21/551. Paradoxical lithium neurotoxicity: a report of five cases and a hypothesis about risk for neurotoxicity.There have been many reports of probable lithium-induced organic brain syndromes occurring when serum lithium levels are within or close to the therapeutic range. The authors report on five patients who developed clinical syndromes suggestive of severe neurotoxicity during lithium treatment. In all cases lithium levels were between .75 and 1.7 mEq/liter. The patients who developed neurotoxicity had markedly higher global ratings of psychotic symptomatology and anxiety in the pretoxic period than did patients who never deveoped neurotoxicity. When the acute manic state is characterized by marked psychotic symptoms and intense anxiety, it may be associated with increased vulnerability to the development of severe lithium neurotoxicity.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
22/551. Neurological accidents caused by repetitive breath-hold dives: two case reports.We report two Japanese male professional breath-hold divers (33 and 39 years of age) who experienced neurological disorders during repetitive dives to over 20 m of seawater. One patient had right homonymous hemianopsia, and the other presented with right hemiparesis with facial involvement and sensory deficit. In addition, they each had a history of neurological problems following such dives. Magnetic resonance images of their brains disclosed multiple T2-weighted hyperintensities corresponding to their neurological symptoms. Their brain lesions suggest a multiple cerebral infarction caused by occlusion of the cerebral arteries. We conclude that the repetitive deep breath-hold dives induced the brain involvement.- - - - - - - - - - ranking = 2keywords = ms (Clic here for more details about this article) |
23/551. Organic syndromes diagnosed as conversion disorder: identification and frequency in a study of 85 patients.BACKGROUND: The percentage of patients initially diagnosed with a conversion disorder and later identified as having an organic disorder has been decreasing in recent studies. METHOD: Consecutive patients with a diagnosis of conversion disorder were referred for psychiatric diagnosis and treatment. research questions were: (1) What incidence of neurological disorder is revealed by neurological reassessment and by which diagnostic technique is the final diagnosis established? (2) What differences can be observed between true-positive and the false-positive results? RESULTS: Ten (11.8%) of the 85 patients examined appeared to suffer from a neurological disorder. In this sample, variables discriminating between the true positives and false positives were: (1) prior suspicion of neurological disorder; (2) older age at referral; (3) older age at onset of symptoms; (4) longer duration of symptoms; and (5) use of medication. Three variables contributed significantly to the prediction of organic disorder: prior suspicion of neurological disorder; age at onset of symptoms; and duration of symptoms. CONCLUSIONS: Although our results are in line with those of other recent studies, the percentage of false positives was still high. The data further emphasize the dangers of making a diagnosis of conversion disorder in the absence of positive evidence. It is important to continue to provide follow up for patients with a diagnosis of conversion disorder. Unfortunately, unreliable psychiatric indications, like certain behavioral characteristics, are still used in the diagnostic process. The results show that a general neurological examination is still a valuable diagnostic instrument in addition to modern diagnostic techniques.- - - - - - - - - - ranking = 4keywords = ms (Clic here for more details about this article) |
24/551. XY sex reversal and a nonprogressive neurologic disorder: a new syndrome?We report a patient with a unique combination of clinical findings: XY sex reversal, spastic paraplegia, mental retardation, dysmorphism, and infantile-onset olivopontocerebellar hypoplasia. The phenotype of our patient did not coincide with any of the described forms of XY reversal syndromes, hereditary or sporadic spastic paraplegias, or congenital or infantile-onset cerebellar or olivopontocerebellar atrophies or hypoplasias. The disorder of this patient likely represents a genetic condition with pleiotropic effects on brain development and sex determination and adds further evidence for the heterogeneity of spastic paraplegia/infantile olivopontocerebellar hypoplasia syndromes and sex reversal syndromes.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
25/551. Herrmann multiple synostosis syndrome with neurological complications caused by spinal canal stenosis.A young man was found to have multiple synostosis syndrome type I after presenting with a neck injury causing a cervical spinal cord contusion. Neurological symptoms and signs suggested spinal cord compression. Magnetic resonance (MR) and computerized tomography (CT) imaging of the spine showed spinal canal stenosis with cord compression at C3-C6, a deformed spinal canal flattened in the anteroposterior dimension, vertebral fusions and deformed lateral processes of the vertebrae. He had a long broad nose with hypoplasia of the alae nasi, conductive hearing loss requiring hearing aids, muscular build, stiff spine, prominent acromia, pectus excavatum, ischial prominences, short fifth fingers, fusion at the proximal interphalangeal joints of the fifth fingers with indistinct overlying creases, and toe syndactyly. Spinal cord stenosis is a serious complication of multiple synostosis syndrome, that should be kept in mind in considering the risk of neck or back injury associated with certain sports or other activities. In both the multiple synostosis syndrome and the less severe proximal symphalangism deafness syndrome, mutations have been detected in the human homologue of the noggin gene on chromosome 17q21-q22.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
26/551. Neurologic and gastrointestinal dysfunction in cardio-facio-cutaneous syndrome: identification of a severe phenotype.Controversy exists over the distinction between cardio-facio-cutaneous (CFC) syndrome and noonan syndrome (NS). Several authors have suggested that they are different phenotypes of the same condition. We present the cases of two patients with CFC syndrome to show that it is a distinct condition with a unique combination of findings and a more complex natural history. These patients, both girls, were born with signs of fetal edema following pregnancies complicated by polyhydramnios. Each has short stature with relative macrocephaly; fuzzy, sparse hair; and the typical craniofacial features, including a square forehead. Both have heart abnormalities, failure to thrive, and severe feeding problems requiring gastrostomy. They are markedly hypotonic and developmentally delayed. They show signs of frequent eyelid fluttering and have oral aversion, tactile hypersensitivity, and sensory integration abnormalities. keratosis pilaris, the characteristic skin symptom, is also present in both patients. In a review we identified 56 cases of CFC syndrome. We scored these cases by 10 clinical criteria and identified a subset with a specific, severe phenotype distinct from that of NS. The serious neurologic and gastrointestinal complications, in addition to the skin abnormalities and characteristic facies in this group, clearly separate these patients from the mildly affected ones, most of whom appear to have NS or another syndrome. We discuss the differences between the severe CFC phenotype and those of overlapping conditions. We set forth stringent diagnostic criteria for CFC syndrome, the initial step toward identifying a molecular basis for this condition.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
27/551. angelman syndrome in three adult patients with atypical presentation and severe neurological complications.angelman syndrome (AS) is a distinct neurogenetic disorder and the phenotype is well known in childhood and adolescence. However, with advancing age the clinical and behavioral phenotype changes. In adulthood, the phenotype can be rather aspecific. We report on AS in 3 severely to profoundly mentally retarded patients, who developed severe neurologic complications of severe tremor, spasticity and coordination problems, resulting into severe loss of function. They presented atypical craniofacial features, short stature, epileptic seizures, microcephaly, brachytelephalangy and absent speech. Two patients presented at an older age a change in day-night rhythm. Based on this experience, we conclude that all severely to profoundly mentally retarded patients with atypical phenotype, spasticity, absent speech, epileptic seizures and changed day-night rhythm are candidates for further cytogenetic and molecular investigation for AS. Clinical photographs of the patient at a younger age can be helpful. The presence of the typical EEG pattern with frontal triphasic delta waves may direct to the diagnosis of AS.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
28/551. Subluxation of a lumbar vertebra in a patient with marfan syndrome. Case report.marfan syndrome is a hereditary disorder of the connective tissue that, in its most classic form, includes cardiovascular, ocular, and skeletal features. The neurological problems associated with the disease are mainly caused by intracranial vascular abnormalities and spinal meningeal defects, but other neurological manifestations are rarely present. scoliosis, a skeletal manifestation of the syndrome, occurs frequently, but its onset, natural history, and radiological characteristics differ from those of the idiopathic form. scoliosis in a patient with marfan syndrome seldom accompanies other spinal deformities. In this article the authors describe the case of a patient with marfan syndrome and scoliosis in whom lumbar subluxation occurred. This rare deformity, diagnosed on three-dimensional computerized tomography scanning, has not been reported previously in association with marfan syndrome. Its development can be explained in terms of the theory of progressive rotatory dislocation. The morphological characteristics, clinical features, and surgical treatment of the deformity are presented.- - - - - - - - - - ranking = 2keywords = ms (Clic here for more details about this article) |
29/551. Neurological symptoms in patients whose cerebrospinal fluid is culture- and/or polymerase chain reaction-positive for mycoplasma pneumoniae.We describe 13 patients with neurological signs and symptoms associated with mycoplasma pneumoniae infection. M. pneumoniae was isolated from the cerebrospinal fluid (CSF) of 9 patients: 5 with meningoencephalitis, 2 with meningitis, and 1 with cerebrovascular infarction. One patient had headache and difficulties with concentration and thinking for 1 month after the acute infection. M. pneumoniae was detected, by means of PCR, in the CSF of 4 patients with negative culture results. Two had epileptic seizures, 1 had blurred vision as a consequence of edema of the optic disk, and 1 had peripheral nerve neuropathy.- - - - - - - - - - ranking = 5keywords = ms (Clic here for more details about this article) |
30/551. Neurologic symptoms in children with systemic lupus erythematosus.Neurologic complications of systemic lupus cerebritis are not as well known in children as in adults. Twenty-five children with neurologic complications were identified after reviewing the hospital medical records of 86 children with systemic lupus erythematosus. Seven children (28%) had neurologic symptoms at the time of initial diagnosis of systemic lupus erythematosus; median time between diagnosis of systemic lupus erythematosus and onset of neurologic complications was 1 month (range 0-5 years). seizures were the most common neurologic symptoms overall, but headaches were the most frequent neurologic manifestation in children without a previous diagnosis of systemic lupus erythematosus. Sixteen children had seizures, and 12 children had seizures as the initial central nervous system involvement. Almost all children who developed seizures had an established diagnosis of systemic lupus erythematosus; only one child had seizures that led to the diagnosis of systemic lupus erythematosus. No patient had status epilepticus, and, in general, seizures were not difficult to control. In six children, headache was the initial symptom of central nervous system involvement. Five children had lupus cerebritis, three children had stroke, and two had isolated cranial neuropathies. chorea was seen in only two cases, and three children had pseudotumor cerebri. Treatment with high-dose intravenous methylprednisolone led to a good response in 18 children; cyclophosphamide was required in 6 patients and plasmapheresis in 1 child. Outcome was generally good, although one child developed fulminant cerebritis with intracranial hypertension and died.- - - - - - - - - - ranking = 6keywords = ms (Clic here for more details about this article) |
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