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At least 1,621 records · Page 90Linked to original sources
PubMed1986

Cerebro-oculo-hepato-renal syndrome (Arima' syndrome): a distinct clinicopathological entity.

Three children with Leber's congenital amaurosis, agenesis of the cerebellar vermis, and infantile polycystic kidneys are described. The common clinical findings of three unrelated patients (two boys and one girl) included severe visual impairment from early infancy, profound psychomotor retardation, hypotonia, nystagmus, characteristic facial appearance with blepharoptosis, and progressive chronic renal insufficiency. The two boys died of uremia at ages 13 and 12 years. The common pathological findings in these two patients consisted of minor disproportions of cerebral lobes, almost total aplasia of the cerebellar vermis, micropolygyria of the dentate nuclei, malformations of the brain stem (including pachygyria of the inferior olivary nuclei and partial absence and anomalous position of the pyramidal tracts), and infantile polycystic kidneys; there was fatty liver in one case and hepatic fibrosis in the other. The clinicopathological findings of our two patients were entirely compatible with those of patients previously reported by Arima and other Japanese authors. Therefore, these patients seem to comprise a distinct clinicopathological entity, cerebro-oculo-hepato-renal syndrome (Arima's syndrome), different from other syndromes with retinal, cerebellar, and renal abnormalities.

Blindness↗
PubMed2004

Cortical neuromagnetic fields preceding voluntary jaw movements.

Slow cortical potentials (readiness potentials, RPs) reflecting the central programming of voluntary jaw movements were reported to appear preceding the movements. However, the current source producing the RP has not yet been localized. This study aimed to determine the cortical regions involved in the central programming of bilaterally symmetrical voluntary jaw movements, by locating the current source of the neuromagnetic counterpart of the RP (readiness field, RF). The RFs were found in the fronto-lateral region bilaterally, starting around 860 and 600 ms prior to the onset of masseter and digastric electromyograms (EMGs), respectively, and gradually increasing in magnitude to the peak within 100 ms before the EMG onset. Thus, the RFs appeared long before the reported onset of the excitability increase of pyramidal tract neurons. The current sources producing the RFs were located in the precentral gyrus bilaterally, with no bilateral differences in strength. We conclude that the primary motor cortex is involved bilaterally in central programming as well as in execution of bilaterally symmetrical voluntary jaw movements.

Adult↗
PubMed2003

Productive infection of cerebellar granule cell neurons by JC virus in an HIV+ individual.

BACKGROUND: In the setting of severe immunosuppression, the polyomavirus JC (JCV) can cause a lytic infection of oligodendrocytes. This demyelinating disease of the CNS white matter (WM) is called progressive multifocal leukoencephalopathy (PML). JCV has a very narrow host-cell range and productive infection of neurons has never been demonstrated. Patient, methods, and results: An HIV-1-infected patient presented with signs of pyramidal tract and cerebellar dysfunction. Brain MRI revealed T2 hyperintensities in the WM of both frontal lobes and cerebellar atrophy. His disease progressed despite therapy and he died 6 months later. In addition to classic PML findings in the frontal lobe WM, autopsy revealed scattered foci of tissue destruction in the internal granule cell layer (IGCL) of the cerebellum. In these foci, enlarged granule cell neurons identified by the neuronal markers MAP-2 and NeuN reacted with antibodies specific for the polyomavirus VP1 capsid protein. Electron microscopy showed 40 nm viral particles, consistent with polyomaviruses, in these granule cell neurons. In addition, JCV DNA was detected by PCR after laser capture microdissection of cells from the areas of focal cell loss. Finally, in situ hybridization studies demonstrated that many granule cell neurons were infected with JCV but did not contain viral proteins. Sequence analysis of the JCV regulatory region from cerebellar virions showed a tandem repeat pattern also found in PML lesions of the frontal lobe WM. CONCLUSION: JCV can productively infect granule cell neurons of the IGCL of the cerebellum. This suggests a role for JCV infection of neurons in cerebellar atrophy occurring in HIV-infected individuals.

Acquired Immunodeficiency Syndrome↗
PubMed2004

Infantile Parkinsonism-dystonia and elevated dopamine metabolites in CSF.

Two girls and one boy are described, with severe infantile parkinsonism-dystonia. This syndrome is usually caused by endogenous dopamine deficiency but in these patients was associated with elevated dopamine metabolites in CSF and an unusual eye movement disorder: ocular flutter together with saccade initiation failure. Pyramidal tract signs also emerged in the course of the disease in two patients. This combination of symptoms and biochemical findings suggests a unique pathogenic mechanism.

Dopamine↗
PubMed2004

Five new cases of a recently described leukoencephalopathy with high brain lactate.

BACKGROUND: A new leukoencephalopathy with brainstem and spinal cord involvement and high brain lactate was recently defined. The authors describe five new patients with this entity. METHODS: Brain MRI was performed in all patients and spinal MRI and proton magnetic resonance spectroscopy (1H-MRS) in four patients. Laboratory examinations ruled out classic leukodystrophies. RESULTS: MRI showed signal abnormalities in the periventricular and deep white matter, in the pyramidal tracts, mesencephalic trigeminal tracts, in the cerebellar connections, and in dorsal columns of the spinal cord. MRS showed decreased N-acetylaspartate and increased lactate in the white matter of all patients. In one patient choline-containing compounds were elevated. A slowly progressive sensory ataxia and tremor manifested at the age of 3 to 16 years and distal spasticity in adolescence. One 13-year-old patient was asymptomatic. CONCLUSIONS: A slowly progressive sensory ataxia is a typical feature in this new leukodystrophy. MRS favors a primary axonal degeneration.

Adolescent↗
PubMed2004

Autosomal dominant congenital non-progressive ataxia overlaps with the SCA15 locus.

BACKGROUND: Most patients with pure nonprogressive congenital cerebellar ataxia have a sporadic form of unknown heredity and etiology. Several small families have been reported with a dominantly inherited nonprogressive congenital ataxia (NPCA). METHODS: The authors ascertained and clinically characterized a four-generation pedigree segregating an autosomal dominant type of congenital nonprogressive cerebellar ataxia associated with cognitive impairment. Following the exclusion of several SCA localizations (SCA-1, 2, 3, 4, 5, 6, 7, 8, 10, 12, 17, IOSCA, and DRPLA), a genome-wide linkage study was performed. RESULTS: Examination of the family showed that all affected members had gait ataxia and cognitive disability with variable features of dysarthria, dysmetria, dysdiadochokinesia, nystagmus, dystonic movements, and cerebellar hypoplasia on imaging. Clinical signs of pyramidal tract dysfunction and sensory changes were absent. A genome-wide search in this family detected linkage to chromosome 3p with a maximum two-point lod score of 4.26 at D3S3630. This localization to the pter is distal to D3S1304, as defined by a recombination event. This overlaps with the SCA15 locus, with the critical overlapping region between the microsatellite markers, D3S1304 and D3S1620 (approximately 8 cM). CONCLUSION: Autosomal dominant congenital nonprogressive cerebellar ataxia with or without cerebellar hypoplasia overlaps with the SCA15 locus on chromosome 3pter.

Adolescent↗
PubMed2006

Electrophysiologic characterization in spinocerebellar ataxia 17.

The authors performed a multimodal electrophysiologic evaluation in nine patients belonging to four SCA17 (spinocerebellar ataxia type 17) families. Peripheral nerve and visual system were not involved. Brainstem auditory evoked potentials were constantly abnormal with central type lesions. Magnetic motor evoked potentials were abnormal only in the lower limbs, suggesting a length-dependent involvement of the pyramidal tract. Somatosensory evoked potentials were abnormal in almost all our patients, and abnormalities were consistent with a somatosensory pathway involvement along the brainstem.

Adult↗
PubMed1976

Familial motor neuron disease. Evidence for at least three different types.

Based on a clinical, pathologic, and genetic study of 14 families, at least three types of familial motor neuron disease can be distinguished, all apparently of autosomal dominant transmission. The first is characterized by rapid, progressive loss of motor function with predominantly lower motor neuron manifestations and a course lasting less than 5 years. Pathologic changes are limited to the anterior horn cells and pyramidal tracts. The second type is clinically identical to the first, but at autopsy additional changes are found in the posterior columns, Clarke's column, and spinocerebellar tracts. The third type is characterized by a much longer survival usually beyond 10 and after more than 20 years in affected family members but is otherwise similar to the second type.

Female↗
PubMed1979

Assessing segmental excitability after acute rostral lesions: II. The blink reflex.

Blink reflexes were obtained in a total of 34 different patients with cerebrovascular lesions; 28 were studied within 1 week of the ictus and 11 were eventually restudied. In 13, the latency of the first component of the reflex on the clinically involved side was significantly delayed (greater than 1.5 msec). This was an acute phenomenon that usually resolved within the first week after the ictus, correlated clinically with the severity of the hemiparesis and decrease in tone, and may be seen with lesions restricted to the pyramidal tract. The second component was absent in 17 of 34 patients during the early phase after stroke, and was associated with a decreased corneal response. These changes, which reflect decreased excitability of certain brainstem structures (rather than a lesion within those structures), are therefore potentially important for documenting CNS pathophysiology.

Cerebrovascular Disorders↗
PubMed1980

Cockayne syndrome: an atypical case.

We report a case of Cockayne syndrome with several atypical features. The patient displayed many of the typical features, including cachectic bird-headed dwarfism, photodermatitis, normal pressure hydrocephalus, and extraphyramidal and pyramidal tract signs. However, she also displayed the unusual and previously unreported features of late age at onset, relative stability until age 19, and fertility, with a successful pregnancy. Brain biopsy showed hypomyelination, no active demyelination, and fibrillary gliosis.

Adult↗
PubMed1980

Partial pontine hematomas.

Two hypertensive men presented clinically with small, largely unilateral hematomas of the pons and midbrain demonstrated by computerized tomography. Clinical manifestations included "ocular bobbing," complex forms of ophthalmoplegia, Cheyne-Stokes respiration, and pyramidal tract signs. The "typical" sings of massive pontine hemorrhage--including coma, pinpoint pupils, and hyperthermia--were absent.

Adult↗
PubMed1981

Corneal reflex latency in lesions of the lower postcentral region.

Corneal reflex latencies were studied in 53 patients with cerebral lesions. Five distinct types of abnormality were identified in 30 of these patients. The findings suggest that the lower postcentral region has an excitatory influence upon interneurons of the lateral reticular formation of the lower brainstem mediating the trigeminofacial connections of the corneal reflex. This influence descends by way of postcentral corticobulbar fibers that pass through the cerebral peduncle and pyramidal tract.

Blinking↗
PubMed1982

Nonabsorptive hydrocephalus associated with nephropathic cystinosis.

A child with nephropathic cystinosis developed seizures and coma. CT showed prominent sulci and slight ventricular enlargement. Nuclear cisternogram was normal. Despite successful renal transplantation and treatment of hypothyroidism, neurologic recovery was poor. CT and nuclear cisternogram 5 months later showed moderate panventricular and subarachnoid space enlargement and abnormal ventricular isotope retention. Ventriculoperitoneal shunt placement was followed by improved intellectual function, resolution of pyramidal tract signs, and control of seizures. Anisotropic crystals consistent with cystine were demonstrated in biopsy samples of arachnoid and cerebral cortex. Nonabsorptive hydrocephalus may have resulted from deposition of cystine in the meninges.

Arachnoid↗
PubMed1983

Myoclonus, cerebellar disorder, neuropathy, mitochondrial myopathy, and ACTH deficiency.

A 50-year-old Japanese woman with action myoclonus, cerebellar signs, neuropathy with axonal degeneration and onion-bulb formation, muscle atrophy with mitochondrial abnormalities, and isolated ACTH deficiency was reported. Her daughter had myoclonus epilepsy and cerebellar ataxia. Neuropathologic findings included atrophy of the dentate and inferior olivary nuclei, Purkinje's cell loss, and demyelination of the posterior columns and spinocerebellar and pyramidal tracts of the spinal cord, besides severe respirator changes. Lafora's bodies were absent. The present case should be included in the entity "myoclonus epilepsy associated with mitochondrial myopathy."

Adrenocorticotropic Hormone↗
PubMed1983

Multifocal central nervous system damage caused by toluene abuse.

Four toluene abusers had evidence of severe multifocal central nervous system damage. Impairment of cognitive, cerebellar, brainstem, auditory, and pyramidal tract function, as well as CT evidence of cerebral cortical, cerebellar, and brainstem atrophy, have been noted. In addition, we found opsoclonus, ocular flutter, and ocular dysmetria. All three patients tested had abnormal brainstem auditory evoked potentials, indicative of brainstem dysfunction. The patient with opsoclonus had CT evidence of brainstem, cerebellar, and cerebral cortical atrophy.

Adolescent↗
PubMed1985

Spinal cord degeneration in AIDS.

Ten months before his death, a 36-year-old homosexual man with AIDS developed progressive symmetric paraparesis with muscle wasting, paresthesias, reflex-uninhibited bladder function, and bowel incontinence. Neuropathologic postmortem examination revealed a symmetric degeneration of the lateral and anterior pyramidal tracts as well as the posterior columns. Scattered microglial nodules were present in the brain and spinal cord. The cause of this spinal cord complication remains uncertain. Possible etiologies of the demyelinating process include infectious, parainfectious, nutritional, paraneoplastic, and toxic causes.

Acquired Immunodeficiency Syndrome↗
PubMed1986

AIDS: calcification of the basal ganglia in infants and children.

CT or postmortem examination demonstrated calcification of the basal ganglia in eight infants and children with acquired immune deficiency syndrome. Serial CT studies documented progression of both bilateral symmetric calcium densities and cerebral atrophy. Clinical features included progressive encephalopathy with dementia, and pyramidal tract signs. Postmortem examination of four children revealed variable degrees of calcific vasopathy of the basal ganglia, involving predominantly the putamen and globus pallidus.

Acquired Immunodeficiency Syndrome↗
PubMed1988

Dementia: characteristics of a referral population and factors associated with progression.

We evaluated in a standard fashion 375 patients presenting with complaints of memory loss. Etiology of memory loss included senile dementia of the Alzheimer type (SDAT)-70%, vascular dementia-5%, mixed dementia (SDAT + vascular)-9%, and other etiologies-16%. Incontinence, transient symptoms, and gait disturbances occurred more frequently in vascular dementia than in SDAT. A history of cardiovascular disease and stroke was more common in vascular dementia than SDAT. Disturbances of gait, bradykinesia, and pyramidal tract findings were commonly seen in vascular dementia. Advanced technology aided diagnosis in only 6% of patients and CT was the most useful of such tests. An earlier age of onset was noted in those with a positive family history of SDAT. Duration of symptoms at presentation for SDAT patients varied inversely with the rate of progression of dementia 15 to 55 months later, suggesting that individuals who progress more slowly require more time to elapse before the family or patient realizes the need for medical attention.

Aged↗