✓ The case is reported of meningioangiomatosis of the brain stem in a 3½-year-old girl who suffered from vomiting, left facial weakness, difficulty in swallowing, and ataxia. This is believed to be the first reported case of meningioangiomatosis in the brain stem. Computerized tomography showed an intensely enhancing hyperdense mass in the left restiform body. Magnetic resonance imaging revealed that the lesion was isointense to gray matter on the T1-weighted image and hypointense on the T2-weighted image, with a surrounding zone of high T2 signal and intense enhancement. Angiography was normal. Surgical exploration demonstrated an intramedullary firm mass that was partially resected. Histologically, the mass consisted of a low-grade lesion of meningeal origin with spindle cells in a whorling pattern that were occasionally focused around small vessels. On 2-year follow-up imaging, the lesion remains unchanged in size. Certain particularities of this lesion are discussed in the context of the literature.
Spyros S. Kollias, Kerry R. Crone, William S. Ball Jr., Erin C. Prenger and Edgar T. Ballard
Yu-tze Ng, John F. Kerrigan, Erin C. Prenger, William L. White and Harold L. Rekate
P The authors report the case of a 12-year-old girl with Pallister—Hall syndrome, long-standing refractory, symptomatic epilepsy, mental retardation, and panhypopituitarism in whom two rare, deep midline lesions were detected. She underwent successful transsphenoidal resection of the Rathke cleft cyst and transcallosal resection of the hypothalamic hamartoma within a 4-day period without complications. Neuropathological studies confirmed the neuroimaging diagnoses for the two lesions. The patient has been seizure free for 6 months postoperatively.
Cornelia Drees, Kevin Chapman, Erin Prenger, Leslie Baxter, Rama Maganti, Harold Rekate, Andrew Shetter, Maggie Bobrowitz and John F. Kerrigan
This study aimed at identifying outcomes with respect to seizures, morbidity, and mortality in adult patients undergoing resective or Gamma Knife surgery (GKS) to treat intractable epilepsy associated with hypothalamic hamartoma (HH).
Adult patients undergoing surgical treatment for HH-related epilepsy were prospectively monitored at a single center for complications and seizure outcome by using a proprietary database. Preintervention and postintervention data for patients 18 years of age and older, and with at least 1 year of follow-up, were analyzed, with specific attention to seizure control, complications, hormonal status, and death.
Forty adult patients were found in the database (21 were women). The median HH volume was 0.54 cm3. In 70% of patients, it was located inside the third ventricle, attached unilaterally and vertically to the hypothalamus (Delalande Type II). Most patients (26) underwent an endoscopic resection, 10 patients had a transcallosal or other type of open (pterional or orbitozygomatic) resection, and 4 patients chose GKS.
Twenty-nine percent became seizure free in the long term, and overall a majority of patients (55%) reported at least > 90% seizure improvement. Only 3 patients were ultimately able to discontinue anticonvulsants, whereas most patients were taking an average of 2 antiepileptic drugs pre- and postoperatively. The only factor significantly correlated with seizure-free outcome was the absence of mental retardation. The HH volume, HH type, and amount of resection or disconnection were not correlated to seizure freedom. A total of 4 patients (10%) died, 2 immediately after surgery and 2 later. All of them had undergone a resection, as opposed to GKS, and still had seizures. Postoperatively, persistent neurological deficits were seen in 1 patient; 34% of patients had mild hormonal problems; and 59% experienced weight gain of at least 6.8 kg (average gain 12.7 kg).
Surgical or GKS procedures in adults with HH provided seizure freedom in one-third of patients. The only significant favorable prognostic factor was the absence of mental retardation. The overall mortality rate was high, at 10%. Other important morbidities were persistent hormonal disturbances and weight gain.