ABSTRACT:A thirty-eight-year-old man presented with a six year history of symptoms resembling an anterior horn cell disorder. There was progressive upper extremity wasting and weakness in the absence of sensory complaints. Electrophysiologic abnormalities were confined to motor nerve conduction and indicated a demyelinating process involving the brachial plexus and major proximal upper extremity nerve trucks bilaterally. Biopsy of the proximal right ulnar nerve revealed changes suggesting a chronic demyelinating process, and onion-bulb formations were present. Immunohistochemical staining for S-100 protein was positive in the cells comprising the onion-bulbs, indicating a Schwann cell, not a perineurial origin of these cells. After 8 years, symptoms have failed to appear in the lower limbs. Recent reports in the literature have begun to delineate the syndrome, which appears to represent an unusual, localized or multifocal, sometimes inflammatory, clinically benign neuropathy that can mimic motor neuron disease in its earlier stages. We report the first such case with underlying pathology.
Summary: Purpose:The surgical treatment of medically intractable temporal lobe epilepsy includes the resection of temporal lobe structures. Although the reported seizure-free outcomes are highly variable, there is growing evidence that the extent of resection of the mesiotemporal lobe directly correlates with seizure control.Methods: A moveable, high-field intraoperative magnetic resonance (MR) system was used to monitor and optimize the resection of the amygdala and hippocampus in 14 epilepsy patients. Fourteen patients with intractable seizures of temporal lobe origin underwent standard preoperative investigations including MR imaging, EEG telemetry, single-photon emission computed tomography, and neuropsychologic and sodium amytal testing. Anterior temporal lobectomy was performed on 10 patients, whereas four were treated with selective amygdalohippocampectomy. Intraoperative electrocorticography was applied as required. For all procedures, the objective was to resect the amygdala completely, and hippocampus to the posterior margin of the brainstem.Results: Interdissection intraoperative MR imaging taken when optimal resection was thought to have been achieved revealed residual unresected amygdala or hippocampus in seven of 14 patients. An unexpected acute hematoma was found in one patient. At 17 months' follow-up, 13 (93%) of 14 patients are seizure free or have significantly improved seizure control.Conclusions: The mobile high-field intraoperative MR system provides high-resolution images without restriction on surgical instruments or techniques. The ability to identify and resect residual mesial temporal lobe targets before craniotomy closure is of potentially tremendous value in optimizing seizure control.
Anti-phospholipid antibodies (APA), initially described with SLE, have in recent years received much attention because of an associated increased risk of thrombo-embolic disease, recurrent abortion and thrombocytopenia. Although commonly seen with SLE or other collagen vascular diseases, the antibodies frequently occur in the absence of any such disease. Neurologic complications include transient or permanent ischemic episodes, migraine or related phenomena, myelopathy and a Guillain-Barré type syndrome. In this report we describe the presenting features and clinical course of six patients with anti-phospholipid antibodies where migraine was an early and prominent symptom. All six patients, however, were recognized only after a second more serious event had occurred. As this entity becomes more widely recognized and better treatments evolve an earlier diagnosis of patients with migraine as the only manifestation of APA may prevent the development of other serious complications.
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