We report a case of a 18-year-old female patient that developed a migraine-like headache following an acute meningococcal meningitis. She had no previous history of recurrent headaches. The pain was intense, pulsatile and throbbing, typically unilateral, without aura. Its frequency increased during the following weeks and a prophylactic treatment with amitriptyline and atenolol was initiated. There was remission of the attacks.

OBJETIVO: Descrever as etiologias da síndrome de West (SW) em um grupo de crianças atendidas no ambiente de um centro de reabilitação. MÉTODO: Análise retrospectiva, avaliando-se os seguintes itens: gênero, idade por ocasião da definição do diagnóstico da SW e sua etiologia. Esta foi dividida em três categorias: sintomática, criptogênica e idiopática. Os casos sintomáticos foram divididos em pré, peri e pós-natais. RESULTADOS: Noventa e cinco pacientes foram incluídos, sendo 59 do gênero masculino (62%). A idade do diagnóstico variou entre 1 e 24 meses, com média de 4,9 (±5,0) meses. Vinte e cinco casos foram considerados criptogênicos (26,3%) e apenas um idiopático (1,1%). Os demais foram classificados com sintomáticos (72,6%), sendo predominantemente casos perinatais. CONCLUSÃO: Nossos achados se assemelham aos da literatura. Conforme se ampliam o conhecimento acerca da SW e os métodos complementares de diagnóstico, haverá tendência à diminuição dos casos hoje considerados criptogênicos ou idiopáticos.

INTRODUÇÃO: A cefaléia do tipo tensional (CTT), dor cefálica mais comum, tem mecanismos fisiopatológicos, epidemiologia e apresentação clínica pouco estudados. Depressão e ansiedade são comorbidades especialmente enfocadas entre seus portadores. OBJETIVO: Estudar a ocorrência de sintomas depressivos e ansiedade entre portadores de CTT episódica (CTTE) e crônica (CTTC). METODOLOGIA: Cinqüenta pacientes com CTTE e cinqüenta com CTTC preencheram os inventários de depressão e de ansiedade de Beck. Somente foram considerados aqueles com escores moderado ou grave. RESULTADOS: Entre portadores de CTTE, ansiedade e sintomas depressivos foram encontrados em 60 e 32 % dos pacientes, respectivamente. Em pacientes com CTTC, ansiedade foi detectada em 44 % e sintomas depressivos foram observados em 40 % da amostra. CONCLUSÃO: ambas as comorbidades são importantes em pacientes com CTT. Negligenciá-las significa risco de falência no tratamento, contribuindo para queda na qualidade de vida .

Com o objetivo de estudar os aspectos clínicos, a história familiar e o impacto da dor nas atividades laborativas, foi conduzido estudo de série de casos de 50 pacientes portadores de cefaléia do tipo tensional episódica (CTTE). Foram avaliados 40 mulheres e 10 homens, com idade média de 30 (±12) anos. Dor constrictiva esteve presente em 40 pacientes (80%). O enjôo foi o principal fenômeno acompanhante (20%). A dor bilateral predominou; entretanto, a localização unilateral também esteve presente (10%). Embora classicamente descrita como uma dor leve, observou-se que a CTTE pode se manifestar como crises de forte intensidade (16%). A história familiar foi positiva em 12 pacientes (24%). O impacto nas atividades laborativas foi detectado em 14% da amostra. Os achados a respeito das características da dor estão de acordo com a literatura. O impacto representado pela CTTE a nível individual e na sociedade deve, entretanto, ser reconsiderado.

Hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC) is a rare disease that has been recently described. It must be remembered as a possible etiology of leukoencephalopathies in children. We describe a typical case of H-ABC in a 11-month-old boy. He presents with global development delay, oral dyskinesia, and global dystonia and spasticity. Magnetic resonance imaging disclosed typical features of H-ABC and clinical laboratory tests were all negative. A slow neurological deterioration has been detected with worsening of involuntary movements.

Congenital muscle dystrophy (CMD) is a heterogeneous group of autosomal recessive myopathies. It is known that CMD may affect the central nervous system (CNS). Some authors have shown that merosin-negative CMD patients may have encephalic metabolic disturbances. In order to study metabolic changes within the brain, the authors performed a magnetic resonance spectroscopy (MRS) study in a 1-year-old girl with merosin-positive CMD (MP-CMD). MRS of brain demonstrated that NAA/Cr ratio was decreased (1.52), while Cho/Cr ratio was increased (1.78). These findings suggest that metabolic changes in CNS can also be found in patients with MP-CMD.

Objective The study aims to investigate the presence of pain amongst multiple sclerosis (MS) patients. Method One hundred MS patients responded to questionnaires evaluating neuropathic and nociceptive pain, depression and anxiety. Statistical analysis was performed using the Mann–Whitney U, Chi-Square and two-tailed Fisher’s exact tests and multivariate logistic regression. Results Women had a statistically higher prevalence of pain (p = 0.037), and chances of having pain after the age of 50 reduced. Women with pain had a statistically significant lower number of relapses (p = 0.003), restricting analysis to those patients with more than one relapse. After the second relapse, each relapse reduced the chance of having pain by 46%. Presence of pain was independent of Expanded Disability Status Scale (EDSS) anxiety, and depression. Conclusion Our findings suggest a strong inverse association between relapses and pain indicating a possible protective role of focal inflammation in the control of pain.

ABSTRACT Superficial siderosis (SS) of the central nervous system (CNS) is a rare and possibly underdiagnosed disorder resulting from chronic or intermittent bleeding into the subarachnoid space, leading to deposition of blood products in the subpial layers of the meninges. Magnetic resonance imaging (MRI) shows a characteristic curvilinear pattern of hypointensity on its blood-sensitive sequences. Methods Series of cases collected from Brazilian centers. Results We studied 13 cases of patients presenting with progressive histories of neurological dysfunction caused by SS-CNS. The most frequent clinical findings in these patients were progressive gait ataxia, hearing loss, hyperreflexia and cognitive dysfunction. The diagnoses of SS-CNS were made seven months to 30 years after the disease onset. Conclusion SS-CNS is a rare disease that may remain undiagnosed for long periods. Awareness of this condition is essential for the clinician.

ABSTRACT Objective To assess safety of the switch between natalizumab and fingolimod without a washout period. Methods Prospective data on 25 JCV positive patients who underwent this medication switch were collected and analyzed. Results After a median period of nine months from the medication switch, there were no safety issues to report. The patients had good disease control and no adverse events were reported. Conclusion Washout may not be necessary in daily practice when switching from natalizumab to fingolimod. Expertise on multiple sclerosis management, however, is essential for drug switching.