1/13. Conservative facial nerve management in jugular foramen schwannomas.OBJECTIVE: Although transposition of the facial nerve is crucial in infiltrative vascular lesions involving the jugular foramen, the objective was to show that a conservative approach to management of the facial nerve is sufficient with jugular foramen neuromas because of their noninfiltrative, less vascular nature and medial location in the jugular foramen. STUDY DESIGN: Retrospective case review. SETTING: Tertiary, private, multiphysician, otologic practice. patients: Sixteen patients with jugular foramen schwannoma (18 procedures) treated between January 1975 and October 1995. The 8 male and 8 female patients ranged in age from 13 to 66 years (mean age 47.7 years). INTERVENTION: One-stage, total jugular foramen neuroma removal without transposition of the facial nerve, using a variety of surgical approaches. MAIN OUTCOME MEASURES: facial nerve transposition (yes or no), House-Brackmann facial nerve grade, lower cranial nerve status, complications. RESULTS: One-stage total tumor removal was accomplished in all the cases. In 13 (72%) of the neuromas, removal was accomplished without facial nerve transposition. Transposition was performed in 2 revision cases in which scar tissue from a previous operation prevented complete control of the carotid artery and safe removal, 2 cases with large tumor extension anteriorly to the petrous apex, and 1 case with extensive involvement of the middle ear. A House-Brackmann facial nerve Grade I or II was obtained in 16 of the 18 procedures, with 1 Grade III and 1 case that remained Grade V, as it was preoperatively. CONCLUSIONS: One-stage, total tumor removal can be achieved with excellent control of the important vascular structures and without transposition of the facial nerve in a majority of jugular foramen schwannomas.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/13. Benign paroxysmal tonic upgaze of childhood with ataxia.Paroxysmal tonic upgaze deviation (PTU) is a rare neuro-ophthalmological disorder with onset in infancy or early childhood. It consists of episodes of sustained, conjugate, upward deviation of the eyes, down beating saccades in attempted downgaze, apparently preserved horizontal eye movements, frequent association with mild ataxia or clumsiness at time of illness, normal metabolic, electroencephalographic and neuroradiological findings. Symptoms are frequently relieved by sleep and can be exacerbated by fatigue or illness. Although PTU generally tends to disappear spontaneously within a few months or years, subsequent case reports have demonstrated the heterogeneous nature of the syndrome with respect to outcome. To date, the pathogenesis of the condition is still unknown. We present a new case of PTU with ataxia occurring in an otherwise healthy child, as documented by video recording of the phenomenon.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/13. Episodic coma in a new leukodystrophy.Among the leukodystrophies of a hypomyelinating nature, childhood ataxia with diffuse central nervous system hypomyelination exhibits the unique feature of rapid decrease in mental status after relatively minor head injuries or otherwise noncomplicated febrile illnesses. This article reports the case of a child with progressive spastic quadriparesis in whom unconsciousness developed repeatedly as a result of minor head trauma and required prolonged critical-care nursing. Although cognition is believed to be relatively preserved in this disorder, this child developed progressive cognitive decline. A detailed review of the literature is presented along with discussion of the potential mechanisms of neurologic deterioration.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/13. Progressive myoclonic ataxia and jc virus encephalitis in an AIDS patient.A case of progressive myoclonic ataxia in an AIDS patient is described, which evolved over a 13 month period. The ataxia persisted as the only clinical finding for several months before the appearance of a severe tetraparesis and cachexia. Throughout the clinical progression, magnetic resonance imaging (MRI) revealed the presence of bilateral, progressive, isolated, and symmetrical lesions involving the red nuclei, subthalami, thalami, lenticular nuclei, and primary motor cortices. Neuropathological examination, supplemented by in situ hybridisation for jc virus dna, confirmed that the lesions were those of progressive multifocal leucoencephalopathy (PML). The exceptional clinical presentation of PML in this case is the first report of progressive myoclonic ataxia caused by PML. The selective nature of the lesions confirms the role of the dentato-rubral-thalamo-cortical tract in the pathogenesis of progressive myoclonic ataxia. The atypical MRI findings further emphasise the need for expanded diagnostic criteria for PML in AIDS patients and support the use of more aggressive diagnostic methods as new treatments become available.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/13. Diagnostic challenges in Creutzfeldt-Jakob disease: case report.Sporadic Creutzfeldt-Jakob disease (CJD) is the most common prion disease. The diagnosis can be confirmed only by histological examination of brain tissue obtained at biopsy or at autopsy. Because of the transmissible nature of the disease, autopsy or brain biopsy cannot be performed at many institutions, which poses numerous challenges in confirming the diagnosis. We report the case of a patient with CJD in which autopsy to confirm the diagnosis was performed after overcoming numerous obstacles and advocating with hospital leadership. This case illustrates the numerous challenges that exist in achieving a definitive diagnosis of CJD and in postmortem disposition of the body, and we provide recommendations to clinicians who face similar challenges.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/13. Petroclival mixed meningioma.We present a 47-year-old lady with a large dumb-bell petroclival-cavernous sinus meningioma. The tumour was of mixed histopathology; the anterior part, in relationship to the cavernous sinus, was a syncitial meningioma and the posterior part in the petroclival region was a transitional meningioma. The two histological subtypes of meningioma within the same tumour displayed remarkable differences in their radiological features, nature of extensions, relationship with the adjoining structures and consistency. The clinical course of the two subtypes was also different. The unusual features of this case are reported and the relevant literature briefly reviewed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/13. Claude's syndrome associated with supranuclear horizontal gaze palsy caused by dorsomedial midbrain infarction.Claude's syndrome caused by dorsal midbrain lesion is characterized by ipsilateral third nerve palsy and contralateral ataxia. To date, reports in the literature concerning Claude's syndrome associated with the midbrain paresis of horizontal gaze are rare. A 62-year-old man suddenly developed left third cranial nerve palsy, right lateral gaze palsy, and right ataxia. Intact Bell's phenomenon and preserved right horizontal oculocephalic reflex suggested the lateral gaze palsy in the right eye was supranuclear in nature. magnetic resonance imaging (MRI) revealed an infarction in the left dorsomedial midbrain. Although the red nucleus has often been suggested as the lesion site responsible for Claude's syndrome, a lesion of the superior cerebellar peduncle just below and medial to the red nucleus could be responsible for this syndrome. This case demonstrates neurological heterogeneity of midbrain infarction.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/13. Two cases of variant Creutzfeldt-Jakob disease (vCJD) referred to the Department of Community mental health, Aldershot Garrison in 2003.In the year 2003 the Department of Community mental health (DCMH) at Aldershot Garrison received referrals of two soldiers, a sergeant and a lance corporal, who presented with a complex picture of neurological and psychiatric symptoms. Both had been investigated very thoroughly by neurologists who, owing to the mainly negative results of their investigations, were unable to make a diagnosis. Of the two patients one had also been assessed as a psychiatric in-patient in a civilian hospital and had been referred to the Aldershot DCMH for continuing care. The other had been transferred, after investigations, to the Defence Services Medical rehabilitation Centre (DSMRC) at Headley Court but, failing to make progress, was also referred for psychiatric assessment. Both patients were obviously unwell but the nature of their illnesses remained obscure. Within a few months both had died and the diagnosis of one of the human transmissible spongiform encaphalopathies (TSEs), vCJD, was made at autopsy in one of them, but both were registered by the National CJD Surveillance Unit (3) with a diagnosis of vCJD. The circumstances were so unusual and prompted the writing of this paper by one of the psychiatrists involved.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/13. Massive breast enlargement in an infant girl with central nervous system dysfunction.A 6-month-old female is described who presented with severe idiopathic macromastia. The breast enlargement began at 2 months of age and progressed such that subtotal mastectomies were necessary at 23 months. Extensive hormonal evaluation prior to surgery revealed no evidence of estrogenization or precocious puberty. There was no galactorrhea. A breast biopsy showed immature mammary tissue. in vitro analysis of the patient's serum using a mouse mammary thymidine incorporation assay revealed similar mitogenic activity in the patient's serum compared to adult controls. Post surgical follow up of this patient, 3.5 years later, has revealed no breast enlargement, precocious sexual development, or growth acceleration. Of interest, however, she has manifested an idiopathic degenerative neurologic condition characterized by psychomotor delay, ataxia, and seizures. Remarkably, hormone studies at age 5.5 years showed an exaggerated gonadotrophin response to intravenous gonadotrophin releasing hormone and prepubertal estrogen levels. While this case may represent an extraordinary example of idiopathic premature thelarche, the severe nature of this infant's macromastia in association with neurologic dysfunction and increased gonadotrophins suggests that central nervous system factors were etiologic.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/13. Deteriorating epilepsies: severely deteriorated cases.A total of 28 cases of severely deteriorated epileptic patients were seen at out- and inpatient services in the past 12 years. In 22 out of these 28 cases, the etiology for the deterioration was considered to be due to either repetitive intractable seizures or antiepileptic drugs (AEDs) or both. Although differential diagnoses were difficult in many cases as to the responsible causes, namely seizure vs. AEDs, it was considered in 6 cases that AEDs took a major role in their deterioration. Details of such cases are presented. Special emphasis was made to the fact in which they frequently showed episodes of acute or ataxia and confusion often associated with febrile illness. They took a course of acute or subacute exacerbation and partial remission. Discussion was held on the nature and possible avoidance of these deteriorations.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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