Lyons, Mark

Pial arteriovenous fistulae (AVFs) are rare vascular abnormalities that are distinct from arteriovenous malformations and dural AVFs. These vascular lesions have been linked with trauma, ischemic syndromes, venous thrombotic diseases, and intracranial surgical procedures. In this report we describe a case of an intracranial ruptured pial AVF immediately following uneventful spinal surgery in an elderly patient with subsequent spontaneous resolution. He was a previous heavy smoker with a 60-pack-year history and alcohol abuse. His examination was positive for morbid obesity and mild weakness of the anterior tibialis and gastrocnemius muscles bilaterally. He underwent uneventful spinal surgery, suffering a generalized seizure shortly after extubation. Imaging studies demonstrated acute subarachnoid hemorrhage and cerebral angiography identified a pial AVF. He was stabilized medically and follow-up angiography demonstrated spontaneous resolution of the pial AVF. This case highlights a rare vascular malformation with rupture following uneventful spinal surgery.

Abstract Thirty patients with histologically confirmed posterior fossa ependymomas operated on between January 1976 and December 1988 were reviewed. The median age was 44 years (range, 1-69 yr). There were 7 children (aged 5 yr or younger) and 23 adults (aged 16 yr or older). There were 18 female patients and 12 male patients. Headache, nausea and vomiting, and disequilibrium were the most frequent symptoms. The most common findings were ataxia and nystagmus. Gross total resection was performed in 8 patients (27%), subtotal resection in 21 patients (70%), and biopsy in only 1 patient (3%), Tumors were low grade in 73% and high grade in 27%. Twenty-seven patients underwent posterior fossa radiotherapy (median dose, 5400 cGy). Fourteen patients also underwent spinal irradiation (median dose, 3520 cGy). Age was the only significant prognostic factor identified (P <0.01). The 5-year survival rates were 76% for adults and 14% for children. All 14 patients who died had recurrent or residual tumor at the primary site. This review suggests that in patients with primary posterior fossa ependymomas the following is true: 1) the young patient (5 yr old or younger) has a poor prognosis: 2) there was a trend toward a better 5-year survival rate with a gross total resection; 3) if recurrence occurs, it will be at the primary intracranial site; and 4) symptomatic spinal seeding does not occur frequently.

When coupled with classical clinical signs and symptoms, magnetic resonance imaging can significantly aid in the diagnosis of multiple sclerosis (MS). However, the differential diagnosis of multiple sclerosis is large and, in the absence of pathognomonic clinical features, can be challenging to diagnose initially. Some conditions, such as primary central nervous system lymphoma (PCNSL) and progressive multifocal leukoencephalopathy (PML), can mimic clinically some of the symptoms prominent in multiple sclerosis. Early treatment with corticosteroids can dramatically improve patient symptoms in MS and PCNSL. We report a case of a man diagnosed with histologically confirmed relapsing remitting multiple sclerosis who subsequently developed histologically confirmed primary central nervous system lymphoma. Immunosuppressant therapy in the treatment of multiple sclerosis may be a potential catalyst in the development of central nervous system lymphoma. The course of his disease and treatment are presented and the current literature reviewed.

Object Meige syndrome is characterized by blepharospasm, cervical dystonia, and facial oromandibular dystonia. The medical treatment of this condition is largely unsuccessful over time and is a major source of decreased quality of life in those patients suffering from this disease. Recent advances in the application of deep brain stimulation (DBS) surgery techniques for many disorders have prompted several recent reports of DBS for medically refractory cases of Meige syndrome. While the etiology for this disorder is unknown, it is considered by many investigators to be a form of idiopathic torsion dystonia. Pallidal stimulation is widely considered to be effective for dystonia. Methods The authors report the long-term results of bilateral globus pallidus internus (GPi) or subthalamic nucleus (STN) stimulation in 3 patients with Meige syndrome and 1 patient with Parkinson disease and associated craniofacial dystonia treated at their center. Results Initial 12-month and long-term follow-up Burke-Fahn-Marsden scores were substantially improved in all 4 patients compared with preoperative scores. Conclusions Bilateral GPi DBS may be an effective and safe treatment for medically refractory Meige syndrome. The results are comparable with those reported in the literature. Sustained and long-term improvement in symptoms does appear to be reproducible across reports. The authors' patient with Parkinson disease and associated craniofacial dystonia syndrome undergoing bilateral STN DBS noted immediate and sustained improvement in his symptoms. Further study is required, but these results, along with the other reports, suggest that bilateral GPi DBS is an effective treatment for medically refractory Meige syndrome.

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a severe primary headache disorder that is often refractory to medical therapy. Although the pathogenesis of this and other trigeminal autonomic cephalalgias is not completely understood, ipsilateral activation of the posterior and inferior hypothalamus has been identified on functional imaging studies during attacks. The authors report on a case of SUNCT syndrome successfully treated with hypothalamic deep brain stimulation and discuss the current literature.

✓The aim of this study is to evaluate the presentation of unusual dementias in 4 patients and correlate these presentations with the histopathological findings after brain biopsy sampling to define a diagnostic entity. The authors detail the cases of 4 patients (2 women, 46 and 56 years of age; and 2 men, 67 and 74 years of age) who presented with acute and subsequently subacute encephalopathies. Their bizarre presenting symptom complexes and the results of neurological examinations, radiographic studies, electroencephalography (EEG) recordings, and serological marker tests are reviewed. The diagnostic surgical procedures and successful treatments in these patients are discussed. All 4 patients underwent craniotomy and excisional brain biopsy sampling as part of a comprehensive evaluation. Histopathological confirmation of nonvasculitic autoimmune inflammatory meningoencephalitis (NAIM) by a neuropathologist was present in all 4 patients. All patients were successfully treated with a course of corticosteroid medication. Nonvasculitic autoimmune inflammatory meningoencephalitis is a potentially treatable form of dementia whose salient features include a younger average age at onset, rapid progression with significant cognitive and behavioral features, absence of family history, abnormal EEG findings, and elevated levels of inflammatory markers. Although it is an invasive procedure, brain biopsy sampling is safe and can offer histological confirmation of this condition. This procedure is important because NAIM can be treated successfully if it is distinguished from the nontreatable dementias that mimic it.