1/10. Cranial polyneuropathies in multiple sclerosis: case report and literature review.Although cranial neuropathies are common disorders in multiple sclerosis patients, multiple cranial nerve involvement is an unusual occurrence. Correlation of clinical symptoms with magnetic resonance imaging evidence of demyelinating central nervous system lesions can confirm the diagnosis. The authors report on the case of a 43-year-old woman who initially was thought to have suffered a brainstem infarct but, in fact, had developed multiple cranial nerve functional deficits. Treatment of multiple sclerosis remains primarily supportive in nature, with corticosteroids used for acute exacerbations and chronic progression.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/10. 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) |
3/10. tongue piercing. The new "rusty nail"?BACKGROUND: Cephalic tetanus is a rare form of the tetanus caused primarily by wounds or other infectious processes involving the head and neck. This condition frequently progresses to the generalized form of tetanus with the attendant risks and complications. methods: A case report of a young female who developed an unusual form of tetanus after a tongue piercing is presented here. We discuss this disorder as it applies to the contemporary caregiver with a focus on its presentation and recognition. RESULTS: A delay in diagnosis of 13 days from presentation occurred. The patient had a slow, uneventful but incomplete recovery course. She never developed significant airway compromise, nor did she demonstrate any evidence of hemodynamic instability but continued to have right facial weakness up to 6 months after discharge. CONCLUSIONS: A few factors were identified that contributed to the significant delay in diagnosis. The unusual nature of the disease and a lowered index of suspicion on the part of the initial caregivers were probably the major causes. Fortunately, no major adverse sequelae resulted from the delay. However, if this case heralds the onset of a rise in the incidence of tetanus, early recognition and diagnosis would seem essential to avoid much of the morbidity and mortality associated with the disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/10. Mononeuritis multiplex in Waldenstrom's macroglobulinemia.We describe a patient with mononeuritis multiplex who had cranial nerve involvement in association with Waldenstrom's macroglobulinemia. sural nerve biopsy showed abundant endoneurial granulofibrillary deposits related to microangiopathic changes. These changes were distributed in a multifocal pattern among the fascicles. immunohistochemistry demonstrated the IgM nature of the deposit located in the endoneurial interstitium.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/10. Isolated sixth nerve palsy due to intracavernous carotid aneurysm in a young woman.A large intracavernous carotid aneurysm was angiographically demonstrated in a 29 years old woman suffering from a persistent isolated abducens nerve palsy. CT brain scan failed to reveal the lesion. The usefulness of performing angiography in the cases of isolated abducens palsy of undetermined nature, is stressed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/10. Unusual vascular events in the territory of the posterior cerebral artery.There is an unusual type of vascular episode in the territory of the posterior cerebral artery which remains relatively unknown. Ten cases are presented in which a posterior cerebral artery deficit developed suddenly in dramatic fashion with headache, visual symptoms, sensory and motor deficits and signs of third nerve involvement. Nine of the patients were female and one was male. Seven were under the age of 33. In all instances there was a permanent neurologic sequela, usually a hemianopia. A similar case was described in 1901. The nature of the underlying process remains obscure, but the evidence favors accompanied migraine in which a particularly severe attack results in permanent damage. The term "catastrophic migraine" is suggested.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/10. Familial congenital fourth cranial nerve palsy.Few reports of hereditary transmission patterns in congenital superior oblique palsy have been made in the past. In this study, three families with congenital superior oblique palsies have been identified and examined. Two members of each family had a superior oblique palsy. After full history, eye examination, and sensory testing, the patterns in each family have been analyzed. Deviations varied from binocular fusion and orthophoria to large vertical deviations, especially in adduction. Sensory patterns were compatible with the congenital nature of the palsy. A discussion of possible causes for a congenital superior oblique palsy is offered and the congenital superior oblique palsies are analyzed. A definite genetic transmission pattern could not be found, but possible explanations are considered. Regardless of etiology, therapy for this problem is unchanged from that for all superior oblique palsies. Occurrences of congenital trochlear palsies in multiple family members should be considered when a patient with this problem is diagnosed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/10. Congenital trigeminal anaesthesia. A review and classification.Forty-three patients with the syndrome of congenital trigeminal anaesthesia (CTA) have been reported to date. The cases have been divided into three groups based on the presence and nature of any associated clinical problems. Three additional cases are presented as characteristic of each of the groups. A different aetiology is proposed for each group. Group I consists of patients with CTA as an isolated finding. It is almost always bilateral, and typically involves the distribution of only the first division of the fifth nerve. There is no evidence of other neurological abnormalities or associated mesoectodermal congenital anomalies. The aetiology is suspected to be a primary neural hypoplasia. patients with associated congenital mesenchymal anomalies were placed in the second group. CTA was often a minor part of another well defined, more extensive clinical syndrome, such as Mobius or oculoauriculovertebral dysplasia (OAVD). The sensory abnormality was either unilateral or bilateral. The skin of the face was almost always involved, as well as the cornea and conjunctiva. This may be a heterogenous group with multiple aetiologies having in common an injury early in embryogenesis. The third group is defined as patients with CTA without evidence of mesenchymal dysplasia. These cases differ from the first group primarily because of the presence of associated focal brainstem signs. The aetiology is thought to be due to focal neural dysgenesis secondary to a prenatal injury, possibly vascular in nature.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
9/10. vestibular nerve pathology in cases of intractable vertigo: an electronmicroscopic study.OBJECTIVE: This study aimed to determine the absence or presence and the nature of pathology of the vestibular nerve in case of intractable vertigo. STUDY DESIGN: This was a prospective study. SETTING: The study was performed at a private practice tertiary referral center. patients: There-were 42 patients with intractable vertigo in the study. INTERVENTIONS: All patients received thorough diagnostic examinations and surgical excision of the vestibular nerves. MAIN OUTCOME MEASURES: Segments of the superior and inferior vestibular nerves were surgically removed, preserved in glutaraldehyde, examined by electronmicroscopy, and the findings were correlated with the clinical diagnosis. RESULTS: A variety of different types of pathologic lesions were identified, including axon and supporting cell degeneration, herpes zoster virus, other viruses, results of bacterial infection, and regrowth of nerve after surgical resection. CONCLUSION: The vestibular nerves were found to be histologically normal in lesions primarily involving the end organ such as most early Meniere's disease cases, benign paroxysmal postural vertigo (BPPV), and mild labyrinthine concussion. Vestibular nerve degeneration was seen with advanced Meniere's disease, severe labyrinthine concussion, and with vascular loops in the internal auditory canal. Herpes zoster involves Scarpa ganglion in herpes zoster oticus. viruses were found in the nuclei of vestibular nerve cells in a patient with delayed hydrops. Regrowth of the vestibular nerve after surgical resection was confirmed in three cases.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/10. Amyloidomas of the nervous system: a monoclonal B-cell disorder with monotypic amyloid light chain lambda amyloid production.BACKGROUND. Amyloidomas or localized tumor-like amyloid deposits rarely affect the nervous system. To the authors' knowledge, no comprehensive studies on central and peripheral nervous system amyloidomas have been published. The amyloid subtype of amyloidomas of the nervous system only recently was characterized and almost invariably was found to be of amyloid light chain (AL) lambda type. The nature of the plasma cell population responsible for AL amyloid production has not been investigated further. methods. The current analysis included the clinical findings, neuroimaging characteristics, and pathology of seven amyloidomas (four cerebral and three involving peripheral nerves). All were subjected to histochemical staining (congo red, thioflavine S) and to immunohistochemical study using primary antibodies detecting serum amyloid component P, serum amyloid protein A (SAA), transthyretin, beta2 microglobulin (beta2m), and free immunoglobulin (Ig) light chain. For the detection of mRNA of light chain Ig, fluorescein-conjugated kappa and lambda mRNA oligonucleotide probes were used. For the assessment of B-cell clonality, polymerase chain reaction (PCR) was applied on extracted dna from two cases using VH FRIII and JH primers. Two cases were assessed ultrastructurally. RESULTS. All amyloidomas were organ restricted and unrelated to systemic amyloidosis. The clinical symptoms of the cerebral lesions were nonspecific, whereas neurologic deficits were noted in the distribution of the involved peripheral nerves. Cerebral deposits, either solitary or multiple, were associated spatially with the choroid plexus and secondarily extended into white matter. All peripheral nerve amyloidomas involved the gasserian ganglion of the trigeminal nerve. Imaging by computed tomography and magnetic resonance imaging scans revealed hyperdense and contrast-enhancing mass lesions unassociated with significant edema. Immunohistochemically, the amyloid was present in the interstitium and within the walls of the intralesional vessels, was invariably of AL lambda subtype, and was negative for free Ig kappa light chains, SAA, transthyretin, and beta2m. plasma cells along the perivascular sheaths and occasionally squeezed between amyloid masses showed no cytologic atypia. in situ hybridization for Ig light chain mRNA reflected a massive preponderance of lambda-producing cells. PCR revealed monoclonal rearrangement of the heavy chain Ig gene. CONCLUSIONS. The results of the current study provide strong support for the concept that amyloidomas of the nervous system are neoplasms of an AL lambda-producing B-cell clone capable of terminal differentiation. Nevertheless, all seven patients lacked clinical evidence of an aggressive or systemic lymphoplasmacytic neoplasm. Unlike plasmacytomas, the relatively indolent course of most nervous system amyloidomas is reminiscent of the similarly indolent biologic behavior of extranodal, low grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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