ABSTRACT
BACKGROUND: Adult moyamoya disease is rare in the United States, and patients mostly present with cerebral ischemia. However, clinical and neurodiagnostic correlates of ischemia are not well known in this population. We sought to characterize the clinical and radiographic features of moyamoya disease in a large urban center in the United States, with a focus on angiographic and neuroimaging patterns of ischemia. METHODS AND RESULTS: We retrospectively reviewed charts of consecutive adult moyamoya disease patients evaluated at 2 centers in Houston, Texas from January 2002 to December 2011. We reviewed all available cerebral angiograms and neuroimaging studies to evaluate the Suzuki grades, presence of intracranial hemorrhage or ischemia, infarct patterns, and vascular territory distribution. Our analysis was mainly descriptive. We identified 31 adults with moyamoya disease who met our inclusion criteria. The female-to-male ratio was 2.4:1. The majority of patients were white, followed by Hispanic, black, and Asian. Most presented with ischemia (61%), followed by headaches, and intracranial hemorrhage. Of the 22 patients with available neuroimaging, 72.7% had ischemic findings, with the vast majority having a watershed pattern (81.3%). CONCLUSIONS: We observed a high burden of ischemia, mostly watershed pattern on neuroimaging in our adult moyamoya disease patients. Long-term monitoring of adult moyamoya disease patients in the United States would be useful to better understand the natural history of this condition.
Subject(s)
Brain Ischemia/diagnosis , Cerebral Hemorrhage/diagnosis , Ischemic Attack, Transient/diagnosis , Moyamoya Disease/diagnosis , Stroke/diagnosis , Adolescent , Adult , Aged , Brain Ischemia/etiology , Cerebral Angiography , Cerebral Hemorrhage/etiology , Female , Headache/etiology , Hospitals, Urban , Humans , Ischemic Attack, Transient/etiology , Magnetic Resonance Imaging , Male , Middle Aged , Moyamoya Disease/complications , Retrospective Studies , Stroke/etiology , Texas , Tomography, X-Ray Computed , Young AdultABSTRACT
Eating-induced seizures are an uncommon presentation of reflex epilepsy, a condition characterized by seizures provoked by specific stimuli. Most reports have identified aetiology associated with malformations of cortical developmental, hypoxic brain injury, previous meningoencephalitis or static encephalopathy. We present a patient with eating-induced reflex seizures, which began several years after treatment for an opercular primitive neuroectodermal tumour (PNET), and who subsequently underwent in-depth clinical and video-EEG analysis for her seizures. This patient noted rapid improvement with decreased frequency of seizure activity after treatment with valproic acid. We discuss the aetiology of reflex epilepsy, the anatomical basis of eating-induced epilepsy, and review the current literature.
Subject(s)
Brain Neoplasms/complications , Cerebral Cortex/pathology , Eating , Epilepsy, Reflex/etiology , Neuroectodermal Tumors, Primitive/complications , Seizures/etiology , Anticonvulsants/therapeutic use , Brain Neoplasms/pathology , Brain Neoplasms/therapy , Diagnosis, Differential , Electroencephalography , Epilepsy, Reflex/drug therapy , Epilepsy, Reflex/pathology , Epilepsy, Reflex/physiopathology , Female , Humans , Magnetic Resonance Imaging , Neuroectodermal Tumors, Primitive/pathology , Neuroectodermal Tumors, Primitive/therapy , Seizures/drug therapy , Seizures/pathology , Seizures/physiopathology , Treatment Outcome , Valproic Acid/therapeutic use , Young AdultABSTRACT
OBJECTIVE: Reports on zonisamide monotherapy are limited despite favourable preliminary data, and typically restricted to tertiary referral centres. The goal of this study is to report clinical experience with zonisamide monotherapy in a large, multi-group clinic setting. METHODS: We reviewed the charts of patients treated with zonisamide monotherapy in the Neurology Department of the Kelsey-Seybold Clinic (Houston, Texas) during an 18-month period. We analysed subgroups of patients who were naive to antiepileptic drug (AED) therapy (Group 1) and those who had previous exposure to AEDs (Group 2). RESULTS: The study included 54 paediatric and adult patients with a variety of seizure types: 15 patients in Group 1 and 39 patients in Group 2. Mean maintenance zonisamide dosages in the two groups were similar (193 mg/day in Group 1 vs. 218 mg/day in Group 2). Thirty-eight patients (70.4%) continued zonisamide monotherapy, with 7 patients (13.0%) adding a second AED and 9 patients (16.7%) switching to a different drug. Of the 24 patients who became seizure free on zonisamide monotherapy, 11 were on the 100-mg initial dosage. Zonisamide monotherapy was well tolerated. CONCLUSIONS: Zonisamide monotherapy is safe and effective for a variety of seizure types and may be appropriate as first-line therapy in some cases.
