1/56. Parkinsonism in children resulting from mesencephalic tumors.Parkinsonism secondary to intrinsic brain stem mass lesions is rare. Two children with parkinsonism caused by mesencephalic tumors are reported. The pathophysiological mechanisms of this association are discussed and the literature describing similar cases is reviewed.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/56. Primary central nervous system lymphoma presenting with a parkinsonian syndrome of pure akinesia.The incidence of primary central nervous system lymphoma (PCNSL), once a rare tumour, has risen significantly in both immunocompetent and immunosuppressed patients. Although infiltration of the basal ganglia is not uncommon in PCNSL, extrapyramidal movement disorders are generally not recognised as a mode of clinical presentation of this type of cerebral tumour. We present the unusual case of a 75-year-old man who developed a parkinsonian syndrome of "pure akinesia" due to autopsy-confirmed PCNSL primarily involving the globus pallidus bilaterally. Parkinsonism due to bilateral pallidal lesions is known but rare, and such cases help in the understanding of basal ganglia function with regard to akinesia and freezing.- - - - - - - - - - ranking = 1.9467689607754keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/56. Parkinsonism due to predominant involvement of substantia nigra in Japanese encephalitis.OBJECTIVE: To study the clinical correlates of lesions seen predominantly in the substantia nigra in some patients with Japanese encephalitis (JE). BACKGROUND: JE typically involves thalamus, brainstem, spinal cord, and cerebral cortex. Rarely, basal ganglia and cerebellum may be affected. Lesions are often widespread and discrete. Predominant involvement of substantia nigra in JE has not been previously reported. methods: Of 52 patients with JE seen in an endemic zone, five were selected on the basis of isolated lesions in the substantia nigra on MRI; all were subjected to detailed clinical and laboratory evaluation. RESULTS: Presenting symptoms were fever, alteration of consciousness, neck stiffness, and decreased body movements. Examination during acute illness revealed restricted eye movements, opsoclonus, upbeating nystagmus, and cogwheel rigidity. There was early and complete recovery of consciousness and eye signs. Parkinsonian features such as positive glabellar tap sign, masklike face, bradykinesia, tremors, and postural instability became apparent as these patients started walking. Reversible mutism was observed in three patients during the acute phase. Response to levodopa, amantadine, and trihexiphenedyl was partial. Three patients were followed for more than 1 year, during which time their parkinsonian features recovered completely. Substantial recovery was also observed in the two other patients 2 months after regaining consciousness. CONCLUSIONS: Some patients with Japanese encephalitis may have lesions predominantly in the substantia nigra. After recovery from acute encephalitic illness, they manifest clinically with typical parkinsonian features. Although several viruses are known to cause parkinsonism, this is the first demonstration of a virus producing lesions predominantly in the substantia nigra and causing parkinsonism.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
4/56. Extrapontine myelinolysis with parkinsonism after rapid correction of hyponatremia: high cerebrospinal fluid level of homovanillic acid and successful dopaminergic treatment.Extrapontine myelinolysis (EPM) is a demyelinating process of the brain. We report the case of an 11-year-old girl who developed EPM with parkinsonism. magnetic resonance imaging revealed demyelinating patterns in the basal ganglia without central pontine lesions. The cerebrospinal fluid levels of homovanillic acid and 5-hydroxyindoleacetic acid were high at the time of onset and normalized upon complete recovery from extrapyramidal symptoms after a dopaminergic treatment. We speculated that demyelination of nerve fibers containing dopamine receptors in the striatum might be a main cause of these symptoms.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
5/56. Parkinsonism, dementia and vertical gaze palsy in a Guamanian with atypical neuroglial degeneration.A 58-year-old Chamorro female patient, who died in 1993, was examined clinicopathologically. At the age of 51, she suffered from hemiparkinsonism, then bradykinesia, rigidity without tremor, and dementia. Extrapyramidal symptoms developed, and at the age of 57, vertical gaze palsy was noted. The clinical diagnosis was parkinsonism-dementia complex (PDC) with vertical gaze palsy. The brain showed atrophy in the frontal and temporal lobes, and the atrophy was accentuated in the dentate gyrus, Ammon's horn and parahippocampal gyrus. The basal ganglia, thalamus and midbrain were moderately atrophic. The substantia nigra and locus ceruleus were completely depigmented. Numerous neurofibrillary tangles (NFTs) were seen in the subiculum and amygdaloid nucleus. Many NFTs were evident in the parahippocampal gyrus, lateral occipitotemporal gyrus, insula, Sommer sector, basal nucleus of meynert, lateral nucleus of the thalamus, subthalamic nucleus and brain stem, and several were observed in the globus pallidus and hypothalamus. The Sommer sector, substantia nigra, locus ceruleus and basal nucleus of meynert showed severe loss of neurons, and a moderate loss of neurons was exhibited by the globus pallidus. These findings were apparently consistent with those associated with PDC. However, in this patient, severe neuronal loss was seen in the subthalamic nucleus and lateral nucleus of the thalamus, and grumose degeneration, which has not previously been reported in PDC, was seen in the dentate nucleus. In addition, many tufted astrocytes, which have been reported to occur in progressive supranuclear palsy (PSP) and postencephalitic parkinsonism, but scarcely observed in PDC, were present. Furthermore, astrocytic plaques, which have been considered as a specific finding of corticobasal degeneration (CBD), were observed in the cerebral cortex. On the other hand, granular hazy astrocytic inclusions, previously reported to occur in PDC, were not seen. Chromatolytic neurons were not observed. The question thus arises as to whether it is appropriate to consider this patient as having suffered from a combination of PDC, PSP and CBD. From the view points of absence of granular hazy astrocytic inclusions and chromatolytic neurons, and of tufted astrocytes in the neostriatum, it is conceivable that this patient is a case of a new disease entity.- - - - - - - - - - ranking = 3keywords = brain (Clic here for more details about this article) |
6/56. A new case of frontotemporal dementia and parkinsonism resulting from an intron 10 3-splice site mutation in the tau gene: clinical and pathological features.Hereditary frontotemporal dementia and parkinsonism (FTDP) linked to chromosome 17 (FTDP-17) constitutes a new form of tauopathy, and mutations in the tau gene have recently been reported in some affected families. This report presents clinical and neuropathological data from a member of a British family (SOT 254) with a history of dementia and movement disorder. The medical history of the affected patient, a woman aged 44 years, was reviewed, and a detailed post-mortem examination of the brain was undertaken. A panel of well characterized phosphorylation-dependent and independent anti-tau antibodies was used to assess tau pathology, and inclusions were examined by electron microscopy. Neuronal loss and gliosis were widely distributed, but most severe in neocortical regions, and were associated with abundant neuronal and glial tau inclusions which consisted of a mixture of paired helical filaments (PHFs), similar to those in Alzheimer's disease, and distinct twisted ribbon-like filaments. Genomic dna was obtained from post-mortem tissue from the index patient, and blood from two unaffected members of the same family. For the index case only, sequencing of intronic sequences flanking exon 10 of the tau gene identified a G to A transition at position 3 of the splice-donor site downstream of exon 10, identical to that reported in multiple system tauopathy with presenile dementia (MSTD). The clinical, neuropathological and genetic findings strongly suggest that SOT 254 represents a new example of FTDP-17 resulting from a mutation in the tau gene. These results are compared with those reported for other FTDP-17 families, i.e. for MSTD.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
7/56. A possible association between exposure to n-hexane and parkinsonism.Recently, some case-control studies and case reports have shown an association between solvent exposure and parkinsonisms. We present a 55-year-old male parkinsonian patient with chronic exposure to n-hexane for 17 years. The results of neurophysiological (electromyography, evoked potentials), neuroradiological (MRI) and neuropsychological tests performed on the patient suggest a role of this solvent at the level of the central nervous system. Biological susceptibility to neurotoxic compounds is discussed briefly.- - - - - - - - - - ranking = 0.38935379215507keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/56. Parkinsonism as unusual neurological complication in childhood systemic lupus erythematosus.Parkinsonism complicating systemic lupus erythematosus (SLE) is extremely rare. We report two girls with SLE who developed extrapyramidal parkinsonian features after an initial stormy course. One patient presented with generalized tonic clonic seizure and was then noted to have akinetic mutism and masked face. MRI brain revealed abnormal signals in bilateral basal ganglia and single photon emission computed tomography (SPECT) showed hypoperfusion in the same area. EEG background was slow and disorganized. Symptoms persisted despite high dose intravenous methylprednisolone and cyclophosphamide. Intravenous immunoglobulin (IVIG) was prescribed empirically and was followed by complete recovery. Both EEG and MRI brain were normal on follow-up. The second patient was found unconscious and then developed bradykinesia, mutism and shuffling gait. MRI and SPECT both detected abnormalities in basal ganglia. EEG was slow. Intravenous immunoglobulin was given after methylprednisolone and cyclophosphamide. This was followed by clinical improvement. The pathogenesis of basal ganglia injury in SLE, along with the management of cerebral lupus and the mechanisms of action of IVIG, are discussed.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
9/56. Juvenile parkinsonism: a heterogeneous entity.We studied the clinical features, laboratory investigation, management and natural history of a cohort of patients with Juvenile Parkinsonism (JP), seen at a tertiary referral centre. JP was defined as Parkinsonism with onset at age 20 years or less. Six patients (five male, one female) entered the study. The mean age at onset of Parkinsonism was 12.5 years (range 7-19) and the mean follow-up time was 49.3 months (range 40-57). Bradykinesia, rigidity, and postural instability were observed in all patients and five subjects had tremor. dystonia was present in four subjects. Other clinical features were dementia (five subjects), supranuclear ophthalmoparesis (five subjects), seizures (three subjects), multifocal myoclonus (one subject), decreased deep reflexes (one subject), pyramidal signs (one subject). family history of Parkinson's disease (PD) was positive in one subject. work-up for Wilson's disease was negative in all patients. neuroimaging studies showed cortical atrophy in two subjects and mild brainstem atrophy in two others. Sea-blue histiocytes were found in one subject. L-dopa improved the Parkinsonism in all subjects but four rapidly developed fluctuations and dyskinesias, requiring, in one, stereotaxic surgery. After a mean disease duration of 6.5 years, five subjects require assistance for performance of all daily activities. JP is a heterogeneous clinical entity. In the majority of patients, no underlying cause is identified. The unusual clinical features suggest most subjects have a CNS degenerative disease distinct from PD. There is, however, evidence suggesting that PD may rarely cause JP. Gangliosidosis is another cause of L-dopa-responsive JP. Regardless of the cause, in the present study JP displays an aggressive and rapidly progressive course in most patients.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
10/56. Parkinsonian syndrome in a patient with a pterional meningioma: case report and review of the literature.The onset of a Parkinsonism in a patient with intracranial meningioma is definitely rare. The authors described the case of a patient suffering from a Parkinsonian syndrome for 10 years with no evidence of clinical improvement after medical treatment. A CT-scan of the brain evidenced a right pterional intracranial meningioma. The complete surgical removal of the neoplasm succeeded in resolving the Parkinsonian syndrome. The extension of the neoplasm and of the peritumoral edema may play an important role in compressing and consequently impairing perfusion of the basal ganglia region.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
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