Introduction: Tolosa Hunt syndrome (THS) is a rare condition, incidence of 1/1.000.000 case per year, characterized by unilateral painful ophthalmoparesis caused by idiopathic inflammation in the cavernous sinus. The oculomotor nerve is most commonly involved (80%), followed by abducens nerve (70%), ophthalmic branch of trigeminal nerve (30%), trochlear nerve (29%). Case presentation: Male, 77 years old, admitted with an acute moderate-intensity orbitofrontal headache on the left, envolving with palpebral ptosis of the left eye. Neurological examination: complete palpebral ptosis on the left and ophthalmoplegia of the entire ipsilateral extrinsic ocular musculature. A complete investigation was carried out: metabolic, rheumatological, serological tests without significant alterations and study of the cerebrospinal fluid with mild hyperproteinorachia, without pleocytosis. Magnetic resonance imaging (MRI) of the skull showed thickening of the cavernous sinus on the left, with contrast enhancement; Angio-MRI of the Skull and Neck without alterations. Therefore, THS was diagnosed and treatment with Methylprednisolone 1 g for five days, with complete improvement of headache and partial improvement of ophthalmoparesis. The patient was discharged with 60 mg of prednisone orally with instructions for gradual weaning off, return to the neurology outpatient clinic. Discussion: THS diagnosis is based on the International Classification of Headache Disorders: unilateral periorbital headache; granulomatous inflammation of the cavernous sinus, superior orbital fissure or orbit on cranial MRI; paralysis of one or more of the oculomotor nerves; the headache must precede the ophthalmoparesis by up to two weeks or appear concomitantly. The exclusion of secondary causes is essential. Treatment of choice is cortico steroids, improvement of headache in the first days, and of ophthalmoparesis in 2–8 weeks. Conclusion: Unilateral headache with ipsilateral ophthalmoparesis should raise the suspicion of THS.

A derivação ventrículo-peritoneal (DVP) é o principal procedimento utilizado no tratamento de pacientes portadores de hidrocefalia, em especial durante o primeiro ano de vida. Em situações especiais, podem ser utilizados outros procedimentos como derivação ventrículo-atrial (DVA), derivação ventrículo sub-galeal, ou neuroendoscopia. Contudo, apesar de altamente eficaz no tratamento da hidrocefalia, as derivações ventriculares possuem alta taxa de complicações (5 a 30%) que incluem principalmente disfunções mecânicas e infecções. Objetivo: investigar a prevalência de deformidade ao uso de derivação ventricular, taxa de complicações e potenciais causas. Métodos: estudo transversal retrospectivo com todas as crianças submetidas à implantação de um sistema de derivação ventricular durante o primeiro ano de vida, procedimentos realizados nos últimos 11 anos (2004 a 2015), no Hospital das Clínicas da Universidade de Campinas e no Centro de Atenção Integral à Saúde da Mulher (CAISM) da Universidade Estadual de Campinas. Resultados: 50 pacientes do sexo masculino (58,13%), 36 pacientes do sexo feminino, idade média na 1ª cirurgia de 43,62 dias, média de cirurgia de shunt/paciente de 1,91. Conclusão: hidrocefalia no 1º ano de vida tem como etiologia principal mal formações congênitas e o tratamento cirúrgico definitivo foi a DVP. Pode se perceber que pacientes submetidos à DVP no 1º ano de vida apresentaram alta taxa de complicações(33,93%), com média de cirurgia shunt/paciente 1,91.

Introduction: Alexia Without Agrafia (AWA) is a syndrome in which the patient loses the ability to read while maintaining the ability to write. It’s described in strokes in the territory of the left posterior cerebral artery (PCA) and is usually accompanied by right homonymous hemianopia (HH) or color anomy. Case presentation: Male, 66 years old, complete higher education, righthanded, woke up two days ago with difficulty orienting himself, bumping into objects, visual difficulty in right hemifields. Neurological examination: preserved naming (when presented through sensory means other than visual), fluency, comprehension and writing, but inability to read, anomie for colors, HH on the right (R). CT Skull: hypoattenuating at occipital-temporal region left (L), in addition areas of encephalomalacia in the R occipital-temporal. Electrocardiogram: atrial fibrillation. US Doppler Carotid: no significant stenoses. Magnetic Resonance Imaging (MRI) Skull and Angio-MRI arterial phase: recent ischemia in the L occipital lobe and in L temporal lobe, involvement of splenium of the corpus callosum (CC), diffusion restriction and hypersignal in T2 and FLAIR (Fluid-Attenuated Inversion Recovery); previous ischemic lesion in the R temporal-occipital; hypoflow of bilateral PCA distal branches. Echocardiogram: enlarged L atrium. CT Skull 11 days after ictus with stability. Hospital discharge with Apixaban 5 mg every 12 hours, return to the neurology clinic. Discussion: Lesion in the L occipito-temporal cortex with involvement of the splenium of CC leads to a disconnection syndrome called AWA. The CC has fibers that connect the two cerebral hemispheres. The occipital lobe and splenium are supplied by the PCA. In addition, PCA infarction L leads to HH on the R, thus, visual information (letters) interpreted in the R visual cortex (visual field L), explaining why the patient can see the letters but not read them. Conclusion: Strokes are one of the main causes of morbidity. In the topography of the left PCA, we observed AWA.

Introduction: Guillain-Barré syndrome (GBS) is an acute inflammatory peripheral neuropathy that occurs after infection or immunization. Wernicke’s encephalopathy (WE) is caused by thiamine deficiency that classically presents with altered mental status, ataxic gait and ophthalmoplegia. Case report: Male, 64 years old, presented with diarrhea, vomiting and hyporexia. Three weeks later, he developed concomitant acute tetraparesis and cognitive impairment. On examination, he presented with persecutory delusions, hallucinations, tetraparesis with global areflexia. The cerebrospinal fluid analysis showed important albuminocytological dissociation. Treatment with intravenous immunoglobulin associated with thiamine was started. The electroneuromyography was compatible with demyelinating sensorimotor polyneuropathy. Brain Magnetic resonance imaging showed FLAIR (Fluid-Attenuated Inversion Recovery) hyperintensities in mamillary bodies and periaqueductal gray matter. The patient was diagnosed with GBS and WE. He was discharged after two weeks with complete resolution of the cognitive impairment and improvement of tetraparesis. Altered mental status in a patient with GBS is not common. There are some reports of patients who presented cognitive disturbances related to autonomic dysfunction and more severe cases, which developed a few days after the onset of the motor condition. The reported patient had a change in mental status concomitantly with a motor condition, reported prolonged gastrointestinal symptoms, in addition to having received intravenous glucose. WE may occur after a short period of thiamine absorption/intake deficiency and may be precipitated by glucose administration. The patient showed rapid cognitive improvement after thiamine supplementation, and had typical EW changes on the brain MRI confirming the diagnosis of WE. Conclusions: We must be aware of changes in mental status as this may indicate thiamine deficiency. The case elucidates the importance of thiamine replacement in patients at risk of vitamin deficiency.