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1/800. Steroid-responsive multifocal demyelinating neuropathy with central involvement.

    We describe 2 patients with associated central and peripheral demyelination. Electrophysiological studies revealed a demyelinating polyneuropathy with sensory and motor conduction blocks. Visual evoked potentials were abnormal. Motor evoked potentials showed abnormal central conduction time in 1 patient. magnetic resonance imaging revealed regions of abnormal high signal in the spinal cord and brain; sural nerve biopsy disclosed a demyelinating neuropathy. Both patients showed clinical and electrophysiological improvement after steroid therapy. ( info)

2/800. Control of primary angiitis of the CNS associated with cerebral amyloid angiopathy by cyclophosphamide alone.

    Corticosteroids combined with cyclophosphamide are currently recommended for primary angiitis of the CNS. The authors report a 71-year-old man with primary angiitis of the CNS and amyloid angiopathy who responded to cyclophosphamide without steroids, suggesting that corticosteroids may not be needed in all cases of primary angiitis of the CNS. ( info)

3/800. arachnoid cyst of the middle fossa with paradoxical changes of the bony structures.

    Two patients with an arachnoid cyst of the middle fossa showed paradoxical changes of the adjoining bony structures of the skull. There was a diminution of the middle fossa and hyperplasia of the sphenoid sinus (pneumosinus dilatans) as well as a marked bulging of the squamous part of the temporal bone. In one case in which scinticisternography was performed, communication between the cyst and the subarachnoidal space was proven as well as an extremely slow cerebrospinal fluid circulation in the cyst. The pathogenesis of the cyst is discussed, based upon the structural changes of the skull, the angiographic findings and the locally disturbed cerebrospinal fluid circulation. The primary disturbance seems to be a temporal lobe agenesis. ( info)

4/800. thrombosis related to the use of L-asparaginase in adults with acute lymphoblastic leukemia: a need to consider coagulation monitoring and clotting factor replacement.

    L-asparaginase (L-asp) has become an important component of combination chemotherapy for acute lymphoblastic leukemia (ALL). However, L-asp can produce depletions in many of the clotting factors with an associated risk for thrombosis and hemorrhage. Three consecutive patients seen at the Mayo Clinic with L-asp related thrombosis are described and an in-depth review of the literature is provided. Two of the 3 patients developed central nervous system (CNS) complications with evidence of thrombosis and hemorrhagic infarction. Two of the patients also developed extensive upper extremity thrombosis. The results of comprehensive hemostatic surveys showed marked abnormalities in all 3 patients. Many of the thrombotic complications related to L-asp involve the CNS, as illustrated in 2 of our patients. These patients should be treated aggressively since full recovery is possible. The precise cause of thrombosis is yet to be determined but is likely multifactorial. The optimal treatment and prevention of thrombosis in this group of patients remains poorly defined. ( info)

5/800. E.N.T. manifestations of Von Recklinghausen's disease.

    Von Recklinghausen's disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade. ( info)

6/800. hepatic encephalopathy--a physostigmine-reactive central anticholinergic syndrome?

    This report describes an association between hepatic encephalopathy and central anticholinergic syndrome (CAS). A 60-year-old anaemic woman was admitted unconscious and with a delayed reaction to pain but with no focal neurological deficits. She had signs of portal hypertension and a history of non-alcoholic liver cirrhosis grade child B. Suspecting upper gastro-intestinal bleeding, she was intubated for gastro-duodenoscopy and a fibrin-covered ulcer was revealed. Raised intra-abdominal pressure resulting from ascites caused cardiopulmonary failure, which required mechanical ventilation for 24 h, but extubation was possible after drainage of the ascites and blood volume replacement therapy. However, her neurological state remained unchanged despite normal blood ammonia concentration and no sedation. CAS was considered and physostigmine injected with immediate effect. The patient opened her eyes immediately and was fully orientated to personal and medical history. We suggest that hepatic encephalopathy may trigger CAS, although the significance of physostigmine in the treatment of hepatic encephalopathy remains to be addressed by controlled investigations. ( info)

7/800. CNS pseudovasculitis in a patient with pheochromocytoma.

    The authors report a patient with angiographic findings resembling CNS vasculitis (CNS pseudovasculitis) who was found to have a pheochromocytoma. The angiographic changes resolved after surgical resection of the pheochromocytoma. pheochromocytoma should be included in the differential diagnosis of angiographic findings suggestive of CNS vasculitis. ( info)

8/800. magnetic resonance imaging anatomy of the cranial nerves.

    Correlation of findings on neurological examination of the cranial nerves with gross anatomy, neuroanatomy, and neuropathology provides the optimal diagnostic use of magnetic resonance imaging (MRI). Illustrations of the anatomy of each of the cranial nerves, with concise labeling of relevant anatomical relationships, are compared with brief neurological summaries and MRIs of patients. ( info)

9/800. fatal outcome due to cyclosporine neurotoxicity with associated pathological findings.

    We present a case of death likely to be directly due to cyclosporine (CsA) neurotoxicity. To date, there have been no reports of deaths directly due to CsA neurotoxicity, nor has an associated histological lesion been described independent of confounding processes. A 54-year-old male received an HLA-matched-unrelated BMT for CML. He developed progressive encephalopathy and on day 79 had a generalized seizure. All CSF studies were negative for infectious causes. MRI revealed diffuse, symmetrical white matter abnormalities located in the occipital sub-cortex, thalamus, mid brain, pons, and cerebellum which were typical of CsA toxicity. The patient died of central respiratory failure within 72 h of discontinuing CsA. autopsy revealed diffuse patchy white matter edema and astrocytic injury without evidence of axonopathy, demyelination, microvascular injury, or infectious/inflammatory process. This case demonstrates previously undescribed lethal CsA neurotoxicity and may reveal an associated primary pathological lesion. ( info)

10/800. Acute abdominal pain as a leading symptom for Degos' disease (malignant atrophic papulosis).

    We report a case of a 16-yr-old white female patient with acute abdominal pain due to visceral involvement of Degos' disease that required extensive small bowel resection. skin manifestations of her disease had been present for 2 yr before the correct diagnosis. She died as a result of central nervous system involvement from Degos' disease. ( info)
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