Cases reported "Nervous System Diseases"

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1/538. Long-chain L-3-hydroxyacyl-coenzyme a dehydrogenase deficiency neuropathy: response to cod liver oil.

    Docosahexaenoic acid (DHA) deficiency has recently been documented in several children with long-chain L-3-hydroxyacyl-coenzyme a dehydrogenase deficiency (LCHADD). We studied a 13-year-old boy with LCHADD who had limb girdle myopathy, recurrent myoglobinuria, and progressive sensorimotor axonopathy with demyelination. At 11 years of age, he was started on cod liver oil extract, high in DHA content. Over 12 months, he demonstrated a marked clinical recovery. Nerve conduction studies (NCS) demonstrated reappearance of previously absent posterior tibial and peroneal nerve responses and the amplitudes on motor ulnar and median NCS markedly increased from 7- to 14-fold, respectively.
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ranking = 1
keywords = neuropathy, nerve
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2/538. Acute inflammatory neuropathy in charcot-marie-tooth disease.

    The authors report an association between acute inflammatory neuropathy and previously undiagnosed Charcot-Marie-Tooth 1A disease in a 15-year-old girl. sural nerve biopsy study showed hypertrophic neuropathy with endoneurial infiltrates of macrophages and lymphocytes. This association may be coincidental, but a particular susceptibility to damage of these peripheral nerves cannot be excluded. This report confirms the importance of pes cavus as a sign of long-standing sensorimotor neuropathy.
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ranking = 1.9010902716012
keywords = neuropathy, peripheral nerve, peripheral, nerve
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3/538. charcot-marie-tooth disease type I diagnosed in a 5-year-old boy after vincristine neurotoxicity, resulting in maternal diagnosis.

    charcot-marie-tooth disease type 1, also known as hereditary motor sensory neuropathy type 1, is an uncommon autosomal dominant disease that causes destruction of peripheral nerves with a varied clinical course, but often leads to muscle weakness. If the peroneal muscle is involved, the patient may develop a characteristic slapping gait. The dose-limiting side effect of the chemotherapeutic agent vincristine is usually its neurotoxicity. We report the case of a 5-year-old patient with leukemia who developed an acute polyneuropathy after treatment with vincristine. charcot-marie-tooth disease type 1 was diagnosed in the patient and, subsequently, in his mother only after vincristine toxicity was observed.
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ranking = 0.65313518445598
keywords = neuropathy, peripheral nerve, peripheral, nerve
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4/538. iliac artery pseudoaneurysm following renal transplantation presenting as lumbosacral plexopathy.

    A renal transplant patient developed chronic and progressive back and lower extremity pain followed by foot weakness. The correct diagnosis of lumbosacral plexopathy was made after electromyography and nerve conduction studies and the etiology of radiculopathy due to nerve root compression was excluded. This prompted further investigations that led to the discovery of a large internal iliac artery pseudoaneurysm. We emphasize the use of electrodiagnostic studies to investigate patients with back and limb pain for correctly localizing responsible pathology. In this case a potentially lethal situation was correctly identified in a transplant patient.
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ranking = 0.016359302838439
keywords = nerve
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5/538. Can immunoadsorption plasmapheresis be used as the first choice therapy for neuroimmunological disorders?

    The subjects were 31 patients in whom immunoadsorption plasmapheresis (IAPP) was performed as the first choice therapy for primary or recurrent neuroimmunological disorders. The clinical manifestations before and after IAPP and the use of corticosteroids were investigated in the present study. IAPP was clinically effective in all patients. The corticosteroids (CSs) administration was begun or CSs were increased after completion of IAPP in 11 patients. IAPP was performed as the first choice therapy, and favorable results were obtained in patients with guillain-barre syndrome and Miller-Fisher syndrome. IAPP alone was also effective in a patient with lupoid sclerosis. When IAPP was used alone in 2 patients with chronic inflammatory demyelinating polyradiculoneuropathy, it completely eliminated the clinical manifestations, but the symptoms recurred about 2 months later. Therefore, although IAPP could be performed as the first choice therapy for many neuroimmunological disorders, subsequent therapies should be carefully investigated.
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ranking = 0.2479550871452
keywords = neuropathy
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6/538. 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.
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ranking = 0.21849074966324
keywords = nervous system
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7/538. Diffuse acute cellulitis with severe neurological sequelae. A clinical case.

    The incidence of head and neck odontogenic infections considerably diminished in the last decades due to appropriate antibiotic therapy. Herein we describe a case of acute diffuse facial cellulitis following tooth extraction in a patient with no apparent risk factor. During the acute process, injury was caused to the hypoglossal, vagal, glossopharyngeal and recurrent nerves of both sides. For this reason the patient currently has a nasogastric line for enteral feedings and a tracheotomy tube, which significantly affects his quality of life.
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ranking = 0.0081796514192197
keywords = nerve
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8/538. 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.
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ranking = 1.3109444979794
keywords = nervous system
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9/538. Liver disease in Navajo neuropathy.

    OBJECTIVE: To describe clinical and histologic features of liver disease in infants and children with Navajo neuropathy (NN). methods: physicians at Navajo Area Indian health Service facilities and neurologists and gastroenterologists at regional referral hospitals were surveyed for identification of patients born between 1980 and 1994 with known or suspected NN. Clinical records and liver histologic findings were reviewed. RESULTS: Liver disease was present in all children with NN. Three clinical phenotypes of NN were observed, based on age at presentation and course: infantile NN presented in 5 infants before 6 months of age with jaundice and failure to thrive and progressed to liver failure before 2 years of age; childhood NN presented in 6 children between 1 and 5 years of age with liver dysfunction, which progressed to liver failure and death within 6 months; and classical NN presented in 9 children with variable onset of liver disease but progressive neurologic deterioration. Liver histologic findings were characterized by multinucleate giant cells, macrovesicular and microvesicular steatosis, pseudo-acini, inflammation, cholestasis, and bridging fibrosis and cirrhosis. Cases of all 3 phenotypes occurred within the same kindred. CONCLUSIONS: Liver disease is an important component of NN and may be the predominant feature in infants and young children. We propose changing the name of this disease to Navajo neurohepatopathy.
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ranking = 1.239775435726
keywords = neuropathy
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10/538. Detection of skin over cysts with Spina bifida may be useful not only for preventing neurological damage during labor but also for predicting fetal prognosis.

    Spina bifida is one of the most common open neural tube defects. There are two common types of spina bifida cystica, myelomeningocele and meningocele. Special attention to the thickness of the cystic sac (presence of intact skin and subcutaneous tissue) on magnetic resonance imaging is advantageous for determination of whether the child will profit from cesarean section in order to prevent neurological change (infection and drying of nerve tissue) and for management of spina bifida (most meningocele) during the perinatal period. Furthermore, skin detection may help to predict the prognosis of spina bifida after birth. meningocele, with intact skin over the cyst, has a better clinical course than myelomeningocele. Some myelomeningoceles with neural tube defects in a lower position, also frequently having an intact skin over the cyst, have almost the same clinical course as a meningocele. From this, we hypothesize that a baby with spina bifida who has intact skin over the cyst might have a good prognosis neurologically. In this report, we concentrate attention on the skin over cysts in 3 cases (1 meningocele and 2 myelomeningoceles).
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ranking = 0.0081796514192197
keywords = nerve
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