopathological evidence in the central nervous system confirmed the clinical diagnosis by presenting with status spongiosus. All except one patient presented with the sporadic form of the disease.

We present clinical data from 14 multiple sclerosis (MS) patients who have been admitted to our hospital between January 1980 and May 1992, whose age of onset ranged from 2-15 years. Our patients could be classified as having a clinically definite form of the disease. Initial symptoms varied from minor, such as motor or sensory impairment, bladder dysfunction, to the worst clinical presentation, suggesting diffuse encephalopathy. All the patients had a relapsing-remitting course. We report the paraclinical and laboratory examinations that were done in these patients. Over the period 1980 to 1992 these patients had 39 attacks. CSF analysis was performed in the phase of activity of the disease on 23 occasions and was normal in 12. At least one brain CT scan was performed in 9 patients and showed white matter abnormalities in 6. Cranial magnetic resonance imaging was done in 6 patients and were abnormal in 5. Visual evoked potential (EP) was abnormal in 7 of 8 patients; brainstem acoustic EP was abnormal in 4 of 8 patients and somatosensory EP in 4 of 8. MS is not so rare in childhood and although its diagnosis is essentially a clinical one, paraclinical investigations are of great value in the identification of demyelinating disorders in childhood.

The role of the nursing staff (which was then not professionally trained) in psychiatric institutes was limited to ensuring that the rules of the institute were respected. After the responsibility for providing assistance had been transferred to Local Psychiatric Services, the role of the professional nurse was radically changed both as a result of the changes in type of patient and the new manner of caring for patients, above all the operational methods used stress the importance of treatment in relations with the patient. The paper analyses the various tasks of the psychiatric nurse which include the initial reception given to the patient, the administration of drugs and evaluation of their effects, the establishment of educational relations with the patient and management of rehabilitation programmes. Whereas the doctor plays a father-role towards the patient due to the authority he possesses, the nurse may be characterised by maternal and caring functions. Given that the lack of theoretical instruments, as possessed by psychiatrists and psychologists, and prolonged contact with patient may expose nursing staff to the risk of damaging their own mental health, it is important to give them specific professional training which will help them handle their relations with psychotic patients.

A case register of multiple sclerosis (MS) was set up in the city of São Paulo, Brazil, situated at a latitude of 23-24 degrees south. Cases were notified by neurologists, neuroradiologists and by an association of patients with MS. Prevalence rate was 4.27 x 10(-5) (men: 2.89 x 10(-5); women: 5.59 x 10(-5)), with a preponderance of young adults. We discuss here whether these rates are artifact due to poor health care facilities, to poor notification and deficiencies in diagnosis, or whether these rates are really low.

Immunoglobulins isotypes (IgG and IgM) for myelin basic protein (MBP), cerebrosides (CER), gangliosides (GANG) and cardiolipin (CARD) were detected in the cerebrospinal fluid (CSF) from 33 patients with multiple sclerosis (MS), 18 with Guillain-Barré syndrome (GBS) and 30 with systemic lupus erythematosus (SLE). In MS patients occurred positive and significant levels of IgG-MBP in 51.5% (p less than 0.05) and IgM-MBP in only 18.2%, IgG-CARD in 46.2%, as long as CER and GANG were detected in almost 20%. From serum samples of MS patients 20.6% presented IgG-MBP, while 53% showed positive levels for IgM-MBP. The CSF analysis of patients with GBS showed that 56.3% revealed IgG-MBP (p less than 0.05), 53% for IgM-MBP, 38.5% for IgG-CER and 23% for IgM-CER, while 50% of patients had IgG-CARD, as long as 31% also had IgG-GANG. The serum evaluation from 14 patients showed that 18.8% had positive concentrations of IgG-MBP and 56.3% presented IgM-MBP (p less than 0.05). Except for 50% of patients with SLE who presented positive CSF levels of IgG-CARD, only 24.1% had positive levels of IgG-MBP. We believe that the presence of antiphospholipid antibodies in CSF of the above mentioned diseases occurred as immune epiphenomena, but their appearance would permit the maintenance of and perpetuate the immune event.

A 60-year-old woman developed progressive spastic crural paraplegia and global anesthesia and global anesthesia below T10, succumbing to urinary infection and bronchopneumonia 8 months after the onset of symptoms. At necropsy, the spinal cord was completely destroyed by a necrotic intramedullary cysticercus at T8. Six cysticerci were found in the brain. A review of the 17 published cases of intramedullary cysticercosis (including this one) showed this condition to be clinically indistinguishable from spinal cord tumours. The surgical prognosis was fair in 8 of 11 operated patients. The role of hematogenous and ventriculo-ependymal pathways in the pathogenesis of intramedullary cysticercosis was examined. The topographical distribution of intramedullary cysticerci (5 cervical, 12 thoracic, 2 lumbar, none sacral) was found to be statistically proportional to the blood flow to each of these regions; this favours the hematogenous route of infestation. The low spinal cord blood flow (100 times less than that to the brain), the type of vascularization of the cord (low calibre vessels under low pressure) and peculiarities of the cord tissue (such as its harder consistency) are all thought to be contributory factors accounting for the scarcity of intramedullary cysticerci. No evidence for an ependymal route of spread could be adduced.

The authors studied 8 patients with Familial Periodic Paralysis on the clinical, hydroelectrolytic, electrophysiologic, histologic and therapeutic points of view. There was significant predominance in males. The onset of the symptoms in our group began under fifteen years of age. The clinical manifestations were similar to those referred in the literature. The hypokalemic form was found in all patients except in one case that had hyperkalemic form. Natremias on the superior border of the normality, were seen in 21.4% of the cases, and in 21.4% the natremias were higher; this fact suggested aldosterone liberation of the muscle. The histologic examination performed in 5 patients, was normal in 20% of them and, in those cases with repetitive and frequent crises, atrophic muscle fibers were seen. Prophylactic treatments were made. The patients with hypokalemic form received supplementary doses of potassium.

The Vogt-Kayanagi-Harada sśyndrome in two negro female patients with 37 and 40 year-old, respectively, are reported. The clinic, epidemiologic, heredo-familial and immunologic aspects are discussed.

Three cases of probable neurobrucellosis are reported. The diagnosis was made on the basis of immunological tests. Two patients with a clinical picture of meningomyelitis showed a definitive clinical improvement under tetracycline and streptomycin therapy. The immunological reactions found in the record case were even more positive in the spinal fluid than in the blood. In the case 3 with a clinical presentation of cerebral hemorrhage the histopathological studies demonstrated non specific chronic leptomeningitis and local hemorrhages in the caudate nucleus bilaterally. The diagnose and treatment of neurobrucellosis are discussed, stressing the importance of an early therapy.

The case of 15 years old boy with an enterogenous cyst causing compression of the spinal cord at T-2 is reported. The diagnosis was made by thoracic laminectomy and confirmed by histological examination. The motor disorders improved progressively after surgery.

The case of a man 34 year-old presenting upward gaze paralyses and external ophthalmoplegia without involvement of the downward gaze, accompanied by cerebellar disorders, generalized areflexia but no muscular weakness, and moderate sensory impairment of the four extremities is reported. The cerebrospinal fluid showed an albuminocytologic dissociation. Recovery was rapid and almost complete, within 3 weeks, remaining minimal paresis of the upward gaze and lateral movement in both eyes. The improvement had been coinciding with the treatment by cortrosina.

The case of a 28-year-old male affected by intracranial tuberculous arachnoiditis with unusual aspects is reported. The patient presented bilateral amaurosis and complete ophthalmoplegia with intrinsic muscle sparing in both sides. The routine laboratory findings showed only persistent leucocytosis and a Mantoux test of 10 mm. Several radiologic studies of the lungs were always normal. The spinal fluid, tomography of the cranial base and carotid angiography were normal. The diagnosis was made by histologic study after craniotomy.

Two cases of patients with relapsing polyradiculoneuropathy with high protein level in cerebrospinal fluid are reported. The immunological features and the natural history of the relapsing polyradiculoneuropathy are discussed. This disease is considered a particular auto-immune nosologic condition, independent from acute polyradiculoneuropathy.

Sheathing of peripheral retinal veins occurs in 10 to 20% of patients with multiple sclerosis and can be seen at any point in the progression of the disease. This finding may represent the retinal correlate of the vascular lesions that are present in the central nervous system of patients with multiple sclerosis. Its pathogenesis is still uncertain. In this paper it is registered the occurrence of retinal periphlebitis as one of the initial manifestations of a patient with multiple sclerosis and it is discussed the diagnostic implications of this finding.