Neuropathologic and morphometric effects of aminoguanidine on rat nerves.

Aminoguanidine prevents some pathophysiologic changes typical of streptozocin diabetes and, therefore, might be efficacious in prevention or treatment of human diabetic polyneuropathy. In order to evaluate the possible toxicity of aminoguanidine on peripheral nerves, Sprague-Dawley rats received aminoguanidine intraperitoneally in dosages of 0, 50, 100, and 300 mg/kg per day for 3 months. Only rats receiving the highest dosages developed acute and chronic behavioral changes and had decreased weight gain. Minor hepatic dysfunction also was observed in this group. Teased-fiber abnormalities were not significantly more frequent in the highest dosage group than in controls. Likewise, a significant morphometric abnormality was not found for the peroneal nerve. Mild changes were found in the highest dosage group compared to the control group in the sural nerve (increased fiber density, decreased myelin area). We interpret the small morphometric differences for the sural nerve as due to maldevelopment. We found no evidence that aminoguanidine at a high dosage (300 mg/kg per day) caused fiber degeneration or demyelination.

Arterial graft reconstruction in an aneurysm of the middle cerebral artery.

A case of a large aneurysm of the middle cerebral artery that was treated by resection of the aneurysm sac and reconstruction of the vessel is presented. The middle cerebral artery was reconstructed using a graft from the superficial temporal artery (STA). Cerebral vessel reconstructions are usually carried out with veins as grafts. This paper shows the possibility of using the STA as a graft. When a vessel reconstruction is contemplated, the skin flap should be planned in order to save the STA.

Esthesioneuroblastoma with intracranial extension.

The authors present five cases of olfactory neuroblastoma with intracranial extension operated on in the Department of Neurosurgery in collaboration with otorhinolaryngologists. This tumor is most frequently reported as growing inside the nasal cavities, and it can extend to the paranasal sinuses. Cases presenting initially as intracranial tumors have been infrequently reported. At present, histological diagnosis of this tumor is aided by the use of electron microscopy and immunocytochemistry. Total resection combined with radiation therapy remains the most satisfactory treatment.

Aseptic meningitis in a large MMR vaccine campaign (590,609 people) in Curitiba, Paraná, Brazil, 1998.

The aseptic meningitis after Measles-Mumps-Rubella vaccine (MMR) is a well recognized complication, and different incidences have been observed in several studies. We retrospectively analyzed forty cases of aseptic meningitis, during a large public immunization campaign (1998) in Curitiba, Southern Brazil (590,609 people), admitted in our Service. The vaccine utilized was Leningrad-3-Zagreb mumps strain, Edmonston-Zagreb measles strain, and RA 27#3 rubella strain. In all county, a total number of 87 cases were reported, resulting in a incidence of 1.7 cases per 10,000 given doses. The mean age was 23.7 +/- 12.8 years. The female:male ratio was 1.35:1. Severe headache with meningismus (92.5%), fever (87.5%), nausea/vomiting (82.5%) were the most common clinical findings. Three cases (7.5%) developed mild mumps. All patients underwent cerebrospinal fluid (CSF) tap with the following findings: mononuclear pleocytosis from 100 to 500 cells/mm(3) in 17 cases (42.5%; 257.5 +/- 260.6 cells/mm3); increased protein 28 cases (67.5%; 92.1 +/- 76.9 mg/dL); glucose was normal in all cases (56.8 +/- 11.2 mg/dL) except in 4 (10%) cases, which presented less than 44 mg/dL. All serological tests (latex to bacterial meningitis, Cryptococcus, cysticercosis, VDRL) and bacteriological cultures were negative. Virus identification were also negative in 8 samples. None of the patients had neurological deficits or related symptoms after one year of onset. We believe the benefit of vaccination clearly outweighs the incidence of benign vaccine-associated meningitis.

Epilepsy is a disease!

According to the definition of disease, epilepsy shall not be considered neither a symptom nor a syndrome. Epilepsy is a generic term for a group of diseases characterized by seizures. It implies a state quite distinct from health. Therefore it seems worthy to keep epilepsy as such in the International Classification of Diseases (ICD).

Etiology of epilepsy. A prospective study of 210 cases.

The objective of this study was to establish the etiology of epilepsy in 210 chronic epileptics (110 female, 100 male), aged 14-82 years (34.2 +/- 13.3). Patients less than 10 years-old and alcoholism were excluded. All underwent neurological examination, routine blood tests, EEG and CT-scan. Twenty patients (10.5%) were submitted to spinal tap for CSF examination. Neurological examination was abnormal in 26 (12.4%), the EEG in 68 (45.5%), and CT-scan in 93 (44.3%). According to the International Classification of Epileptic Seizures (1981), 101 (48.1%) have generalized seizures, 66 (31.4%) partial seizures secondarily generalized, 25 (11.8%) simple partial and complex partial seizures, and 14 (6.6%) generalized and partial seizures. Four patients (2.0%) could not be classified. In 125 (59.5%) patients the etiology was unknown. Neurocysticercosis accounted for 57 (27.1%) of cases, followed by cerebrovascular disease 8 (3.8%), perinatal damage 5 (2.4%), familial epilepsy 4 (1.9%), head injury 4 (1.9%), infective 1 (0.5%), and miscelanea 6 (2.8%).

[Classification of hereditary cerebellar ataxias]. / Classificação das ataxias cerebelares hereditárias.

The hereditary cerebellar ataxias are one of the most complex group of neurogenetic diseases. A review of the several attempts do classify the heredoataxias is presented. The classifications based on clinical and genetic grounds are the most useful. The recent advances in molecular genetics are prone to give important clues for elucidation and understanding of the pathogenesis of heredodegenerative cerebellar diseases.

Frontal lobe syndrome from bilateral globus pallidus lesions. A complication of Wernicke's encephalopathy.

A 38 year-old man developed the classical clinical picture of Wernicke's encephalopathy as a consequence of prolonged total parenteral nutrition. As a late complication he developed a frontal lobe syndrome. Bilateral globus pallidus lesions were observed in the CT-scan examination. Some aspects related to the cortical syndromes caused by subcortical lesions are discussed.

[Wilhelm Conrad Röntgen: 100 years of the discovery of X-rays]. / Wilhelm Conrad Röntgen. 100 anos da descoberta dos Raios X.

One hundred years after its discovery, one can hardly conceive the medical practice without the existence of X rays. A brief account of X rays discovery and about it notable discoverer is presented.

[Hereditary cerebellar ataxias: from hammer to genetics]. / Ataxias cerebelares hereditárias. Do martelo ao gen.

The hereditary ataxias comprise a complex group of neurological disorders involving the cerebellum and its connections. Several classifications based on clinical and/or pathological data have been only partially successful. Recent progress in molecular genetics has identified the genic loci of hereditary ataxias and has allowed a more precise diagnosis of distinct genetic diseases. Trinucleotide repeat expansions has been recognized as a mechanism of disease in some autosomal dominant spinocerebellar ataxias (ADCA) (SCA1 to SCA7), including Machado-Joseph disease/SCA3, probably the most common form of ADCA in South Brazil, and Friedreich ataxia (GAA expansion-chromosome 9p). Familial alpha-tocopherol deficiency (chromosome 8q) may have a Friedreich ataxia phenotype and responds to the oral supplementation with vitamin E. Familial episodic ataxias with (EA1-chromosome 12p) and without (chromosome 19p-EA2) myokimia were identified, the first one caused by point mutations in the gene encoding the KCNA1 potassium voltage-gated channel. The gene responsible for ataxia-teleangiectasia (chromosome 11q) was found to encode a putative DNA binding protein kinase (ATM), related to the cell cycle control. One to 3% of the population are heterozygotic ATM gen carry and pose a higher risk of cancer when exposed to ionizing radiation. Molecular biology has provided us with useful tools to diagnosis and genetic counseling and, hopefully, will provide us with a better understanding of the pathogenesis and eventual treatment of the several forms of hereditary ataxias.

Primary lateral sclerosis. A case report with SPECT study.

Primary lateral sclerosis (PLS) is a neurodegenerative disease with progressive corticospinal involvement and characterized by lower limbs spasticity followed by upper limbs involvement,rare cranial nerve involvement, typical sparing of all sensory modalities, sphincteric function and eventually mild cognitive changes. The authors report a case of PLS in a 43-year-old woman with 3 years of clinical follow-up and extensive laboratory investigation, including a SPECT study which disclosed bilateral frontal motor area hypometabolism. Several aspects about this unique disease were reviewed,including differential diagnosis with other more common neurological disorders.

Electrocardiographic findings in acute cerebrovascular hemorrhage. A prospective study of 70 patients.

Seventy patients with hemorrhagic stroke were prospectively evaluated regarding the electrocardiographic abnormalities observed within the first 48 hours of the ictus. Group I comprised 55 patients with spontaneous cerebral hemorrhage, and group II 15 patients with subarachnoid hemorrhage. Patients taking cardiac drugs (beta blockers, calcium-channel blockers, inotropic drugs) or with severe metabolic/electrolyte disturbances were excluded. The most common ECG abnormality was a prolonged Q-Tc interval: group I, 37 (67.2%); group II, 8 (53.3%). Only 4 (7.2% patients of group I and no patient of group II had a normal ECG. No relation was found between the site of the intracerebral hematoma and the occurrence of any particular ECG change. A prolonged Q-Tc may be related to the development of severe cardiac arrhythmias observed in some patients with acute cerebral hemorrhage.

[Genetic aspects in multiple sclerosis: I. Twin studies]. / Aspectos genéticos da esclerose múltipla. I. Estudos em gêmeos.

The authors make a review and critical analysis of twin studies in multiple sclerosis (MS). The concordance rate among homozygotic twins is significantly greater than in heterozygotic twins. The average hertability in MS is about 28%; no single gene was identified as a sole risk factor for the development of the disease. MS may be considered a polygenic disease triggered by environmental factors in a genetically predisposed individual.

[Genetic aspects in multiple sclerosis. II: HLA system]. / Aspectos genéticos da esclerose múltipla. II. Sistema HLA.

Review of studies about HLA antigens and multiple sclerosis (MS). The HLA system, in special class II antigens, subregions DR and DQ, is probably involved in the immunopathogenesis of MS. Haplotype DRB1*1501.DQA1*0102.DQB1*0602, corresponding to phenotype DR2.Dw2.DQ6, is positively associated with MS in several caucasoid populations. Clinical heterogeneity of MS, as well as different diagnostic criteria adopted by investigators are potential sources of confusion and may lead to discrepant results. A better standardization of clinical and laboratorial methodology, appropriate subdivision of patients with different clinical forms of MS, may allow a more accurate evaluation of the role of genetic factors in the pathogenesis of MS.

Brainstem cysticercosis simulating cystic tumor lesion. A case report.

The authors report the case of a 37 year-old man with a solitary cysticercus cyst in the brainstem (pons) successfully removed through a suboccipital craniectomy. Surgery in neurocysticercosis has been indicated in patients with hydrocephalus and/or large cystic lesions. Cystic lesions in the brainstem and spinal cord may have indication for surgery for two reasons: (1) diagnosis; and (2) treatment. Aspects related to differential diagnosis and therapeutic alternatives are discussed.

[Chronic muscular dysfunction of the bulbar innervation in hyperthyroidism: report of a case]. / Disfunção crônica da musculatura de inervação bulbar no hipertireoidismo: relato de caso.

The authors report a case of chronic bulbar muscular dysfunction in a thyrotoxic man, with complete remission of the symptoms after the use of propranolol, with and without concomitant use of propylthiouracil. Some aspects of this unique complication of hyperthyroidism are discussed.

Guillain-Barré syndrome after varicella-zoster infection. Case report.

The authors report a case of Guillain-Barrè syndrome (GBS) following a varicella infection in a 13 year-old boy. During his admission he developed respiratory insufficiency and dysautonomic events, as well as a severe sensitive peripheral neuropathy. Some aspects related to the etiology and pathogenesis of GBS are discussed.

Neurocysticercosis: an epidemiological survey in two small rural communities.

The authors describe the epidemiological findings related to human taeniasis and cysticercosis, and swine cysticercosis in two small rural communities, Postinho (P) and Tigre (T), of South Brazil. The prevalence of epilepsy was 2.04% (P) and 2.25% (T). The prevalence of neurocysticercosis was 0.47% (P) and 0.93% (T), and prevalence of swine cysticercosis was 12.8% (P) and 27.8% (T). Taenia sp. infestation was detected in 4.3% (P) and 4.6% (T) of stool examinations. The hyperendemic human taeniasis and cysticercosis and swine cysticercosis seems to be related to poor hygienic habits of the population, and the free access to human excreta by the pigs.

Severe optochiasmatic arachnoiditis after rupture of an internal carotid artery aneurysm.

The case of a 24-year-old man with progressive visual loss due to optochiasmatic arachnoiditis is presented. The cause of the arachnoiditis was subarachnoidal bleeding due to rupture of an internal carotid artery aneurysm. The aneurysm was clipped 5 years after the first episode of bleeding. The diagnosis of optochiasmatic arachnoiditis was confirmed during the operation. This case is presented in order to discuss the causes, the symptoms and the therapeutical possibilities of this rare condition.

[Machado-Joseph disease: description of 5 members of a family]. / Doença de Machado-Joseph: descrição de cinco membros de uma família.

The authors report the clinical and laboratorial findings of 5 affected members (all males) of a family with Machado-Joseph disease. The mode a inheritance was autosomal dominant. The mean onset age was 38 years (range 30-50 years). The clinical picture was pleomorphic and included cerebellar ataxia, external ophthalmoplegia with bulging eyes, extrapyramidal/pyramidal syndromes, amyotrophy with fasciculations and peripheral neuropathy, in variable degrees of severity. In one patient parkinsonian rigidity was greatly improved with the use of trihexaphenidyl and L-dopa. CT scan examinations disclosed a variable degree of cerebellar atrophy, with mild cerebral atrophy in one patient. Brainstem evoked potentials were normal in two patients. EMG showed denervation in three patients. Muscle biopsy (gastrocnemium) with histochemical studies revealed chronic muscle denervation in four cases. Sural nerve biopsy with conventional pathological study was normal in four cases. This family was living in Florianopolis, Santa Catarina, where there is a great number of Portuguese descendants from the Azores Islands. The worldwide presence of the disease seems to result from the genic diffusion of the disease with the Portuguese emigration during the Great Navigations Era and with some later emigratory settlement.