Cases reported "Reflex, Abnormal"

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1/107. Cervical foraminotomy: an effective treatment for cervical spondylotic radiculopathy.

    Between 1983 and 1994, posterior cervical foraminotomy as described by Frykholm was performed on 89 patients with exclusively radicular symptoms caused by cervical osteophytes. The main presenting feature was arm pain. Objective neurological signs were present in 50% of the patients. At mean postoperative follow-up of 8.6 months, 95.5% of patients reported excellent or good results, while 4.5% were not improved. No patient was rendered worse following the procedure. There were no deaths and the complication rate was 2.2%. Further surgery for recurrent root symptoms was required by 6.7% of patients. Our findings are in keeping with the good results and low complication rate of this procedure as described in other studies. Informal inquiries suggest that this procedure is not widely used, at any rate in the United Kingdom, and we present this series in order to emphasize the efficacy and safety of this procedure.
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ranking = 1
keywords = neurologic
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2/107. Lessons to be learned: a case study approach. Primary hyperparathyroidism simulating an acute severe polyneuritis.

    The case is presented of a 65 year old lady with recent onset of neuromuscular manifestations, comprising paraparesis, areflexia and unsteady gait, along with episodes of slurring of speech and diplopia, later confirmed to be due to severe hypercalcaemia--which itself was caused by primary hyperparathyroidism. Restoration of normocalcaemia, by means of rehydration and bisphosphonate therapy, resulted in clinical improvement--whilst subsequent parathyroidectomy was followed by complete resolution of all symptoms. In order to make prompt differentiation between the neurological sequelae of hyperparathyroidism and a primary neurological disorder, a high index of suspicion is required. An urgent serum calcium assay, as part of a bone profile, is mandatory in patients who present with neurological symptoms--especially the elderly, amongst whom hyperparathyroidism is especially common.
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ranking = 3
keywords = neurologic
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3/107. Neurological deficit following spinal anaesthesia: MRI and CT evidence of spinal cord gas embolism.

    A 62-year-old diabetic woman developed permanent neurological deficits in the legs following spinal anaesthesia. MRI showed oedema in the spinal cord and a small intramedullary focus of signal void at the T10 level, with negative density at CT. Intramedullary gas bubbles have not been reported previously among the possible neurological complications of spinal anaesthesia; a combined ischaemic/embolic mechanism is hypothesised.
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ranking = 2
keywords = neurologic
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4/107. Spinal angiolipoma: case report and review of literature.

    Spinal extradural angiolipomas are distinct, benign, and rare lesions composed of mature lipocytes admixed with abnormal blood vessels. They account for 0.14% of all spinal axis tumors. The case described here was a 72-year-old patient presenting with a history of paraparesis, hypoesthesia under the T2 level, hyperreflexia, and urinary overflow incontinence that appeared within 7 days after the administration of a coronary vasodilator drug regimen. The spinal magnetic resonance scan showed a lipomatous mass with signal void lesions, suggesting a vascular component of the tumor. The patient improved rapidly after surgical resection of the epidural tumor and decompression of the cord. According to the present literature, the duration of neurological symptoms ranges from 1 to 180 months (mean 28 months). But this patient's neurological deterioration took place 4 days before hospitalization. We believe that this can be explained by the increased tumor blood volume caused by vasodilator drugs, which in turn exerted a pulsatile compressive effect on the cord.
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ranking = 2
keywords = neurologic
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5/107. Hyperekplexia phenotype due to compound heterozygosity for GLRA1 gene mutations.

    Hyperekplexia (MIM 149400), or startle disease, is a neurological disorder characterized by generalized stiffness during the neonatal period, excessive startle reflexes, and generalized stiffness related to the startle response. Linkage analysis mapped a major gene for this disorder to chromosome 5q33-35. Subsequently, mutations in the GLRA1 gene, encoding the alpha1 subunit of the glycine receptor, were found in hyperekplexia families with an autosomal dominant or recessive inheritance pattern. In the present study, we describe the genetic analysis of the GLRA1 gene of a family consisting of 2 children with hyperekplexia, 2 nonaffected children, and their healthy nonconsanguineous parents. Although the pedigree suggested the presence of a recessive mutation, haplotype construction showed that the 2 affected children shared the same haplotype combination in which the maternal haplotype differed from the paternal haplotype, suggesting the presence of compound heterozygosity. mutation analysis revealed different missense mutations on the two haplotypes, changing an arginine to a histidine at amino acid positions 252 and 392, respectively. It is interesting that the hyperekplexia phenotype was only seen in individuals compound heterozygous for the two mutations, whereas family members carrying either one of the two mutations had no clinical signs.
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ranking = 1
keywords = neurologic
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6/107. Hypertonia, hyperreflexia, and excessive startle response in a neonate.

    Following an uneventful gestation, a newborn girl presented with hypertonia, hyperreflexia, tremor, and excessive startle response. nose tap elicited a dramatic head recoil. Her mother had similar symptoms beginning as a child that improved but persisted into adulthood. In addition, several members of mother's family died unexpectedly in infancy. Hypertonia in the newborn period indicates central nervous system dysfunction of several possible causes, most of which are associated with severe cognitive deficits and limited neurological development.
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ranking = 5.0874566548819
keywords = nervous system, neurologic
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7/107. 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.
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ranking = 0.012916136609524
keywords = toxicity
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8/107. Friedreich's ataxia presenting as adult-onset spastic paraparesis.

    We have studied a man with an atypical form of Friedreich's ataxia (FRDA), who presented at age 26 years with a 2-year history of unsteadiness and clumsiness. The predominant feature of his initial neurological examination was a spastic paraparesis, along with a mild distal weakness and hyperreflexia of the upper limbs. He also displayed limb ataxia. Frataxin GAA repeat sizes were 1,040/690. This unusual FRDA presentation is not dissimilar to that of Acadian spastic ataxia.
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ranking = 1
keywords = neurologic
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9/107. The significance of a crossed extensor hallucis response in neurologic disorders: a comparison with the Babinski sign.

    The significance of a crossed extensor hallucis response on active flexion of the hip was analysed in various neurological disorders. It is concluded that this sign, not formerly described, is a pathological reflex or synkinesia. In the cooperative patient the crossed extensor response is a more sensitive indicator of a minor disturbance within the cortico-spinal motor pathways than the Babinski sign. With lesions above the foramen magnum a crossed extensor hallucis response is observed more frequently than the Babinski sign.
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ranking = 5
keywords = neurologic
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10/107. Spasticity due to phenytoin toxicity.

    A young epileptic presented with spasticity as well as ataxia, diplopia and nystagmus; his serum phenytoin level was very high. All the abnormal signs disappeared after withdrawal of phenytoin. Spasticity, hyperreflexia, and clonus are features of phenytoin intoxication, present in this case, which are not commonly seen, and which have rarely been mentioned previously in the literature.
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ranking = 0.051664546438096
keywords = toxicity
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