Cases reported "Disease Progression"

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1/71. Facial neuromas in children: delayed or immediate surgery?

    OBJECTIVE: The objective of this study was to investigate the clinical characteristics and outcome of facial nerve neuromas in children. To date, no specific study has focused on children, and the management of these tumors is not codified. DESIGN AND SETTINGS: A review of case series treated in a tertiary care center of pediatric otolaryngology. SUBJECTS: The treatment and outcomes for 7 children (4 infants and 3 adolescents) were analyzed. RESULTS: Six patients underwent complete removal of tumor and immediate restoration of the nerve continuity. The grade of facial palsy improved in 4 of the 6 children, but did not get better than grade 3 (House classification). The remaining patient was managed conservatively and remained stable clinically and radiologically after 9 years follow-up. CONCLUSIONS: These findings support the reasonable strategy of combining conservative assessment of these slow-growing tumors with regular clinical and radiologic evaluations and radical surgery using various procedures. The choice depends on the age of the child, the extent and growth rate of the tumor, the grade of facial palsy, and the hearing function.
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ranking = 1
keywords = palsy
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2/71. White matter hyperintensities on MRI in a patient with corticobasal degeneration.

    We describe a patient who presented with the clinicopathological features of corticobasal degeneration (CBD). Over the course of 8 years, the patient developed myoclonus, dystonia, and supranuclear gaze palsy associated with an akinetic-rigid syndrome. To our knowledge, no previous report of a patient with CBD has described clear-cut regional white matter changes as revealed by magnetic resonance imaging (MRI) scans. In our patient, a T2-weighted MR image of the brain showed focal atrophy of the bilateral frontal cortex and asymmetric regional hyperintensities of the subjacent white matter. These signal changes seemed to primarily reflect the progression of neuronal degeneration, especially the demyelination secondary to axonal loss or change.
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ranking = 426.24619392237
keywords = supranuclear, palsy
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3/71. Borderline malignant change in recurrent mullerian papilloma of the vagina.

    Malignant change occurred in a benign, recurrent vaginal mullerian polyp. The patient, a 49 year old woman with cerebral palsy, presented with a polypoid mass in the vagina. At four years of age she had presented with a haemorrhagic polyp, and over the following years she had recurrent irregular bleeding and regrowth of the polypoidal mass, requiring a total of 10 operations to excise the polyp. Histological examination of the specimen showed typical mullerian features with tubal, endometrioid, and endocervical cell types. There were significantly abnormal nuclei, indicating low grade or borderline malignancy. review of previous biopsies showed similar mullerian features but no atypia. This is the first reported case of borderline malignant change in a previously benign recurrent mullerian papilloma of the vagina. Definitive radical surgery or radiotherapy is contraindicated in this patient and she remains under follow up.
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ranking = 0.5
keywords = palsy
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4/71. Primary brainstem injury: benign course and improved survival.

    Primary brainstem injury following head injury is a rare event. The victims often have features of supratentorial injury, and a primary isolated injury to the brainstem occurring due to shearing stresses or to injury from the tentorial edge is extremely rare. In the presence of supratentorial injury, these patients may have altered sensorium. Isolated brainstem injury may manifest itself as internuclear ophthalmoplegia, anisocoria, rigidity and cerebellar tremor. Such injuries are now being diagnosed more often due to improved imaging techniques. We treated nine such cases who had sustained primary brainstem injury in road traffic accidents, all but one of whom were subsequently independent. Primary brainstem injuries need not be associated with poor prognosis and mortality and may run a benign course with good quality of survival.
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ranking = 40.882991810034
keywords = ophthalmoplegia
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5/71. Spinal deformity after selective dorsal rhizotomy in patients with cerebral palsy.

    Selective dorsal rhizotomy is used widely as a means of treating spasticity associated with cerebral palsy. Little is known regarding the effect of the procedure on the development or progression of spinal deformity. The authors reviewed six patients with progressive deformity after rhizotomy. Prerhizotomy and postrhizotomy records of physical examinations and radiographs were reviewed retrospectively in an attempt to identify risk factors for development of and/or rapid progression of, spinal deformity. Detailed preoperative and postoperative evaluation of spinal alignment should be undertaken, particularly in those patients who may be at risk of rapidly progressive deformity.
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ranking = 2.5
keywords = palsy
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6/71. Palatal tremor, progressive multiple cranial nerve palsies, and cerebellar ataxia: a case report and review of literature of palatal tremors in neurodegenerative disease.

    We describe a patient with an unusual clinical presentation of progressive multiple cranial nerve palsies, cerebellar ataxia, and palatal tremor (PT) resulting from an unknown etiology. magnetic resonance imaging showed evidence of hypertrophy of the inferior olivary nuclei, brain stem atrophy, and marked cerebellar atrophy. This combination of progressive multiple cranial nerve palsies, cerebellar ataxia, and PT has never been reported in the literature. We have also reviewed the literature of PT secondary to neurodegenerative causes. In a total of 23 patients, the common causes are sporadic olivopontocerebellar atrophy (OPCA; 22%), Alexander's disease (22%), unknown etiology (43.4%), and occasionally progressive supranuclear palsy (4.3%) and spinocerebellar degeneration (4.3%). Most patients present with progressive cerebellar ataxia and approximately two thirds of them have rhythmic tremors elsewhere. Ear clicks are observed in 13% and evidence of hypertrophy of the inferior olivary nucleus in 25% of the patients. The common neurodegenerative causes of PT are OPCA/multiple system atrophy, Alexander's disease, and, in most of them, the result of an unknown cause.
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ranking = 1361.1053743479
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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7/71. Progressive visual loss and ophthalmoplegia.

    A 51-year-old woman with hyperthyroidism presented with a 4-week history of bilateral progressive visual loss despite treatment with oral prednisone. Her visual function improved after bilateral orbital decompression. The indications for and advantages and disadvantages of radiation therapy and orbital decompression in TAO are discussed. The management of intraocular pressure, strabismus, and lid abnormalities in TAO is also addressed.
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ranking = 163.53196724014
keywords = ophthalmoplegia
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8/71. Malignant pilocytic astrocytoma in the medulla oblongata: case report.

    A 27-year-old woman visited our hospital with chief complaints of abducens nerve palsy and cerebellar symptoms. On computerized tomographic scanning and magnetic resonance imaging, a tumor with strong enhancement was found on the dorsal side of the medulla oblongata. A tumor was excised by suboccipital craniotomy and C1 laminectomy. Histologically, many Rosenthal fibers together with pilocytic tumor cells were found in some regions, but a very high Ki-67 labeling rate accompanied by cells with nuclei of irregular size and giant cells was observed in other regions. The tumor was diagnosed as malignant pilocytic astrocytoma originating from pilocytic astrocytoma by transformation. The biological behavior of pilocytic astrocytoma is obscure in several respects. We report our experience of a case of malignant pilocytic astrocytoma that developed in the brain stem and progressed extremely rapidly.
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ranking = 0.5
keywords = palsy
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9/71. lyme neuroborreliosis mimics stroke: a case report.

    lyme neuroborreliosis is diagnostically challenging because of its diverse manifestations. The well-documented neurologic spectrum includes lymphocytic meningitis, cranial neuropathy, and radiculoneuritis in the early disseminated stage; and peripheral neuropathy, chronic encephalomyelitis, and mild encephalopathy in the late persistent stage. This case report describes a 74-year-old man who developed progressive left hemiparesis and facial palsy. The patient was hospitalized to rule out a cerebral vascular accident. The diagnosis of Lyme borreliosis was established with serologic studies. The patient was treated with intravenous ceftriaxone and responded with rapid clinical and functional recovery. lyme neuroborreliosis presenting as hemiparesis has rarely been reported. Prompt diagnosis and treatment appear to facilitate symptomatic relief and prevent persistent neurologic deficits.
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ranking = 0.5
keywords = palsy
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10/71. Minicore myopathy in children: a clinical and histopathological study of 19 cases.

    Minicore myopathy is a congenital myopathy characterized by multifocal areas of degeneration in muscle fibres. genetic heterogeneity expected on the basis of clinical variability awaits further resolution. We reviewed 19 cases in order to further delineate the phenotype. Marked hypotonia was the predominant presenting feature, with evidence of antenatal onset in 30% of cases. Weakness was most pronounced axially and proximally, often more severely affecting the shoulder girdle. Mild facial involvement was frequent. Varying degrees of scoliosis were obvious in all patients older than 10 years. In addition, two patients who were also the most severely affected had complete external ophthalmoplegia. One patient showed marked distal involvement. Respiratory failure developed in half of all patients after 10 years of age and correlated strongly with the degree of scoliosis. Cardiac involvement occurred mainly secondary to respiratory impairment. The course appeared static in most cases. Loss of independent walking was observed only in one case at the age of 10 years. On ultrasound scan, differential involvement within the quadriceps was documented in several patients. Variability in fibre size, type 1 predominance and atrophy with occasional type 2 hypertrophy were prominent but nonspecific histological changes. Apart from typical minicores, a marked increase in internal nuclei was the most prominent histological feature. With the exception of one family in which two generations were affected, inheritance appeared autosomal-recessive or sporadic in all cases.
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ranking = 40.882991810034
keywords = ophthalmoplegia
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