Sneddon's syndrome (SS) is characterized by ischemic cerebrovascular episodes and livedo reticularis. It is more common in young women and can also be associated with valvulopathy, a history of spontaneous abortion, renal involvement and vascular dementia. We describe three cases of young women with this disease. The patients had repeated ischemic cerebral episodes, livedo reticularis and thrombocytopenia. CT and MRI showed strokes and cerebral atrophy. Autopsy in one of the patients revealed cerebral infarctions. Anticardiolipin antibodies were detected in two patients. Antiphospholipid antibodies may be found in some patients with ischemic cerebrovascular events and livedo reticularis. SS may thus be associated with antiphospholipid syndrome. We described three new cases of SS and discuss the pathophysiology of this disease.
Spinal vascular malformations in child are rare and still under-diagnosed entities that typically lead to progressive spinal cord symptoms and myelopathy if not properly treated 1 . This malformation can be differentiated into congenital (arteriovenous malformations and cavernomas) or acquired (dural arteriovenous fistulae) forms 1 . Spinal cord arteriovenous malformations (SCAVM) comprise a complex and heterogeneous group of lesions with a number of subtypes that have distinct clinical presentations, anatomic features and natural histories 2,3 . While there are important differences between subtypes, the majority of patients with SCAVM present first clinical manifestations only in adolescence or adulthood 2 . We describe the characteristics on child with SCAVM and show the possible treatment of this disease on childhood because only few cases have been published. CASEA 4-year-old girl presented sudden onset of lumbar and lower limbs pain, weakness in right lower limb, difficulty walking, constipation and urinary retention. Her parents denied previous similar episodes. She was born at term and was the first child of non-consanguineous parents and the only affected case in the family. The psychomotor development was normal.Physical examination did not reveal any abnormalities. Neurological examination showed hypotonia in lower limbs (right more than left); muscle strength grade 3 (MRC scale) in the right lower limb and grade 5 in the bilateral upper and left lower limbs; absent right patellar reflex; bilateral Babinski sign; decreased pain, pinprick and light touch sensibility in the right lower limb; and abnormal joint position sense in the right lower limb.The symptoms and signs were thought to be consistent with spinal cord abnormality, and the investigation showed a cerebrospinal fluid with 5 leukocytes/mm 3 , 140 erythrocytes/mm 3 , glucose 55 mg/dl and protein 25.7 mg/dl; needle electromyography and sensory nerve conduction study were normal, but motor nerve conduction study demonstrated absent F-wave in right peroneal nerve; magnetic resonance imaging (MRI) revealed high signal on T1-weighted images and hypointense signal on T2-weighted images in area of the T11-T12 spinal cord with concomitant swelling of the T11-L1 cord (Fig 1); and selective spinal angiography demonstrated a small arteriovenous fistula with posterior aneurysmatic formation on T11-T12 spine cord area which was fed by the anterior spinal artery arising from the right 12 th and left 9 th intercostal arteries (Fig 2A and 2B).After this initial investigation the patient was submitted to management of the SCAVM. Surgery was performed with clipping of the supplies arteries resulting complete obliteration of the fistula on postoperative spinal angiography (Fig 2C). Followup shows that her symptoms and neurological status improved after surgery.All studies were done following informed consent of parents.
Lipoma de corpo caloso é uma condição clínica rara. A epilepsia é a manifestação mais comum, mas também há casos assintomáticos, sendo um achado ocasional em exames de imagem ou autopsia. O caso relata uma situação clínica infrequente, cujo diagnóstico foi definido após realização de exames de neuroimagem. Paciente de 39 anos, que aos 10 anos de idade apresentou crise convulsiva, sendo iniciado tratamento com fenobarbital, ficou em uso deste por 4 anos com bom controle do quadro, sendo suspenso após esse período, e permanecendo assintomática. No entanto, durante a gravidez, houve recidiva das crises, com retomada do uso das medicações, mesmo assim esta persistiu com dois episódios convulsivos ao mês. A paciente foi submetida a exames de neuroimagem que evidenciaram o lipoma de corpo caloso, sendo optado pelo tratamento clínico. Diante dos avanços nos métodos de neuroimagem para diagnóstico, a probabilidade de essa má-formação ser diagnosticada, mesmo quando assintomática, tem aumentado. A ressecção do lipoma de corpo caloso, como descritos na literatura, tem mostrado resultados catastróficos, sendo assim, em casos assintomáticos ou achado incidental o tratamento deve ser conservador, como no caso da paciente em estudo.
Introduction: Leprosy, still considered a public health problem in many countries, among them Brazil, causes multiple sensory and/or motor signs and symptoms of the peripheral nervous system (SM-SSPNS). This study aimed to analyze a pretreatment neurological profile of patients with leprosy. Methods: Cross-sectional study, whose participants 400 patients diagnosed with leprosy. Data collection took place through the collection from medical records and clinical examination of patients. It determined the frequency distribution of SMSSPNS, thickening of the nerves and committed peripheral nerves (PNC) as well as the sex and the average age. Results: Over 93% of the patients presented at least 1 (one) sensory symptom, and hypoesthesia was the most prevalent among them. 59% displayed motor dysfunction and a 50.25% rate of neural thickening. Hypoesthesia had a higher prevalence among individuals whose symptoms appeared more than 12 (twelve) months ago (58.86%, p = 0.018), and there was no association between other SMSSPNS and the disease's time of onset; or between SMSSPNS and CPN, according to the leprosy forms. The most affected nerve is the sensory-motor ulnar (57.48%). Conclusion: The most frequent alterations were categorized as sensitives, including hypoesthesia (with a higher prevalence in comparison to when it first appeared, more than 12 months ago) and impairment of the sensory-motor ulnar nerve.
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