Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030552 (paresis)
 5,831 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In 25 patients with subacute sclerosing panencephalitis in various phases of the disease CT and NMR imaging of the brain were done at the same times and the findings were related to clinical symptomatology. NMR imaging, in contrast to CT imaging, demonstrated even very small brain changes in the initial stage. The inflammatory-demyelinizing process begins in SSPE as a rule in the white matter of the occipital lobes, and only later in appears in the vicinity of the anterior horns of the lateral ventricles. Spreading of subcortical changes in the occipital lobes and their later penetration into the parietal lobes causes the development of ideatory apraxia frequent in these cases, and involvement of the cortex of the occipital lobes leads to visual agnosia. The paresis of extremities in later stages not always are correlated with greater intensity of changes in the contralateral cerebral with greater intensity of changes in the contralateral cerebral hemisphere. Hydrocephalus developing after longer duration of the disease is an expression of postinflammatory brain atrophy and not of disturbances in cerebrospinal fluid absorption.
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PMID:[Changes in NMR and CT images in SSPE]. 806 44

Achondroplasia is an autosomal dominant condition that occurs in approximately 1 of 25,000 births. It has long been associated with neurologic morbidity and mortality in adults, but more recently it has been increasingly identified in children. Neurological sequelae of achondroplasia includes spinal stenosis, spinal cord compression at the foramen magnum (which can result in fatal acute craniocervical junction compression), hydrocephalus, radiculopathy, paresis, and abnormal spinal curvature. We report the case of a 12-year-old achondroplastic patient who incurred an apparently nontraumatic cervical spinal cord infarction, with resultant quadriplegia, with no apparent cause, which was complicated by impaired tolerance of temperature changes and hypercalcemia of immobilization. Whereas persons with achondroplasia have many of the same physical and functional impairments from spinal cord injury as other SCI patients, they are more likely to experience certain types of neurologic deficits and are more subject to other problems because of their altered body habitus.
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PMID:Spinal cord injury rehabilitation in a pediatric achondroplastic patient: case report. 829 50

Osteopetrosis is an inherited skeletal condition characterized by increased bone radiodensity. There are three clinical groups: infantile-malignant autosomal recessive, fatal within the first few years of life (in the absence of effective therapy); intermediate autosomal recessive, appears during the first decade of life but does not follow a malignant course; and autosomal dominant, with full-life expectancy but many orthopaedic problems. The infantile variant shows a myelophthisic anemia, granulocytopenia, and thrombocytopenia, and patients eventually die from infection or bleeding or both. Neurologic sequelae include cranial nerve compression (optic nerve, blindness; auditory nerve, deafness; facial nerve, paresis), hydrocephalus, convulsions, and mental retardation. Radiographs show uniform bone density without corticomedulary demarcation, broadened metaphyses, "bone within a bone" or endobone phenomena (tarsals, carpals, phalanges, vertebra, ilium), and thickened growth plates if there is superimposed rickets. Transverse pathologic fractures occur, often followed by massive periosteal bone formation. Computed tomographic scans, magnetic resonance imaging, and bone scans provide specific information. Iliac crest bone biopsy is valuable to quantitate osteoclast and marrow changes by light and electron microscopy. Medical treatments involve high-dose calcitriol to stimulate osteoclast differentiation and bone marrow transplantation to provide monocytic osteoclast precursors. Orthopaedic problems in the intermediate and autosomal dominant forms include increased fractures, coxa vara, long-bone bowing, hip and knee degenerative arthritis, and mandibular and long-bone osteomyelitis. Cranial nerve compression also occurs. Osteotomy, plating, intramedullary rodding, and joint arthroplasty can be done, but are difficult because of bone hardness.
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PMID:Osteopetrosis. Current clinical considerations. 835 40

The indications for hindbrain decompression in neonates and young infants with spinal dysraphism who experience brain stem dysfunction in association with Chiari II malformations have remained controversial. This largely reflects the fact that the postoperative outcome in such patients has often been poor, which has supported the belief that much of the brain stem compromise in these patients is congenital and inherently irreversible. However, in a previous retrospective review of our operative results between 1975 and 1989, we noted that a significant component of the brain stem dysfunction in these children was an acquired phenomenon that potentially was reversible with prompt operative intervention. Accordingly, we hypothesized that with early craniocervical decompression, excellent functional outcome could be achieved in a majority of neonates and young infants with symptomatic Chiari II malformations. On the basis of this premise, we prospectively treated all such patients since 1989 with urgent brain stem decompression after other potential causes for brain stem dysfunction, such as progressive hydrocephalus, had been ruled out. All children underwent limited suboccipital craniectomies, cervical laminectomies extending beneath the inferior extent of the cerebellar tissue, and dural decompressions. The outcome in these patients has been favorable in comparison with previous studies. Ten of the 13 children treated according to this protocol recovered normal or nearly normal brain stem function shortly after decompression; 1 child had mild residual unilateral lower cranial nerve paresis. None of these children required a tracheostomy for ventilatory support, and only one required a temporary gastrostomy. The other three children all exhibited bilateral vocal cord paralysis and severe central hypoventilation by the time decompression was performed and failed to have any meaningful recovery of function. We conclude that early recognition of the symptoms of brain stem compromise in neonates and young infants with spinal dysraphism coupled with urgent evaluation and decompression are effective in producing prompt resolution of the brain stem dysfunction in most affected patients. Conversely, the prognosis for recovery is poor in children who exhibit bilateral vocal cord paralysis by the time of decompression.
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PMID:The effect of early craniocervical decompression on functional outcome in neonates and young infants with myelodysplasia and symptomatic Chiari II malformations: results from a prospective series. 869 88

We report a 69-year-old woman of Mexican origin with a 6-year history of progressive paresis, mild peripheral neuropathy, and recent onset of fluctuating mental status. Head and spinal MRI revealed contrast enhancing thickened meninges which on biopsy disclosed amyloid deposition. Immunohistochemistry identified the amyloid as transthyretin (TTR), and polymerase chain reaction/restriction fragment length polymorphism analysis of blood revealed a Val30Met mutation in one of her TTR genes. This mutation causes familial (hereditary) amyloidotic polyneuropathy of the Portuguese type (FAP 1). However, unlike FAP 1, in which peripheral neuropathy is a dominant feature, our patient's clinical manifestations, which included communicating hydrocephalus and myelopathy, were more suggestive of familial oculoleptomeningeal amyloidosis (FOLMA). In summary, the clinical presentation of TTR Met 30 mutation is more varied than previously suspected, and leptomeningeal amyloidosis should be considered in the differential diagnosis of obscure conditions involving meninges.
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PMID:Massive leptomeningeal amyloidosis associated with a Val30Met transthyretin gene. 885 32

Hydrocephalus associated with aqueductal stenosis is not uncommon after the 2nd year of life. In some patients, stenosis is due to slow-growing periaqueductal tumors that can only be revealed by magnetic resonance examination. We reviewed 31 cases of children with aqueductal stenosis and hydrocephalus diagnosed after the second year of life, and operated on in the Section of Paediatric Neurosurgery of UCSC between 1982 and 1993. Eighteen cases (Group I) had nonneoplastic aqueductal stenosis, while in 13 cases (Group II) it was demonstrated a periaqueductal tumor by NMR. Intracranial hypertension was the most frequent symptom at diagnosis in both groups. In Group I mental and growth retardation were frequent, while cerebellar signs were quite common in Group II. The treatment of choice for hydrocephalus was a V-P shunt. Tumors in Group II were not directly treated, because of their benign behaviour. Subdural hematoma was a relatively common shunt complication in these patients. In 2 cases we observed an atypic complication: patients developed an altered level of consciousness, upsight paresis and distony, without any sign of increased intracranial pressure, or cerebro-spinal fluid infection. One of these patients died; we treated the second patient with L-Dopa (Sinemet 150 mg/die); he progressively improved and had a complete remission of symptoms. Surgical mortality was nil; long-term mortality was 12.5%. In 76.1% of Group II patients we have not observed any tumor growth; 50% of Group I patients has still a complete remission of preoperative symptoms (follow-up 2-12 years).
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PMID:[Late clinical manifestations of hydrocephalus associated with aqueductal stenosis]. 890 May 60

In a 35-year-old woman who presented with acute somnolence, confusion and slow irregular breathing, Epstein-Barr virus (EBV) meningoencephalitis was diagnosed after serological testing and a polymerase chain reaction of the cerebrospinal fluid. She developed papilloedema and bilateral nervus abducens paresis. A CT scan showed generalized oedema of the brain and triventricular hydrocephalus. Treatment with a ventriculoperitoneal shunt and ganciclovir led to complete recovery. Meningoencephalitis is a not uncommon, yet rarely reported complication of infectious mononucleosis. It usually runs a mild course with spontaneous and full recovery. Hydrocephalus secondary to aqueduct stenosis is a complication of Epstein-Barr virus (EBV) meningoencephalitis which has not been reported in adults before. The disease should be considered whenever the clinical condition deteriorates or neurological symptoms increase.
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PMID:[Meningo-encephalitis and hydrocephalus caused by Epstein-Barr virus]. 962 31

We present the case of a 13-month old Turkish boy of Kurdish origin with tuberculous meningitis. Fever of unknown origin and neurologic symptoms (loss of ability of walking and free sitting, cerebral seizures, central paresis of the VII. cranial nerve, coma) led to the diagnosis. Cranial CT demonstrated hydrocephalus and enhancement of the basal meninges after contrast injection; the chest x-ray showed an infiltrate in the right upper lobe of the lung and the cerebrospinal fluid (CSF) mild pleocytosis with elevated protein and reduced glucose concentrations. Diagnosis was confirmed by detection of Mycobacterium tuberculosis in the CSF by polymerase chain reaction (PCR). Immediately, surgical and level-controlled tuberculostatic treatment was initiated. The patient recovered completely.
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PMID:[Tuberculous meningitis in a 13-month-old boy: a case report]. 962 48

A 5-year-old boy with hydrocephalus and a lumbar myelomeningocele underwent extension of peritoneal tube of ventriculoperitoneal (VP) shunt system. Prior to the operation he had been able to walk independently with the use of braces, to speak complex sentences and to sing songs. After the surgery, he lost consciousness and became critically ill with irregular respiration. He was artificially ventilated for 10 days, and then recovered, with sequelae of right facial paresis and slight dysarthria. T1 weighted magnetic resonance image showed high intensities of the medial part of the inferior lobe of the cerebellum and medulla oblongata, which were interpreted as representing edema. This case illustrates that Chiari II malformation can become symptomatic after a VP shunt trouble.
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PMID:[Manifestation of Chiari II symptoms following peritoneal shunt tube extension]. 978 Jul 41

In a hospital population-based retrospective study of neonatal meningitis, 55 cases were identified over a period of 10 years. The prevalences of meningitis for preterm and term newborns were 3.66 and 0.97 per 1000, respectively (22/6465 vs 33/36638; p < 0.01). The overall prevalence was 1.37 per 1000 live births. Twenty-two (40%) babies with meningitis died, more preterm than term (13/22 vs 9/33; p < 0.05). Known maternal risk factors for neonatal meningitis were observed in 15 (27%) babies. The risk factors were more common in preterm than in term newborns (10/22 vs 5/33; p < 0.05). The common causative organisms were Klebsiella pneumoniae, Escherichia coli and Enterobacter spp. which together accounted for 67% of all CSF isolates. These organisms were evenly distributed between early- and late-onset meningitis, and among term and preterm newborns. Seven of 33 (21%) of the surviving newborns developed neurological complications. The short-term sequelae were hydrocephalus, spastic paresis and seizures.
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PMID:Neonatal meningitis in Addis Ababa: a 10-year review. 992 82


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