NoVs were responsible for almost 50% of outbreaks, with about 70% of them resulting from genotype GII.4 and its variants. The seasonality observed could help health authorities to establish a system of active surveillance in order to reduce NoV impact especially in congregate settings.
-The Dandy-Walker syndrome (DWS) is a rare posterior fossa malformation. The DWS can occur associated with other brain or systemic malformations, but ocular abnormalities in this disease are rare and clinical findings mimicking myasthenia gravis have not been described to date. We report a 23-yearold woman who presented mild limitation of the ocular movements with progressive palpebral ptosis, which changed in intensity during the day. The investigation showed negative anti-acetylcholine receptor antibody, repetitive nerve stimulation and "Tensilon test", but the brain magnetic resonance image reveals DWS with hydrocephalus associated with calosal dysgenesis. The characteristic of disease, clinical manifestations and pathologic features, specially the clinical evaluation of ocular abnormalities in suspicion of DWS, including the MG in differential diagnosis are discussed.KEY WORDS: Dandy-Walker syndrome, hydrocephalus, myasthenia gravis, palpebral ptosis. Síndrome de Dandy-Walker em adulto simulando miastenia gravisRESUMO -A síndrome de Dandy-Walker (DWS) é uma rara malformação da fossa posterior que pode ocorrer associada com outras malformações cerebrais ou sistêmicas. As alterações oculares são raras e as manifestações clínicas, simulando miastenia gravis (MG), não foram descritas até o momento. Descrevemos uma mulher de 23 anos apresentando discreta limitação da movimentação ocular com progressiva ptose palpebral que mudava de intensidade durante o dia. A investigação mostrou negativos o anticorpo anti-receptor de acetilcolina, a estimulação nervosa repetitiva e o "teste do Tensilon", porém a ressonância magnéti-ca de crânio revelou DWS com hidrocefalia associada à disgenesia de corpo caloso. As características da doença, manifestações clínicas e patológicas, especialmente a avaliação clínica de anormalidade ocular na suspeita de DWS serão discutidas, incluindo a MG no diagnóstico diferencial.
-The case of a 70 year-old, previously healthy woman who developed a severe bleeding diathesis shortly after touching a Lonomia obliqua caterpillar and finally died from multiple intracerebral hemo rrhages is described. Brain hemorrhages are the leading cause of death in patients envenomed by the Lonomia species. The pertinent literature is reviewed and the most relevant clinical features highlighted, with emphasis on diagnosis. The use of new therapeutic options such as anti-Lonomia serum is discussed.KEY WORDS: intracerebral hemorrhage, Lonomia obliqua, caterpillar envenoming.Hemorragia intracerebral fatal causada por acidente com Lonomia obliqua: relato de caso RESUMO -O caso de uma mulher de 70 anos, previamente hígida, que desenvolveu diátese hemorrágica grave após contato com uma lagarta Lonomia obliqua, resultando em óbito por hemorragia intracere b r a l é relatado. Hemorragias cerebrais são uma das causas de morte em acidentes por Lonomia. A literatura p e rtinente é revisada, sendo as características clínicas e laboratoriais mais relevantes discutidas com ênfase para o diagnóstico, e o uso de novas abordagens terapêuticas como o soro anti-Lonomia. PALAVRAS-CHAVE: hemorragia intracerebral, Lonomia obliqua, envenenamento, lagartas urticantes.First described by Arocha-Pinango and Layrisse in Venezuela in 1967 1 , the hemorrhagic diathesis caused in humans by touching the Lonomia species begins with inflammatory changes at the site of envenoming, followed by systemic symptoms such as headache, f e v e r, vomiting and malaise. After 24 hours, a severe bleeding disorder ensues, leading to echimosis, hematuria, pulmonary and intracranial hemorrhages, and acute renal failure 2 . Two species of Lonomia a re known to cause the hemorrhagic syndrome, L o n o m i a achelous, found in Venezuela and the north of Brazil, and Lonomia obliqua, found in the south of Brazil 1 , 3 , 4 . Although there are diff e rences in the effect of the venom of both species, both venoms may lead to intense fibrinolytic activity associated with consumption coagulopathy, resembling a diffuse intravascular coagulation 5 -7 . A case of accidental Lonomia obliqua envenoming is described, and its implications a re discussed. The re p o rt was approved by the institutional regulatory committee. CASEA 70 year-old, previously healthy woman developed a sudden coma. Four days before, she had started to pre s e n t hematuria. Shortly after admission, her coma was rated as Glasgow 3. Physical examination revealed several skin hemo rrhages, and gross hematuria was present. Based on information in a note left by the patient, two small hyperemic lesions were identified on the tip of her left toe. Along with the note w as the the green caterpillar (Fig 1) which was hidden inside of her slipper. CT-scan imaging re v e a l e d multiple intracerebral hemorrhages (Fig 2). She died seven days after being envenomed. Her laboratory data is summarized in the Table. DISCUSSION P resentation of the symptoms of caterpillar envenoming can generally be...
Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant peripheral neuropathy characterized by recurrent episodes of paralysis of peripheral nerves, usually after a minor trauma to topographies prone to compression 1,2 . Involvement of the cranial nerves has rarely been described in these patients [3][4][5][6][7][8] . Reports of swallowing dysfunction in patients with HNPP are even rarer.We report a patient diagnosed with HNPP confirmed by molecular testing who presented with recurrent episodes of swallowing dysfunction (SD). CaSeA 41-year-old man who presented with recurrent episodes of acute mononeuropathies affecting mainly the ulnar, radial and peroneal nerves followed by spontaneous improvement since the age of 12 years. In the years prior to diagnosis, the patient started experiencing sudden episodes of reversible SD without a specific trigger, but it worsening with progressive increased of volume and solid consistency of bolus food. The SD had a spontaneous improvement after few weeks, but with residual dysfunction when he drinking liquid. Some of his relatives (sisters and nephew) had similar symptoms, but none had SD. He denied episodes of aspiration (airway protection), exposure to drugs or toxic agents.Cranial nerves showed mild weakness in posterior tongue during the swallow. Gag reflex, taste and sensation of tongue and oropharynx were normal. Mild asymmetrical muscle atrophy was observed in both hands and forearms while weakness was detected in the left first dorsal interosseus dorsal and flexor carpi ulnaris (grades 3 and 4, respectively, according to the Medical Research Council scale). Tendon reflexes were absent in both biceps and left brachioradial and ankle tendon reflexes were also absent. Pain, pinprick and light touch sensory impairment were detected on the territories of the left median, bilateral sural and superficial peroneal nerves.Nerve conduction studies showed a nonuniform demyelinating neuropathy, mainly the ulnar, radial and peroneal nerves, together with evidence of asymmetrical acute and/or chronic denervation affecting predominantly the first dorsal interosseus, tibialis anterior and gastrocnemius muscles. Nerve conduction studies with similar findings were observed in the patient's sisters and nephew.Laboratory tests as well as serological tests were normal. Biopsy of the sural nerve showed a reduction in the number of large myelinated fibers and focal thickening of the myelin sheath (formation of tomaculae). Biopsy of the gastrocnemius muscle biopsy showed recent and chronic denervation.Phonoaudiological evaluation, videoendoscopic and fluoroscopic examination of deglutition revealed normal oral (preparatory and transport) phase; swallowing test (liquid, semisolid and solid consistencies) presented mild nasal escape, normal oral transit time, residual bolus in pyriform sinuses and valleculae cleared by dry swallow and cough, episodes of laryngeal penetration only after great volume of liquid, but with normal coughing reflex, and absence of asp...
We report a patient diagnosed with stiff-three limbs syndrome, without continuous motor unit activity in the electrophysiologic exam, with successful treatment with intravenous immunoglobulin.
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