Sudden, Unexpected Death in a 5-Year-Old Boy with an Unusual Primary Intracranial Neoplasm

Nelson, J.; Frost, James L.; Schochet, Sydney S.

doi:10.1097/00000433-198708020-00013

Cited by 26 publications

(4 citation statements)

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“…Although not previously reported, severe sweating as observed in our case may support the sympathetic hyperactivity. Among posterior fossa tumors with sudden unexpected death, only ganglioglioma is clearly associated with apneic spells [6,7]. These findings suggest that a cervicomedullary ganglioglioma potentially presents with a cyanotic BHS in the natural course or after surgery, and that the spell should be kept in mind as lifethreatening during the early postoperative period.…”

Section: Discussionmentioning

confidence: 88%

“…According to one previous report [3], 8 of 51 (16%) severe cyanotic breath-holders died: 4 died during the episodes and 6 had brain stem abnormalities such as Arnold-Chiari malformation, or medullary tumor. Cervicomedullary gangliogliomas have been detected in such victims at autopsy [6,7].…”

Section: Discussionmentioning

confidence: 99%

“…Although BHS is believed to be benign and self-limiting as the child grows, it is occasionally associated with sudden death [3,5]. In most of these fatal cases, brain stem tumors or abnormalities were recognized afterward [3,6,7].…”

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confidence: 99%

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Cyanotic Breath-Holding Spell: A Life-Threatening Complication after Radical Resection of a Cervicomedullary Ganglioglioma

et al. 2005

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Cyanotic breath-holding spell is a benign and self-limiting disease of young children but occasionally associated with sudden, unexpected death. The authors report a rare case in a 2-year-old girl with a severe form that started after radical resection of a cervicomedullary ganglioglioma. She was admitted to our hospital because of delayed and unstable gait. Since magnetic resonance imaging showed a cervicomedullary tumor, she underwent a radical resection and histology showed the tumor to be a ganglioglioma. Postoperatively, the function of the lower cranial nerves and cerebellum deteriorated and hemiparesis on the left became apparent, but she returned to the preoperative state in a few months. In addition, mild sleep apnea (Ondine curse) and severe cyanotic breath-holding spells occurred. The former responded to medication but the latter failed and continued several times per day with a rapid onset and progression of hypoxemia, loss of consciousness, sweating and opisthotonos. Five months after the operation, the patient returned home with a portable oxygen saturation monitor equipped with an alarm. This case indicates that cyanotic breath-holding spell, as well as sleep apnea, is critical during the early postoperative period. This is the first report observing that such spells may occur as a complication of radical resection of a cervicomedullary tumor.

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Section: Discussionmentioning

confidence: 88%

Section: Discussionmentioning

confidence: 99%

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Cyanotic Breath-Holding Spell: A Life-Threatening Complication after Radical Resection of a Cervicomedullary Ganglioglioma

et al. 2005

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“…To our knowledge, including our case, only 30 cases of cerebellar GG have been described 1, (Table 1). Cerebellar GGs have a generally short mean history (1.6 years), whereas longer histories have been found in cases of brainstem GGs 4,7,29,[33][34][35][36][37][38][39][40][41][42][43][44][45][46][47] (Table 1).…”

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confidence: 99%

Posterior Cranial Fossa Gangliogliomas

et al. 2007

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Ganglioglioma (GG) is an uncommon primary lesion of the central nervous system that is typically located supratentorially. There are only a few reports of GG arising from the cerebellum. To the best of our knowledge this is the first case of a cerebellar GG with supratentorial extension and a longstanding history before its recognition. In fact, this 29-year-old male presented with an 11-year history of intermittent headaches. A cranial computerized tomography (CT) performed at the onset of his complaints failed to reveal the tumor. After a particularly longstanding cephalalgic episode, the patient underwent a new CT scan that was also negative. However, magnetic resonance (MR) imaging of the brain revealed a space-occupying lesion in the right cerebellar hemisphere with extension to the level of the superior colliculi and pineal recess. The tumor was partially removed through a midline suboccipital craniotomy and supracerebellar approach. Pathological examination of the tumor showed composition of atypical ganglion cells and astrocytes, indicating the diagnosis of cerebellar GG. At last follow-up, 24 months after surgery, the patient reported a marked improvement of his clinical condition with significant reduction of intensity and frequency of the headache. The present report illustrates how cerebellar GG may remain undetectable by CT and may therefore present with a longstanding history and nonspecific signs and symptoms. MR investigation can lead to the proper diagnosis. Even after partial removal the prognosis remains good and remission of the symptoms may be achieved. In this article, we review the literature and summarize the current understanding of infratentorial GGs.

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