1/55. Neuro-ophthalmologic manifestations of Maffucci's syndrome and Ollier's disease.patients with Ollier's disease (multiple skeletal enchondromas) and Maffucci's syndrome (multiple enchondromas associated with subcutaneous hemangiomas) may develop skull base chondrosarcomas or low-grade astrocytomas as a delayed consequence of these disorders. We report three patients with Ollier's disease and Maffucci's syndrome who had diplopia as the initial manifestation of intracranial tumors. Since patients with Maffucci's syndrome and Ollier's disease are at risk for the delayed development of brain and systemic neoplasms, neuroophthalmologists must be aware of the need for long-term surveillance in patients affected by these conditions.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/55. Bilateral phrenic neuropathy as a presenting feature of multifocal motor neuropathy with conduction block.Diaphragmatic paralysis has previously been reported as a result of diverse pathologic processes involving the peripheral nervous system. We report the clinical history, physical findings, and antibody profile of an atypical case of multifocal motor neuropathy with conduction block initially presenting with respiratory failure secondary to bilateral phrenic neuropathy.- - - - - - - - - - ranking = 41.302524495637keywords = nervous system (Clic here for more details about this article) |
3/55. Neoplasm as a cause of brachial plexus palsy in neonates.Two patients with neonatal onset of arm weakness resulting from neoplastic involvement of the brachial plexus who were initially considered to have obstetric brachial plexus palsies are reported. The first patient was a 7-day-old female who presented with a left supraclavicular mass that was first detected at 2 days of age and left proximal arm weakness. The weakness involved the whole arm within 3 days. The mass was a malignant rhabdoid tumor. The second patient was a 28-month-old male who presented with slowly progressive right arm weakness, which began at 3 weeks of age, and episodes of scratch marks on the arm that began at 4 months of age. magnetic resonance imaging revealed a plexiform neurofibroma of the brachial plexus. The features that are suggestive of a brachial plexus palsy caused by a neoplasm rather than of obstetric brachial plexus palsy include the following: the onset of weakness after the first day of age, with a progressive course; a history of a normal delivery and birth weight; the absence of signs of a traumatic injury or injuries; the appearance before 7 days of age of a growing supraclavicular mass without radiographic evidence of a clavicular fracture; and recurrent scratch marks on the weak arm.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/55. Asymmetric sweating in a child with multiple sclerosis.A 10-year-old male with multiple sclerosis complained of excessive sweating on the right side of the forehead and shoulder on relapse 3 months after the onset of multiple sclerosis. Because the neurologic evaluation revealed no abnormalities in the sudomotor function, it is likely that the hyperhidrosis resulted from a lesion in the central or preganglionic sympathetic nervous system. magnetic resonance imaging demonstrated a high-intensity lesion involving the left hypothalamus on T(2)-weighted imaging. Thus hypothalamic involvement might be the reason for the hyperhidrosis in this patient.- - - - - - - - - - ranking = 41.302524495637keywords = nervous system (Clic here for more details about this article) |
5/55. A local outbreak of paralytic rabies in Surinam children.A rapidly fatal encephalomyelitis, which was in most cases characterized by ascending paralysis, developed in seven children of the age of 3 to 10 years in a bushnegro village in the interior of Surinam. rabies virus was recovered from the central nervous system of three autopsied children. Although the source of infection has not been detected, there is an indication that, at least in some cases, the disease has been transmitted by rat-bite rather than by vampire bats. During the same period a few cases of minor febrile illness occurred in the same community. Since virological and serological evidence of a wide-spread distribution of Coxsackie A virus type 4 was obtained, the latter illness may presumably be attributed to this virus.- - - - - - - - - - ranking = 97.811926513525keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/55. Neuroinvasion during delayed primary HHV-7 infection in an immunocompetent adult with encephalitis and flaccid paralysis.Antibody avidity tests have been used to detect primary human herpesvirus-7 (HHV-7) infection in an immunocompetent 19-year-old man with encephalitis and flaccid paralysis for which all other suspected causes had been excluded. The finding of the viral dna in the cerebrospinal fluid (CSF) but not in serum samples suggests that primary HHV-7 infection with invasion of the central nervous system and consequential disease had occurred. As almost all adults are infected with HHV-7 in early childhood, the present case of delayed primary infection with serious symptoms must be exceptionally rare and no cases of such late acquisition of the virus have been documented in the literature. This report of HHV-7 dna in the CSF of an immunocompetent adult is also unique.- - - - - - - - - - ranking = 97.811926513525keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/55. Bilateral sixth nerve palsy after head trauma.Gaze deficits are not uncommon after head trauma and might be caused by injury to the central nervous system, the peripheral nerve, or the motor unit. Traumatic bilateral sixth cranial nerve palsies are a rare condition and are typically associated with additional intracranial, skull, and cervical spine injuries. We describe a case of a complete bilateral sixth nerve palsy in a 44-year-old male patient with trauma with no intracranial lesion, no associated skull or cervical spine fracture, and no altered level of consciousness. The emergency physician should be aware of the differential diagnosis, initial workup, and injuries associated with a traumatic gaze deficit.- - - - - - - - - - ranking = 97.811926513525keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/55. False diagnosis caused by Warthin tumor of the parotid gland combined with actinomycosis.A case is reported in which a unilateral parotid gland cystadenolymphoma was combined with actinomycosis. A 48-year-old woman presented with a mass in the left parotid region and paresis of the lower left palpebra. The computed tomography, echography, and parotid radiographic findings did not exclude a neoplasm of the left parotid gland. The ramus of the mandible was involved in the process. Intraoperative freezing histology, total parotidectomy, and partial mandibulectomy were performed, with sacrifice of the facial nerve followed by nerve reconstruction. The final histological evaluation was Warthin tumor with actinomycosis. Four years after treatment, the patient is free of disease. No similar cases seem to have been reported thus far.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/55. A child with neurobrucellosis.An 11-year-old boy presented with chronic meningitis followed by acute flaccid paralysis. The aetiology remained uncertain until the brucellar serology test became positive and there was a good response to specific antimicrobial therapy. Nerve conduction studies confirmed a proximal radiculopathy. awareness of the condition and performance of the appropriate tests will differentiate neurobrucellosis from other chronic central nervous system infections.- - - - - - - - - - ranking = 97.811926513525keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/55. Does parkin play a role in the peripheral nervous system? A family report.Two genes were identified for autosomal recessive forms of early onset Parkinson's disease: parkin and DJ-1. We describe 2 siblings with EOPD due to parkin mutations and peripheral neuropathy, which presented as neuropathy with liability to pressure palsies (HNPP) in the index case. RT-PCR experiments revealed that the parkin gene is expressed in sural nerves from both controls and patient with parkin-related disease. Our findings support the view that parkin may play a role in the peripheral nervous system.- - - - - - - - - - ranking = 206.51262247819keywords = nervous system (Clic here for more details about this article) |
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