-We report on the clinical characteristics of amyotrophic lateral sclerosis (ALS) in Fortaleza (Northeastern Brazil). For this, we analyzed retrospectively (from 1980 to 1999) 78 cases of ALS from the Service of Neurology of the University Hospital of Fortaleza diagnosed clinically and laboratorially (EMG, muscle biopsy, myelography, blood biochemistry, muscle enzymes and cranio-cervical X-ray). The results showed that they were mostly sporadic ALS (76/78), and they were divided into definite (n= 36), probable (n= 20), possible (n= 15) and suspected (n= 7), according to the level of diagnostic certainty. They were also subdivided into juvenile (n= 17), early-onset adult (n= 18), age-specific (n= 39) and late-onset (n= 4) groups. Clinically, they presented as initials symptoms, principally, asymmetrical (30/78) and symmetrical (24/78) weakness of extremities, besides bulbar signs, fasciculations, and atrophy. Curiously, pain as first symptom occurred in an expressive fashion (17/78). The predominant initial anatomic site, in this series, was the spinal cord, and mainly affecting the arms. As to the symptom accrual from region to region, this occurs more quickly in contiguous areas, and fasciculations are predominant when bulbar region was associated.KEY WORDS: ALS, sporadic, early-onset, pain, anatomy, symptom accrual, fasciculations. Esclerose lateral amiotrófica: análise clínica de 78 casos de Fortaleza (Nordeste Brasileiro)RESUMO -Descrevemos as características clínicas da esclerose lateral amiotrófica (ELA) em Fortaleza (Nordeste Brasileiro). Para isso, fizemos uma análise retrospectiva (de 1980 a 1999) de 78 casos de ELA do Serviço de Neurologia do Hospital Universitário de Fortaleza, diagnosticados do ponto de vista clínico e laboratorial (EMG, biópsia de músculo, mielografia, análises hematológica e bioquímica, e raio X da junção crânio-cervical). Os resultados mostraram que esses casos eram principalmente da forma esporádica (76/78), e estes foram divididos, de acordo com o nível de certeza diagnóstica, em definidos (n= 36), prováveis (n= 20), possíveis (n= 15) e suspeitos (n= 7). Eles foram também subdivididos nos grupos juvenil (n= 17), adulto jovem (n= 18), idadeespecífico (n= 39) e tardio (n= 4). Clinicamente, os principais sintomas iniciais foram fraqueza assimétrica (38/ 78) e simétrica (24/78) das extremidades, além de sinais bulbares, fasciculações e atrofia. Curiosamente, dor como sintoma inicial ocorria de maneira expressiva (17/78). O local anatômico inicial predominante foi, nessa série, a medula espinhal, afetando principalmente os braços. O avanço regional de sintomas ocorreu mais rapidamente em áreas contíguas, e as fasciculações eram predominantes quando a região bulbar estava associada. PALAVRAS-CHAVE: ELA, esporádica, início precoce, dor, anatomia, avanço dos sintomas, fasciculações.Amyotrophic lateral sclerosis (ALS) is a condition of undetermined cause, which affects the anterior horn cells and the corticospinal tract.The etiology is manifold so that different causes have been...

Pure neural leprosy (PNL) is often difficult to diagnose when acid-fast bacilli (AFB) cannot be detected. We undertook the present study to evaluate use of the polymerase chain reaction (PCR) in diagnosing PNL. Fifty-eight patients (41 men and 17 women) suspected of pure neural leprosy (PNL) were examined. Patients were classified as borderline tuberculoid (BT, 40 cases) and polar tuberculoid (TT, 18 cases) types. Nerve biopsy was performed and was positive for AFB in 20 patients (all BT patients), i.e., 34.5% of total cases. DNA was extracted from the nerve biopsy samples and amplified using PCR for a specific repeated sequence of DNA from Mycobacterium leprae. PCR analysis was positive in the nerve samples from 29 patients (50%), 27 of the BT type, and 2 of the TT type patients. Further, PCR analysis was positive in 14 of 38 cases that were negative for AFB by nerve biopsy, of which 12 were of the BT type and 2 the TT type. PCR analysis proved to be a useful method to investigate pure neural leprosy, enabling confirmation of the diagnosis in more than a third of the cases that were negative for AFB by nerve biopsy.

The aim of this study was to describe the results of a Brazilian Consensus on Small Fiber Neuropathy (SFN). Fifteen neurologists (members of the Brazilian Academy of Neurology) reviewed a preliminary draft. Eleven panelists got together in the city of Fortaleza to discuss and finish the text for the manuscript submission. Small fiber neuropathy can be defined as a subtype of neuropathy characterized by selective involvement of unmyelinated or thinly myelinated sensory fibers. Its clinical picture includes both negative and positive manifestations: sensory (pain/dysesthesias/pruritus) or combined sensory and autonomic complaints, associated with an almost entirely normal neurological examination. Standard electromyography is normal. A growing list of medical conditions is associated with SFN. The classification of SFN may also serve as a useful terminology to uncover minor discrepancies in the normal values from different neurophysiology laboratories. Several techniques may disclose sensory and/or autonomic impairment. Further studies are necessary to refine these techniques and develop specific therapies.

Twenty four cavernous sinuses from tufted capuchin monkey (Cebus apella) were studied throughout gross anatomy, stereoscopic and microscopic serial analysis. The results could show us that the cavernous sinus in monkeys is similar to the other primates, including man. Its pattern is the same as the venous channels located between the dura mater layers. It is attached by trabeculae in the intracavernous portion of the internal carotid artery. Its lateral wall is formed by two layers of connective tissue. The deep layer is continuous with the oculomotor, troclear and ophthalmic sheath nerves. The abducent nerve runs almost all the time between the internal carotid artery and the cranial nerves placed on the lateral wall. Their trabeculae are ensheated by endothelium that divides the sinus in superior, lateral and medial venous spaces in relation to the internal carotid artery. There are communications among them in the intercavernous sinus named anterior, inferior and posterior sinus. The internal carotid artery presents a foreward ascendent retiform course until the anterior segment when it bents abruptly toward the roof of the cavernous sinus. Bundles of the nerve fibers in the trabeculae are greater in number between the internal carotid artery and the abducent nerve. These trabeculae exhibit perikaryon of neurons near the lateral wall and between the abducent and ophthalmic nerves.KEY WORDS: dura-mater, cavernous sinus, cranial nerves, internal carotid artery, Cebus apella. EDUARDO CARLOS DA SILVA** A hundred patients underwent microscopic removal of unilateral lumbar herniated disc in a single level. * Contribuição ao estudo anatômico do seio cavernoso do macaco-prego, Cebus apella (ResumoThe results were: good in 86%, regular in 12% and bad in 2%. Hospital staying after surgery was: 77% just for one day; 12% for two days, 10% for three days and 1% for four days.Selected patients after lumbar herniated disc microsurgery performed by a skillful surgeon may show positive results such as: short hospital staying: a day or less, minor complications, early walking and quick returning to daily activities.

Objectives– To study the effectiveness of thymectomy (TY) in a group of patients with myasthenia gravis compared to a group of patients submitted to conservative treatment (CT) at a similar clinical stage. Methods – Among 153 patients with myasthenia gravis, we paired 28 patients who underwent TY, with 28 cases under CT. The following data were analyzed: gender, age, and age at the beginning of symptoms, illness duration, follow‐up time and type of medical treatment. There was no statistical difference between these 2 groups. The mean time for TY was 2.5 (0.2‐13) years after the onset of the disease. The cases were evaluated through a functional scale at the beginning and at the end of the study. Results – We found complete remission in 15 cases (TY 6, CT 9), improved (normal life with or without minimal symptoms and with or without medication) 9 cases (TY 8, CT 1), improved with partial control and minimal limitation 32 cases (TY 14, CT 18), and poor control 2 cases (TY 2). No death was found in this group. Conclusion – There was no statistical difference between the conservative treatment and thymectomy groups, regarding remission or improvement. Furthermore TY done in the first year of the disease or latter, did not change the ®naloutcome.