1/156. Abnormal liver function tests following inadvertent inhalation of volatile hydrocarbons.The use of aerosols containing volatile hydrocarbons in conditions of poor ventilation can result in accidental overexposure which can cause central nervous system effects and hepatic injury. We present a case in which inadvertent usage of an adhesive spray used to make greeting cards resulted in vague neurological symptoms and abnormal liver function tests both of which fully resolved on discontinuation.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/156. Apparent central nervous system depression in infants after the use of topical brimonidine.PURPOSE: To report two cases in which topical brimonidine resulted in apparent central nervous system depression and unresponsiveness in an infant. methods: review of two patients. An 11-day-old infant became lethargic and apneic after a single drop of brimonidine. These symptoms were reproduced after a second administration of brimonidine. A 5-month-old infant became lethargic and poorly responsive after receiving 1 drop of brimonidine in each eye. RESULTS: The first patient required admission to the hospital for medical stabilization. He recovered without sequelae. The second patient recovered spontaneously approximately 2.5 hours after administration of brimonidine. CONCLUSIONS: Topical brimonidine may be associated with central nervous system depression in infants. The use of brimonidine is not recommended in these patients until further data are available.- - - - - - - - - - ranking = 6keywords = nervous system (Clic here for more details about this article) |
3/156. Neurologic symptoms in children with systemic lupus erythematosus.Neurologic complications of systemic lupus cerebritis are not as well known in children as in adults. Twenty-five children with neurologic complications were identified after reviewing the hospital medical records of 86 children with systemic lupus erythematosus. Seven children (28%) had neurologic symptoms at the time of initial diagnosis of systemic lupus erythematosus; median time between diagnosis of systemic lupus erythematosus and onset of neurologic complications was 1 month (range 0-5 years). seizures were the most common neurologic symptoms overall, but headaches were the most frequent neurologic manifestation in children without a previous diagnosis of systemic lupus erythematosus. Sixteen children had seizures, and 12 children had seizures as the initial central nervous system involvement. Almost all children who developed seizures had an established diagnosis of systemic lupus erythematosus; only one child had seizures that led to the diagnosis of systemic lupus erythematosus. No patient had status epilepticus, and, in general, seizures were not difficult to control. In six children, headache was the initial symptom of central nervous system involvement. Five children had lupus cerebritis, three children had stroke, and two had isolated cranial neuropathies. chorea was seen in only two cases, and three children had pseudotumor cerebri. Treatment with high-dose intravenous methylprednisolone led to a good response in 18 children; cyclophosphamide was required in 6 patients and plasmapheresis in 1 child. Outcome was generally good, although one child developed fulminant cerebritis with intracranial hypertension and died.- - - - - - - - - - ranking = 2keywords = nervous system (Clic here for more details about this article) |
4/156. recurrence of acute disseminated encephalomyelitis at the previously affected brain site.BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a usually monophasic demyelinating disorder of the central nervous system. Recurrences pose a diagnostic challenge because they can be overlooked or suggest an alternative diagnosis. OBJECTIVE: To examine the frequency, nature, and outcome of recurrent ADEM. DESIGN: review of the medical records of patients diagnosed in our institution as having ADEM between January 1, 1983, and May 31, 1998. Recurrences were defined as appearance of new symptoms and signs at least 1 month after the previous episode. RESULTS: Five (24%) of 21 patients with ADEM developed recurrent disease episodes. In all, diagnosis was confirmed by brain biopsy. One patient had 4 disease episodes, 2 had 3, and the other 2 each had 2. recurrence appeared 1.5 to 32 months after initial presentation and involved the same brain territory in 6 of 9 recurrences in 3 of 5 patients. In 2 patients, recurrences included neuropsychiatric signs. A good response to corticosteroid therapy was observed in 10 of 13 of treated ADEM attacks: in 3 of the 4 treated initial events and in 7 of 9 recurrences. CONCLUSIONS: Recurrent ADEM may be more prevalent than previously recognized. patients who relapse tend to have more than 1 recurrence that usually involves, clinically and radiologically, a brain territory that was affected before and can simulate a space-occupying lesion that requires histologic diagnosis. Neuropsychiatric features may be the main presentation of a relapse. Since recurrent ADEM is a corticosteroid-responsive condition, awareness and early diagnosis are mandatory.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
5/156. Navajo neurohepatopathy: a mitochondrial DNA depletion syndrome?Navajo neurohepatopathy (NNH) is an autosomal recessive disease of full-blooded Navajo children living in the Navajo Reservation of southwestern united states. Clinical features of NNH include peripheral and central nervous system involvement, acral mutilation, corneal scarring or ulceration, liver failure, and metabolic and immunologic derangement. The cause of NNH is unknown, but the clinical features of NNH are similar to those of patients with mitochondrial DNA (mtDNA) depletion. Therefore, we studied mtDNA concentration in the liver from 2 patients with NNH. Using histochemical, biochemical, and molecular techniques, we found evidence of mtDNA depletion, and we propose that the primary defect in NNH is in the nuclear regulation of mtDNA copy number.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
6/156. adult-onset xeroderma pigmentosum neurological disease--observations in an autopsy case.xeroderma pigmentosum (XP) is an inherited disease with defective dna repair. patients develop skin cancer because of unrepaired dna damage produced by the ultraviolet radiation (UV) in sunlight. Many XP children also develop XP neurological disease (ND), consisting of sensorineural hearing loss (SNHL) and a primary neuronal degeneration of the central and peripheral nervous systems. Since the harmful UV in sunlight cannot reach the nervous system, the cause of the death of XP neurons has been hypothesized to result from the inability to repair their DNA that has been damaged by endogenous metabolites. Progressive XP ND originating in an adult has been identified in only a single case. Although clinically asymptomatic at the age of 47 years, the patient had audiometric evidence of a developing mild SNHL together with elicited signs and electrophysiologic evidence of a peripheral neuropathy. She died of metastatic endocervical adenocarcinoma at 49 years of age. We describe here the neuropathological findings in this patient, including examination of the inner ear. Despite clinical evidence of SNHL, there were no anatomic abnormalities of the inner ear. However, the dorsal root ganglia (DRG) showed ongoing neuronal loss. Our findings indicate that XP ND originating in this adult is, like XP ND in children, a primary neuronal degeneration that manifests first in the peripheral nervous system.- - - - - - - - - - ranking = 3keywords = nervous system (Clic here for more details about this article) |
7/156. The neurological masquerade of intravascular lymphomatosis.BACKGROUND: Intravascular lymphomatosis (IVL) is an uncommon systemic disease characterized by occlusion of small vessels by malignant lymphomatous cells. central nervous system involvement usually presents as subacute encephalopathy, dementia, seizures, or multifocal cerebrovascular events. OBJECTIVE: To increase awareness about IVL, an uncommon cause of neurological disease. DESIGN: This is a retrospective case series of 8 pathologically proved cases of IVL with neurological disease. patients were part of a pathological series collected between April 1962 and October 1998 at indiana University School of medicine and the Armed Forces Institute of pathology, washington, DC. SETTING: Neurological and neuropathological examinations were performed at tertiary referral hospitals. patients: Eleven patients were diagnosed pathologically as having IVL, but 3 were not included in this evaluation because of a lack of appropriate clinical information. Of the final sample (n = 8), there were 4 men and 4 women (mean /- SD age, 62.9 /- 9.9 years). RESULTS: All 8 patients had focal neurological deficits, 7 had encephalopathy or dementia, 5 had epileptic seizures, and 2 had myelopathy. death occurred at a mean of 7.7 months (range, 1-24 months) after the onset of symptoms. All patients had elevated cerebrospinal fluid protein levels, 4 had pleocytosis, and 2 had an elevated IgG level in their cerebrospinal fluid. Of the 4 patients who underwent a brain biopsy, 1 was diagnosed as having IVL before death. CONCLUSIONS: Intravascular lymphomatosis is an uncommon disease with a myriad of potential neurological manifestations. Diagnosis requires a high index of suspicion and a pathological examination. If diagnosed early, aggressive chemotherapy is potentially curative, although the overall prognosis remains dismal.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
8/156. Diagnostic and therapeutic quandaries in primary manifestation of Hodgkin's disease in the central nervous system.We report the case of a 23-year-old female with severe neurologic dysfunction without a clear cause at the time of initial presentation. The search for an underlying malignancy revealed a slightly enlarged cervical lymph node with Hodgkin's disease (HD). There was no evidence of a brain tumor despite nonspecific bright changes in proton density in the basal ganglia of the right hemisphere of the cerebellum, right cerebellar tonsil, posterior limb of the internal capsule, and the right side of the medulla spinae as shown by magnetic resonance imaging (MRI) as well as reactive lymphocytosis with slightly elevated protein levels in the cerebrospinal fluid (CSF). The findings suggested a cerebellar disorder, with main differential diagnosis between neurologic paraneoplastic syndrome (NPS) and HD involving the CNS. Based on limited experience with NPS and HD in the CNS, possible diagnostic and therapeutic options are discussed.- - - - - - - - - - ranking = 4keywords = nervous system (Clic here for more details about this article) |
9/156. Neuro-ophthalmic sarcoidosis.sarcoidosis is a multisystem disorder in which ocular involvement occurs in about one-quarter and neurosarcoidosis in 7 per cent of patients. When the retina is involved, the reported incidence of central nervous system sarcoidosis is 37 per cent. The patient described had a transient papular eruption of the legs, bilateral hilar lymphadenopathy, polyarthralgia with knee effusions, and bilateral facial and peripheral neuropathy. Ocular involvement was characterized by anterior uveitis (in the initial stages), vitreous flare, bilateral disc oedema, macular oedema, streak haemorrhages, peripheral periphlebitis, nerve fibre bundle defects, and candle-wax spots. fluorescein angiography showed no fluorescence of the candle-wax spots nor of the adjacent vessels. However, there was hyperfluorescence of two retinal lesions. This patient had unilateral internal ophthalmoplegia, only three cases of which have been reported in the literature. Her health was restored by heavy, prolonged corticosteroid therapy. Her family history revealed that an uncle died of sarcoidosis complicated by cryptococcal meningitis. The literature on retinopathy in sarcoidosis is reviewed and the lesions noted in the posterior segment are listed.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
10/156. Neurologic services in sub-Saharan africa: a case study among Zambian primary healthcare workers.INTRODUCTION: In many parts of the developing world, access to physician consultation and neurologic expertise is limited or nonexistent. We conducted a survey among non-physician, primary healthcare workers (PHCWs) to determine the neurological needs and services in rural zambia. methods: Semi-structured written questionnaire utilizing fill-in-the-blank, multiple-choice likert-scaled questions, and open-ended questions. RESULTS: seizures were reported as the most common neurologic disorder by 66% of the PHCWs. Only 1/3 of PHCWs reported feeling adequately trained to care for seizures and seizure disorders. PHCWs reported even less expertise for other neurologic conditions. Over 40% of PHCWs surveyed work in primary care clinics without a physician available for consultation. Their patients must travel a median of 50 km to access a physician and geographic barriers are a frequent problem. In addition to difficulty physically accessing care, PHCWs reported that financial barriers to physician referral are substantial. Expenses cited include additional user fees for physician-level care, transportation costs, and the cost of maintaining the patient and/or family at a site distant from the home village. Traditional beliefs, social stigma, and discriminatory healthcare policies associated with neurologic conditions were also noted to deter and defer care and care seeking. CONCLUSIONS: PHCWs lack sufficient training and experience to care for the neurologic disorders in their patient populations, although such disorders are relatively common. Geographic, financial and cultural barriers substantially limit physician referrals. To assure at least a minimal quality of care for people with nervous system disorders in zambia, PHCWs' neurologic education must be increased and barriers to physician referral decreased.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
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