Thiago Cardoso Vale

Bilateral posterior circulation stroke secondary to a crotalid envenomation: case report.

Snake bite envenoming is a disease with potential serious neurological complications. We report a case of an adolescent who was bitten by a rattlesnake and developed bilateral posterior circulation stroke. The rattlesnake was later identified as being Crotalus durissus terrificus. Stroke was probably due to toxic vasculitis or toxin-induced vascular spasm and endothelial damage.

Cranial hypertrophic pachymeningitis secondary to neurocysticercosis.

We report a case of a 46-year-old Brazilian woman, a farmer, who presented with recently uncontrolled epilepsy, daily headaches and ataxia. Cranial CT revealed hydrocephalus which was treated with ventricular drainage. Brain MRI revealed multiple parenchymal cysts of varying stages of neurocysticercosis. In addition, the patient presented with diffuse dural enhancement consisted with pachymeningitis, which is quite an unusual manifestation of neurocysticercosis.

Neurosyphilis presenting as mania

Barbosa IG, Vale TC, de Macedo DL, Gomez RS, Teixeira AL. Neurosyphilis presenting as mania. Bipolar Disord 2012: 14: 309–312.

Atypical subacute sclerosing panencephalitis: case report

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disorder of the central nervous system with both poor prognosis and high mortality. The disease has been related to a persistent and aberrant measles virus infection and no effective treatment has been available. We report a case of SSPE with atypical features including seizures at onset and a fulminant course in a 8 years-old boy who had been previously immunized against measles.

FATAL OUTCOME OF INFECTION BY DENGUE 4 IN A PATIENT WITH THROMBOCYTOPENIC PURPURA AS A COMORBID CONDITION IN BRAZIL

Dengue is currently a major public-health problem. Dengue virus (DENV) is classified into four distinct serotypes, DENV 1-4. After 28 years of absence, DENV-4 was again detected in Brazil in 2010 in Roraima State, and one year later, the virus was identified in the northern Brazilian states of Amazonas and Pará, followed by Rio de Janeiro and São Paulo. In Minas Gerais, the first confirmed case of DENV-4 occurred in the municipality of Frutal in 2011 and has now been isolated from a growing number of patients. Although DENV-2 is associated with the highest risk of severe forms of the disease and death due to the infection, DENV-4 has also been associated with severe forms of the disease and an increasing risk of hemorrhagic manifestations. Herein, the first fatal case of confirmed DENV-4 in Brazil is reported. The patient was an 11-year-old girl from the municipality of Montes Claros in northern Minas Gerais State, Brazil. She had idiopathic thrombocytopenic purpura as a comorbid condition and presented with a fulminant course of infection, leading to death due to hemorrhagic complications. Diagnosis was confirmed by detection of Dengue-specific antibodies using IgM capture enzyme-linked immunosorbent assay and semi-nested RT-PCR. Primary care physicians and other health-care providers should bear in mind that DENV-4 can also result in severe forms of the disease and lead to hemorrhagic complications and death, mainly when dengue infection is associated with coexisting conditions.

Teaching Video NeuroImages: Hemichorea-hemiballismus secondary to nonketotic hyperglycemia

A 59-year-old woman presented with a 10-day history of involuntary movements of the right body. She initially complained of continuous, irregularly timed, and unpredictable movements of her right hand that ultimately spread to her entire right upper limb, sometimes having an abrupt violent character (video, segment 1, on the Neurology® Web site at www.neurology.org). She had poorly controlled type 2 diabetes.1 Her neurologic examination revealed slight right facial weakness and right hemichorea-hemiballismus. Blood glucose was at 31 mmol/L with no ketone bodies in the urine. Brain imaging revealed spontaneous high signal of the left caudate nucleus and putamen (figure). She received insulin and neuroleptics (risperidone) with rapid improvement (video, segment 2).

Takayasu's Arteritis in a Patient with Sydenham's Chorea: is There an Association?

Background Takayasu’s arteritis (TA) has been associated with many conditions. Herein described is a case of TA in a patient with rheumatic fever complicated with Sydenham’s chorea. Case Report A 17-year-old female presented at age 6 with rheumatic fever followed by chorea a month later. At the age of 16, she developed a blood pressure discrepancy between the arms and faint pulses. Computed tomography angiography revealed diffuse aortic involvement and narrowing of the arteries. Discussion The presence of rheumatic fever and Sydenham’s chorea in TA raises the possibility of an immunological basis for the pathogenesis of the disease.

Long-term mood disorder antedating the diagnosis of Wilson's disease

Dear Editor,We evaluated a young patient with a long history of psychiatric symptoms and misdiagnosis. After nine years of receiving many ineffective symptomatic therapies, she was diagnosed with advanced Wilson’s disease (WD). We would like to present this case in order to raise awareness around the wide clinical spectrum of WD as well as around the need to establishing high clinical suspicion for this diagnosis. A 17-year-old woman, the daughter of consanguineous parents, was admitted to a university hospital with the diagnosis of cryptogenic cirrhosis. Her clinical history revealed that, since the age of nine, she had been experiencing a series of episodes of excessive fear, anxiety and depression. As her depressive symptoms grew steadily worse, she was put on antidepressants, including amytriptiline and fluoxetine. At the age of 12, she committed two suicide attempts, which were followed by an episode of frank mania with psychotic symptoms, evidenced by her attempt to bury herself up. Between the ages of 12 and 17, she remained on mood stabilizing agents, including carbamazepine and valproic acid. This course of treatment was, however, unsuccessful. Six months before hospitalization, she experienced weight gain and diffuse abdominal pain, followed by nausea and hyporexia. Ultrasonography and computed tomography (CT) of the abdomen revealed cirrhosis and ascites. Extensive serological studies, including HIV, hepatitis B and C, anti-LKM1, anti-mitochondrial, anti-smooth muscular and anti-nuclear antibodies were unrevealing. When admitted to the hospital, her abdominal pain had worsened and she presented with severe ascites and delirium. Neurological examination showed diffuse hyperreflexia and ankle clonus. Kayser-Fleischer rings were present bilaterally. Further investigation yielded 13 points on the Child-Pugh’s classification (severe ascites, stage II encephalopathy, albumin level 2.1, international normalized ratio 2.74, bilirubin levels equal to 4.2). Alpha-fetoprotein at 45.1ng/ml (normal value: < 300ng/ml) and serum ceruloplasmin at 9.0mg/dl (normal value: 15-60mg/dl). Cranial CT showed enlargement of the ventricles and caudate atrophy. She rapidly became hemodynamically unstable, thus making death seemingly inevitable. Upon immediate investigation, family members disclosed that her two brothers had been diagnosed with WD. Her mother, who used fluoxetine on an irregular basis, had been diagnosed with depression and her uncle with schizophrenia.WD is an autosomal recessive genetic disorder related to the metabolism of copper, which accumulates in several tissues such the brain, liver and cornea. Neurological and psychiatric symptoms may occur due to the presence of such copper deposits in the brain.

Schistosoma mansoni Encephalomyelitis

A 27-YEAR-OLD BRAZILian woman had a history of lumbar and lower-limb pain associated with a progressive decrease in muscle strength and paresthesias. Neurological examination findings revealed flaccid paraparesis, lack of patellar and Achilles-tendon bilateral reflexes, and urinary retention. Thoracic and lumbar spinal cord magnetic resonance imaging showed a hyperintense signal extending from T3 to T6. She was diagnosed as having an inflammatory myeloradiculopathy, and corticosteroid therapy was started with partial improvement in her condition. Five months after being discharged from hospital, she was readmitted owing to tonicoclonic seizures and visual impairment. Brain magnetic resonance imaging revealed a right parieto-occipital mass (Figure 1) and a lesion in the left cerebellar hemisphere (Figure 2). Microscopy showed multiple granulomas within which Schistosoma mansoni ova were observed (Figure 3). The patient received a combination of praziquantel (60 mg/kg, body weight, single dose) and prednisone (1 mg/kg, body weight, daily for 6 months), and her condition improved quickly. Three months later, she was seen again, and she reported no seizures. Less intense lower extremity sensory and motor deficits persisted. Her visual impairment was resolved. Ten months after treatment, new magnetic resonance imaging showed almost complete resolution of the brain lesions (Figure 4). COMMENT

Teaching NeuroImages: A neuroendocrine rarity: Wolfram syndrome

Wolfram syndrome 1 (WS1) is an autosomal recessive disorder characterized by diabetes insipidus, diabetes mellitus, optic atrophy, and deafness (DIDMOAD syndrome). It is caused by a mutation in the WFS1 gene (chromosome 4p16.1, involved in regulation of intracellular calcium) that encodes wolframin, a transmembrane protein of pancreatic β cells. WS1 is a rare disorder characterized by the development of insulin-dependent diabetes mellitus at an average age of 6 years, followed by optic atrophy (figure) at an average age of 11 and partial central diabetes insipidus and deafness in adolescence. Additional neurologic manifestations include truncal ataxia, myoclonus, epilepsy, nystagmus, and hyposmia.1,2