We report a case of sudden, unexpected death associated with meningioangiomatosis in a 13-year-old, previously healthy male without a history of seizures, neurologic deficits, or clinical stigmata of neurofibromatosis. There was no family history of neurofibromatosis. The postmortem examination showed a 5-cm mass involving the right posterior frontal and orbital frontal cortex that had microscopic features diagnostic of meningioangiomatosis. Because no other cause of death was found, we postulate that he likely died as a result of a seizure secondary to meningioangiomatosis.
"In cases of intractable seizures, resection is indicated(Jallo et al., 2005). At least one case of sudden death, presumably secondary to a fatal seizure, has been reported involving a previously-asymptomatic 13-year old boy(Wixom et al., 2005). "
[Show abstract][Hide abstract] ABSTRACT: Sudden unexplained death in childhood (SUDC) is rare, with a reported incidence in the United States of 1.5 deaths per 100,000 live births compared with 56 deaths per 100,000 live births for sudden infant death syndrome in 2001. The objectives of this study include a proposal for a general definition for SUDC and presentation of 36 cases of SUDC and 14 cases of sudden unexpected death in childhood. Cases were accrued through referrals or unsolicited via our Web page (http://www.sudc.org ). Our analyses tentatively suggest a SUDC profile characterized by cases being 1 to 3 years in age, predominantly male, and frequently having a personal and family history of seizures that are often associated with a fever. A history of recent minor head trauma is not uncommon. They are usually born at term as singletons and occasionally have a family history of sudden infant death syndrome or SUDC. Most are found prone, often with their face straight down into the sleep surface. Minor findings are commonly seen at postmortem examination but do not explain their deaths. Comprehensive review of the medical history and circumstances of death and performance of a complete postmortem examination including ancillary studies and extensive histologic sampling of the brain are critical in determining the cause of death in these cases of sudden unexpected childhood death. Legislation enabling research and formation of a multicenter research team is recommended to unravel the mystery of SUDC.
[Show abstract][Hide abstract] ABSTRACT: Meningioangiomatosis (MA) is a rare congenital tumor that occurs mostly in 5-15 year old children. There have been only 5 cases previously reported that described the cystic nature within these tumors. We present a case of a MA accompanied by a separate macrocyst. A normally developed 2 year-old female patient presented with partial and generalized seizures. The brain computerized tomogram and magnetic resonance imaging revealed the presence of a calcified mass accompanied by a cyst in the right parietal area, surrounded by low density and high attenuation edema and hemorrhage. Upon right parietal craniotomy, a 1.6 cm x 1.2 cm x 0.5 cm sized plate-like, gray-white, slightly hard mass was seen and it was completely excised. Approximately 1 cm from the mass in the anterior lateral direction, a cyst was found and subsequent biopsy of the cyst wall revealed no tumor tissue, and therefore the cyst was not removed. Pathologic report demonstrated the meningioangiomatosis. Follow up examination 2 years later showed no recurrence of the tumor, and there was no evidence of neurological deficits. Authors suggest that cysts that arise in the surrounding tissues of tumors may not be tumor cysts, and do not require surgical removal.
Journal of Korean Neurosurgical Society 09/2009; 46(3):252-6. DOI:10.3340/jkns.2009.46.3.252 · 0.64 Impact Factor
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