1/108. Acute axonal polyneuropathy in chronic alcoholism and malnutrition. In contrast to the classic, slowly progressive polyneuropathy in alcoholic patients, acute forms, clinically mimicking guillain-barre syndrome, are rare. We present a patient who developed motor weakness and sensory loss in all four limbs within four days. Laboratory data were consistent with long-term alcohol abuse and documented thiamine deficiency. Repeated cerebrospinal fluid examinations were normal. Electrophysiological studies showed an acute sensorimotor polyneuropathy with predominantly axonal involvement. We conclude that acute alcoholic neuropathy has to be distinguished from guillain-barre syndrome and other forms of acute polyneuropathy by using clinical, laboratory, and electrophysiological data. Both ethanol toxicity and vitamin deficiency could play a role in the pathogenesis. ( info) |
2/108. Spontaneous expulsion of large vesicle calculi in a woman with paraparesis. INTRODUCTION: urolithiasis is a common but preventable complication of spinal cord Disorders (SCD). CASE REPORT: We report a 25-year-old woman with paraparesis who spontaneously passed two large calculi perurethra without pain and developed urethral scarring. Detrusor hyperreflexia, absence of sensations and lack of sphincter tone could have contributed to painless expulsion of the large calculi in this patient. CONCLUSION: dysuria, a prominent symptom of urolithiasis may not be present in subjects with SCD. awareness about urolithiasis among health professionals involved in the care of SCD patients is necessary for prevention and early intervention. ( info) |
3/108. filtration of cerebrospinal fluid for acute demyelinating neuropathy in systemic lupus erythematosus. We report a patient with systemic lupus erythematosus complicated by an acute demyelinating neuropathy. Conventional therapy with intravenous immunoglobulins and immunoadsorption complemented by pulse methylprednisolone and cyclophosphamide failed. Institution of filtration of the cerebrospinal fluid was followed by a rapid improvement of the paresis. ( info) |
4/108. Cervical and thoracic juxtafacet cysts causing neurologic deficits. STUDY DESIGN: case reports and review of the literature. OBJECTIVES: To review the clinical features, treatment, and outcome of juxtafacet cysts. SUMMARY OF BACKGROUND DATA: There have previously been 4 reported cases of thoracic juxtafacet cysts and 19 cases of cervical juxtafacet cysts. Cervical cysts have usually originated from the cruciate ligament and caused myelopathy. Thoracic cysts are usually signaled by myelopathy. methods: The records of the neurosurgery Department of Royal Adelaide Hospital from 1980 through 1995 were reviewed for cases of intraspinal juxtafacet cysts. RESULTS: Eight cases of intraspinal juxtafacet cysts were identified; six were in the lumbar spine. One patient had a cervical cyst related to a facet joint and had unilateral radiculopathy. A second patient with a thoracic cyst had the gradual onset of myelopathy. Both patients had surgical excision of the cyst without resection of the adherent dura. The symptoms and neurologic signs improved in each case. CONCLUSIONS: Cervical and thoracic juxtafacet cysts are rare lesions that are usually signaled by myelopathy. Results of surgery are excellent in most cases, even if the cyst is not completely excised. ( info) |
| We report a case of decompression illness in which the patient developed paraparesis during scuba diving after rapid ascent. MRI of the spine revealed a focal intramedullary lesion consistent with the symptoms. The pathophysiological and radiological aspects of spinal decompression illness are discussed. ( info) |
6/108. hydroxyurea therapy in paraparesis and cauda equina syndrome due to extramedullary haematopoiesis in thalassaemia: improvement of clinical and haematological parameters. patients with beta-globin disorders show amelioration of clinical condition by sustained synthesis of fetal haemoglobin in adult life. We report data on a patient with beta(o)-thalassaemia genotype and thalassaemia intermedia clinical phenotype. He received therapy with hydroxyurea (20 mg/kg/d) because of the presence of extramedullary masses causing paraparesis, neurogenic bladder and impotence. During therapy, the patient showed an improved clinical picture and a significant increase in total Hb (from 71.8 to 103.2 g/L) and a gamma/alpha globin synthetic ratio (from 0.39 to 0.68). The myelosuppressive effect of hydroxyurea was revealed by a decrease in CFU-GEMM, BFU-E, and CFU-GM. Therefore hydroxyurea can be effective in the treatment of patients with extramedullary haematopoiesis (EMH) who are not transfusion-dependent and cannot be treated with radiotherapy. ( info) |
7/108. guillain-barre syndrome: perspectives with infants and children. An acute flaccid paraparesis or ascending quadriparesis in an infant or child constitutes a very important pediatric neurology emergency. The guillain-barre syndrome (GBS) is the most frequent cause. This is primarily an autoimmune, post-infectious, demyelinating, peripheral nervous system process. A small percentage of children develop a primary axonal process not unlike that identified more commonly in china. Because of the potential for acute respiratory compromise, any child suspected of having GBS needs immediate hospitalization. The major considerations in differential diagnosis include transverse myelitis, toxic neuropathies, tick paralysis, infantile botulism, myasthenia gravis, and dermatomyositis. On occasion, some younger children present with an acute severe pain syndrome that may mask as a pseudo-encephalopathy. Another clinical variant is the Miller-Fisher syndrome characterized by ataxia, ophthalmoparesis, and areflexia. This is associated with a high frequency of the anti-GQ-1-b antibodies. Although most children with GBS have a relatively benign clinical course, some become very ill and require intubation with intensive care monitoring. Immunomodulating treatment should be used for any child who loses the ability to walk. To date, no well-controlled study has been completed analyzing the relative merits of the two most commonly used therapies, namely plasmapheresis or intravenously administered immunoglobulin. ( info) |
| A diagnostic lumbar puncture was performed in a 12-year-old male with acute lymphoblastic leukemia. Because of thrombocytopenia (platelet count 42,000/mm(3)), a platelet transfusion was given immediately before the lumbar puncture. However, the platelet count was not re-examined. The patient developed progressive paraparesis shortly after the lumbar puncture. magnetic resonance imaging revealed an extensive spinal subdural hematoma from the T2 to S2 level. This case report illustrates the sometimes dramatic consequences of lumbar puncture in patients with childhood leukemia. Guidelines for the examination of the platelet count and correction of thrombocytopenia before lumbar puncture are discussed. ( info) |
| Pheochromocytomas are a feature of the von hippel-lindau disease spectrum, a multisystem disorder of autosomal dominant inheritance. Pheochromocytomas are, however, observed during life with a lower frequency than other features of this disease, such as retinal angiomas, haemangioblastomas of the CNS, and renal carcinomas. We present the highly unusual case of a patient who required an emergency operation for an intradural extramedullary thoracic tumour which was clinically suggestive initially of neurinoma. We present evidence from NMR, histological and isotope scan investigations of this being a pheochromocytoma metastasis and of an additional right-sided paraganglioma at the same height. A detailed history revealed that this patient had suffered from four other pheochromocytomas and two other paragangliomas, in addition to retinal angiomatosis of von hippel-lindau disease. This case is extraordinary due to (i) the unusual site of the metastasis, (ii) the neurological requirement for an emergency operation of pheochromocytoma, (iii) metastasis of pheochromocytoma in von hippel-lindau disease (only eight previous cases), and (iv) the number of recurrent pheochromocytomas. It clearly demonstrates the necessity for frequent and life-long follow-up in von hippel-lindau disease. ( info) |
10/108. Enlargement of a chronic aseptic lumbar epidural abscess by intraspinal injections--a rare cause of progressive paraparesis. The frequent use of invasive procedures at the spinal cord such as epidural injections has led to an increased incidence of iatrogenic abscesses. We report the case of a patient who suffered from low back pain. During epidural lumbar injections of steroids the patient developed severe radicular symptoms, resulting in severe paraparesis. We demonstrate the rare cause of this progressive deterioration, being a combination of a preexisting chronic aseptic epidural abscess and an iatrogenic enlargement by repeated epidural injections. MR-Scans demonstrated a mass lesion at the L4/5 vertebral level, which was surgically removed. Histological evaluation revealed the presence of a chronic aseptic spinal epidural abscess with acute bleedings. histology and MR-Data disclosed multiple deposits of the applied drug within the abscess and in the surrounding paravertebral soft tissue. The authors prove that the cause of the neurological deterioration was due to epidural injections into a preexisting lumbar chronic aseptic epidural abscess. Harmful and unpleasant complications may occur following epidural injections. Though we present a very rare cause of such complications, a careful monitoring of the neurological status of the patient is necessary as well as the early application of MR imaging in the case of deterioration. ( info) |