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  • Author or Editor: Scott D. Wait x
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Scott D. Wait, Francisco A. Ponce, Brendan D. Killory, Donna Wallace and Harold L. Rekate

Neuroleptic malignant syndrome (NMS) is a potentially life-threatening entity characterized by hyperthermia, autonomic deregulation, decreased mental status, increased muscle tone, and, frequently, by renal failure due to rhabdomyolysis. Classically, it follows administration of antipsychotic medication.

The authors report on 4 patients (2 children and 2 adults) in whom NMS was diagnosed after a CNS insult. No patient was receiving antipsychotic medication. The patients' hospital and clinic charts, radiographic data, and follow-up telephone conversations were reviewed retrospectively.

All 4 patients met diagnostic criteria for NMS. Three patients presented with shunt failure, and 1 patient had undergone a functional hemispherectomy 2 days earlier. One patient with shunt failure received the diagnosis retrospectively. An endoscopic third ventriculostomy alleviated his shunt failure and he remains free of NMS. The other 2 patients underwent treatment for shunt failure, but NMS remained. These 2 patients and the one who had undergone hemispherectomy underwent a trial of intrathecal baclofen, and the NMS resolved. Subsequently, an intrathecal baclofen infusion device was placed in all 3 patients, and the NMS resolved. The 2 patients in shunt failure had a lumbar intrathecal baclofen infusion device. The patient who had undergone hemispherectomy had an intracranial baclofen catheter.

Neuroleptic malignant syndrome is a rare, life-threatening disorder that can occur without the administration of neuroleptic medications. Alleviation of any CNS insult is the first order of treatment. Some patients with persistent symptoms of NMS may benefit from intrathecal delivery of baclofen.

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Scott D. Wait, Adib A. Abla, Brendan D. Killory, Peter Nakaji and Harold L. Rekate

Object

Hypothalamic hamartomas (HHs) are devastating lesions causing refractory epilepsy, rage attacks, social ineptitude, and precocious puberty. Microsurgical and/or endoscopic resection offers an excellent risk/benefit profile for cure or improvement of epilepsy.

Methods

The authors reviewed a prospective database maintained during the first 7 years of the Barrow Hypothalamic Hamartoma program. They describe and illustrate their surgical methods, and they review data from several previous publications regarding surgical outcome.

Results

To date, the authors have performed surgery in 165 patients for symptomatic HHs. Patients underwent an endoscopic, transcallosal, or skull base approach, or multiple approaches. Twenty-six patients (15.8%) required more than 1 treatment for their HH.

Conclusions

Microsurgical and endoscopic resection of symptomatic HHs are technically demanding but can be performed safely with excellent results and an acceptable risk profile. Meticulous attention to the subtleties of surgical management helps optimize outcomes.

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Adib A. Abla, Andrew G. Shetter, Steve W. Chang, Scott D. Wait, David G. Brachman, Yu-Tze Ng, Harold L. Rekate and John F. Kerrigan

Object

The authors present outcomes obtained in patients who underwent Gamma Knife surgery (GKS) at 1 institution as part of a multimodal treatment of refractory epilepsy caused by hypothalamic hamartomas (HHs).

Methods

Between 2003 and 2010, 19 patients with HH underwent GKS. Eight patients had follow-up for less than 1 year, and 1 patient was lost to follow-up. The 10 remaining patients (mean age 15.1 years, range 5.7–29.3 years) had a mean follow-up of 43 months (range 18–81 months) and are the focus of this report. Five patients had undergone a total of 6 prior surgeries: 1 transcallosal resection of the HH, 2 endoscopic transventricular resections of the HH, 2 temporal lobectomies, and 1 arachnoid cyst evacuation. In an institutional review board–approved study, postoperative complications and long-term outcome measures were monitored prospectively with the use of a proprietary database. Seven patients harbored Delalande Type II lesions; the remainder harbored Type III or IV lesions. Seizure frequency ranged from 1–2 monthly to as many as 100 gelastic seizures daily. The mean lesion volume was 695 mm3 (range 169–3000 mm3, median 265 mm3). The mean/median dose directed to the 50% isodose line was 18 Gy (range 16–20 Gy). The mean maximum point dose to the optic chiasm was 7.5 Gy (range 5–10 Gy). Three patients underwent additional resection 14.5, 21, and 32 months after GKS.

Results

Of the 10 patients included in this study, 6 are seizure free (2 after they underwent additional surgery), 1 has a 50%–90% reduction in seizure frequency, 2 have a 50% reduction in seizure frequency, and 1 has observed no change in seizure frequency. Overall quality of life, based on data obtained from follow-up telephone conversations and/or surveys, improved in 9 patients and was due to improvements in seizure control (9 patients), short-term memory loss (3 patients), and behavioral symptoms (5 patients); in 1 patient, quality of life remains minimally affected. Incidences of morbidity were all temporary and included poikilothermia (1 patient), increased depression (1 patient), weight gain/increased appetite (2 patients), and anxiety (1 patient) after GKS.

Conclusions

Of the approximately 150 patients at Barrow Neurological Institute who have undergone treatment for HH, the authors have reserved GKS for treatment of small HHs located distal from radiosensitive structures in patients with high cognitive function and a stable clinical picture, which allows time for the effects of radiosurgery to occur without further deterioration. The lack of significant morbidity and the clinical outcomes achieved in this study demonstrated a low risk of GKS for HH with results comparable to those of previous series.