Report of a Case with an Unusual Neurological Syndrome
Michael Scott and Alexander Silverstein
Alexander C. Whiting, Michael Y. Oh and Donald M. Whiting
The mechanisms of appetite disorders, such as refractory obesity and anorexia nervosa, have been vigorously studied over the last century, and these studies have shown that the central nervous system has significant involvement with, and responsibility for, the pathology associated with these diseases. Because deep brain stimulation has been shown to be a safe, efficacious, and adjustable treatment modality for a variety of other neurological disorders, it has also been studied as a possible treatment for appetite disorders. In studies of refractory obesity in animal models, the ventromedial hypothalamus, the lateral hypothalamus, and the nucleus accumbens have all demonstrated elements of success as deep brain stimulation targets. Multiple targets for deep brain stimulation have been proposed for anorexia nervosa, with research predominantly focusing on the subcallosal cingulate, the nucleus accumbens, and the stria terminalis and medial forebrain bundle. Human deep brain stimulation studies that focus specifically on refractory obesity and anorexia nervosa have been performed but with limited numbers of patients. In these studies, the target for refractory obesity has been the lateral hypothalamus, ventromedial hypothalamus, and nucleus accumbens, and the target for anorexia nervosa has been the subcallosal cingulate. These studies have shown promising findings, but further research is needed to elucidate the long-term efficacy of deep brain stimulation for the treatment of appetite disorders.
Tony P. Smith, Michael J. Alexander and David S. Enterline
✓ Three patients with carotid artery (CA) pseudoaneurysms were treated using four polyethylene terephthalate endografts (Wallgraft endoprostheses). Two patients received a single graft and one patient with bilateral pseudoaneurysms received two grafts. Complete occlusion of the pseudoaneurysm with patency of the arterial lumen was achieved following endograft placement in all patients. The clinical follow-up interval ranged from 12 to 18 months and included angiography or ultrasonography studies or both. One patient experienced neurological symptoms, and in-graft stenosis ranging from 50 to 100% occurred in three of the four grafts. Although the Wallgraft endoprosthesis produced good initial results for the treatment of cervical CA pseudoaneurysms, as demonstrated on radiography, it was associated with a high rate of stenosis or occlusion in all three patients.
Jeffrey Myers, Michael Scott and Alexander Silverstein
Alexander Semmler, Matthias Simon, Susanna Moskau and Michael Linnebank
Functionally relevant polymorphisms of methionine and folate metabolism have been shown to be associated with various human cancer entities including cerebral lymphoma and glioblastoma multiforme. The authors investigated the association of 7 functional polymorphisms of methionine metabolism with meningioma formation.
This case-controlled, monocenter association study included 290 patients of Caucasian origin undergoing surgical resection for intracranial meningioma (World Health Organization [WHO] Grade I, 190 cases; WHO Grade II, 82 cases; WHO Grade III, 18 cases) and 287 age- and sex-matched local controls. The authors analyzed the following genetic variants: dihydrofolate reductase c.594+59del19, 5,10-methylenetetrahydrofolate reductase c.677C > T and c.1298A > C, 5-methyltetrahydrofolate-homocysteine S-methyltransferase (MTR) c.2756A > G, reduced folate carrier 1 c.80G > A, cystathionine beta-synthase (CBS) c.844_855ins68 and transcobalamin 2 c.776C > G.
The variant CBS c.844_855ins68—that is, the allele carrying the insertion (“ins” or “i”) as opposed to the wild-type allele designated as deletion (“del” or “d”)—was significantly overrepresented in meningioma patients (dd/ id/ii: 0.81/0.18/0.01) in comparison with the controls (dd/id/ii: 0.88/0.12/0; 2 df, chi-square 8.97, p = 0.011; multiple nominal regression with age and sex as covariables). In addition, explorative analyses revealed an association of the MTR c.2756A > G variant with meningioma WHO Grade III (AA/AG/GG: patients, 1.0/0/0; controls, 0.64/0.32/0.04; 2 df, chi-square 14.44, p = 0.001).
The results of this study suggest that genetic variants of methionine metabolism are associated with meningioma formation.
Alexander A. Khalessi, Bryan C. Oh and Michael Y. Wang
✓ In the following literature review the authors consider the available evidence for the medical management of patients with ankylosing spondylitis (AS), and they critically assess current treatment guidelines. Medical therapy for axial disease in AS emphasizes improvement in patients' pain and overall function. First-line treatments include individualized physical therapy and nonsteroidal antiinflammatory drugs (NSAIDs) in conjunction with gastroprotective therapy. After an adequate trial of therapy with two NSAIDs exceeding 3 months or limited by medication toxicity, the patient may undergo tumor necrosis factor–α blockade therapy. Response should occur within 6–12 weeks, and patients must undergo tuberculosis screening. Evidence does not currently support the use of disease modifying antirheumatic drugs, corticosteroids, or radiotherapy in AS.
Michael J. Alexander, Antonio A. F. DeSalles and Uwamie Tomiyasu
✓ This 53-year-old man presented with a syncopal episode 31 years after undergoing craniotomy and external-beam radiation for a pituitary macroadenoma. A gadolinium-enhanced magnetic resonance (MR) image of the brain demonstrated a 2.5-cm enhancing mass in the right caudate region that had not been seen on previous studies. A stereotactically guided biopsy procedure was performed to obtain specimens from the mass, which were consistent with ependymoma. The MR image also revealed two additional lesions that appeared to be within the radiation fields: a right temporal meningioma and a left frontal cavernous malformation. A review of the literature found three previous reports in which ependymomas presented after radiation therapy.
Gayle S. Storey, Michael P. Marks, Michael Dake, Alexander M. Norbash and Gary K. Steinberg
✓ The authors report initial results and follow up using stent placement to treat atherosclerotic stenosis in vertebral arteries. Three patients with severe atherosclerotic vascular disease underwent vertebral artery stent placement using a balloon expandable stent. Medical therapy (aspirin and warfarin) and conventional percutaneous angioplasty failed to resolve the disease and the patients developed symptomatic restenosis within 3 months of angioplasty. Two patients had symptoms of anterior circulation ischemia with carotid artery occlusions and reduced supply to the anterior circulation from the stenosed vertebral arteries. One patient had recurrent posterior circulation symptoms. Stents were successfully placed in all three, resulting in immediate reversal of stenosis and resolution of symptoms. Clinical follow-up study (mean 9 months) has shown no recurrent symptoms in the patient with posterior circulation symptoms, but the two patients with anterior circulation ischemia did develop recurrent symptoms. Angiographic follow up in these two patients at 3 months and 1 year, however, demonstrated continued patency of vertebral artery lumina. They underwent extracranial—intracranial bypass surgery to relieve their symptoms. This experience suggests stents can be placed without complication in the proximal vertebral arteries and may have an adjunctive role in the treatment of atherosclerotic cerebrovascular disease following unsuccessful angioplasty.
Michael J. Rauzzino, R. Shane Tubbs, Eben Alexander III, Paul A. Grabb and W. Jerry Oakes
Neurenteric cysts are infrequently reported congenital abnormalities believed to be derived from an abnormal connection between the primitive endoderm and ectoderm. The authors report a series of 13 patients treated over a 50-year period.
Of the 13 patients, seven were female and six were male. Their ages at presentation ranged widely from 5 weeks to 52 years of age. Children presented more commonly with cutaneous stigmata of occult spinal dysraphism (OSD) whereas adults presented primarily with pain. Neurological deficit as a presenting symptom was less common in our series, a finding that reflects the slow growth of these lesions. In all but one patient some form of vertebral anomaly was associated with the cystic lesions, including two patients with Klippel–Feil abnormalities. There was a high incidence of associated forms of OSD including split cord malformation, lipoma, dermal sinus tract, and tethered spinal cord. In previous reports the authors have suggested that neurenteric cysts are more common in the cervical region and in a position ventral to the cord. In the present series these cysts most commonly occurred as intradural, extramedullary masses in the thoracolumbar region, situated dorsal to the spinal cord. The median follow-up period was 7.5 years, and postoperative outcome reflected a patient's preoperative neurological status; in no patient was outcome worsened due to surgery.
Complete excision of the neurenteric cyst remains the treatment of choice, as subtotal excision is associated with recurrence.
Pascal Jabbour, Michael Fehlings, Alexander R. Vaccaro and James S. Harrop
In this paper the authors review spine trauma and spinal cord injury (SCI) in the geriatric population. The information in this study was compiled through a literature review of clinical presentation and management of SCI in the elderly population. This was done to define, identify, and specify treatment algorithms and management strategies in this unique patient population.