1/38. An autopsy case of myotonic dystrophy with mental disorders and various neuropathologic features.An autopsy case of myotonic dystrophy (MD) is reported. The patient was a 58-year-old male. He presented with muscular weakness and muscular atrophy at the age of 33 and was diagnosed as having MD from myotonic symptoms (i.e. percussion and grip myotonia) at 49 years old. mental disorders including a delusional hallucinatory state, mental slowness, indifference, and lack of spontaneity as well as visual cognitive impairments were noted at the age of 55. He showed Parkinsonism and died of septic shock. T2-weighted magnetic resonance imaging demonstrated diffuse cortical atrophy with a marked frontal atrophy and high-intensity signals in the white matter. Single photon emission computed tomography demonstrated hypoperfusion in the frontal cortex. Neuropathologic observation revealed neuronal loss in the superficial layer of the frontal and parietal cortices and extensive neuronal loss in the occipital cortex, intracytoplasmic inclusion body in the nerve cell of the medial thalamic nuclei, neuronal loss and presence of lewy bodies in the substantia nigra and locus ceruleus corresponding to the pathologic features of Parkinson's disease, as well as abnormalities of myelin in the white matter. The present case suggests that in MD brain, various neuropathologic changes may occur and they contribute to the mental disorders.- - - - - - - - - - ranking = 1keywords = state (Clic here for more details about this article) |
2/38. Diffuse lewy body disease presenting as multiple system atrophy.OBJECTIVES: The majority of patients with diffuse lewy body disease have cognitive or psychiatric manifestations as part of their initial presentation. A sizable minority present with parkinsonian features alone. Autonomic features may also occur, typically after the development of cognitive changes. We aim to demonstrate that diffuse lewy body disease may rarely also present with parkinsonism accompanied by marked autonomic dysfunction in the absence of significant cognitive or psychiatric abnormalities. methods: Case report based on a retrospective chart review and neuropathological examination. RESULTS: We report on a patient in whom a clinical diagnosis of multiple system atrophy was made based on a presentation of parkinsonism with prominent and early autonomic involvement. The former included postural tremor, rigidity and bradykinesia, while the latter consisted of repeated falls due to orthostasis and the subsequent development of urinary incontinence midway through the course of her illness. She was poorly tolerant of dopaminergic therapy due to accentuated orthostasis. Benefit from levodopa was limited and only evident when attempted withdrawal resulted in increased rigidity. There was no history of spontaneous or drug-induced hallucinations, delusions or fluctuating cognition, and in contrast to the prominence and progression of her parkinsonian and autonomic features over the first several years, cognitive impairment did not occur until the final stages of her illness, seven years after the onset of initial symptoms. Neuropathological examination revealed numerous lewy bodies in both neocortical as well as subcortical structures consistent with a diagnosis of diffuse lewy body disease. There was marked neuronal loss in the substantia nigra as well as the autonomic nuclei of the brainstem and spinal cord. CONCLUSIONS: In addition to cognitive, psychiatric, and parkinsonian presentations, diffuse lewy body disease may present with parkinsonism and prominent autonomic dysfunction, fulfilling proposed criteria for the striatonigral form of MSA.- - - - - - - - - - ranking = 28836.400383257keywords = rigidity (Clic here for more details about this article) |
3/38. Acute hypernatremia and neuroleptic malignant syndrome in parkinson disease.neuroleptic malignant syndrome is a clinical syndrome characterized by fever, muscle rigidity, and mutism. Some patients with neuroleptic syndrome may have elevated creatine phosphokinase values and abnormal liver aminotransferase values. precipitating factors are important clues for prompt diagnosis. Typical precipitating factors include antipsychotic agents and major tranquilizers. In parkinson disease, drug withdrawal, menstruation, and hyponatremia are precipitating factors. We report a case of neuroleptic malignant syndrome in a patient with parkinson disease and hypernatremia. In addition, we hypothesized that sudden change of sodium concentrations in the central nervous system could trigger neuroleptic malignant syndrome in patients with parkinson disease. According to our experience, neuroleptic malignant syndrome is a clinical diagnosis and prompt diagnosis avoids unnecessary, expensive work-ups.- - - - - - - - - - ranking = 14418.200191629keywords = rigidity (Clic here for more details about this article) |
4/38. NMSP binding to dopamine and serotonin receptors in MPTP-induced parkinsonism: relation to dopa therapy.We tested the hypothesis that N-methylspiperone binding to dopamine D2 receptors must be reduced when L-dopa therapy of parkinsonism augments the binding of dopamine to the receptors and improves the clinical state expressed by the Hoehn & Yahr stage. A patient with MPTP-induced parkinsonism underwent two positron emission tomographic studies of the D2-like dopamine receptors with N-[11C]methylspiperone (NMSP). The first study took place 3 days after cessation of the L-dopa medication, the second 5 days after its resumption. Noticeable clinical deterioration occurred during both studies, consistent with significant dopamine receptor blockade by NMSP and elevated NMSP binding in both scans. The dopa treatment did not reduce the NMSP binding. On the contrary, the rate of binding of NMSP (k3) was increased on-dopa, compared to off-dopa. The increase was consistent with the slightly greater dopamine receptor density estimated after resumption of the dopa therapy. The NMSP binding to serotonin receptors suggested lower synaptic serotonin on-dopa than off-dopa. The results are consistent with negative correlation between the Hoehn & Yahr stage and the amount of dopamine bound to dopamine D2 receptors. Low synaptic serotonin may explain the depression seen in some patients on dopa for Parkinson's disease.- - - - - - - - - - ranking = 1keywords = state (Clic here for more details about this article) |
5/38. Chronic subdural haematomas and Parkinsonian syndromes.We describe three men with parkinsonian syndromes caused or aggravated by chronic subdural haematomas. A 63-year-old man developed tremor at rest, rigidity and bradykinesia one week after he fell and hit his head. A 70-year-old patient suffering from tardive dyskinesia and drug-induced parkinsonism experienced deterioration of his bradykinetic symptoms over two weeks. There was no history of trauma. The third patient, a 82-year-old man with idiopathic Parkinson's disease had a marked increase of his left-sided parkinsonian symptoms. Again, there was no history of trauma. In all three patients chronic subdural haematomas were demonstrated by computed tomography. Evacuation of the chronic subdural haematoma resulted in disappearance respectively improvement of the movement disorder. Diagnostic evaluations appear to be delayed and initial misinterpretations are frequent. The findings of our report and review of the literature point out that a favourable outcome after appropriate surgical treatment is achieved in most instances.- - - - - - - - - - ranking = 14418.200191629keywords = rigidity (Clic here for more details about this article) |
6/38. Parkinsonism as an initial manifestation of brain tumor.Parkinsonism secondary to neoplasm is uncommon. We report two patients with bilaterally symmetric parkinsonism as the initial presentation of their brain tumors. The first patient was a 71-year-old woman who presented with a gradual onset of bilateral resting tremor, bradykinesia and rigidity. Computerized tomography (CT) of the brain revealed a large parasagittal tumor in the left frontal lobe. The patient completely recovered from the parkinsonian symptoms after removal of the brain tumor. The second patient, a 74-year-old man with a history of renal cell carcinoma of the right kidney suffered from an insidious onset of bilateral bradykinesia, rigidity and gait difficulty. Cerebral metastasis was noted on the brain CT scan. Early recognition of intracranial tumor as the cause of parkinsonism is important for the management of this type of movement disorder. Moreover, brain CT scanning plays an important role in the differential diagnosis of patients with parkinsonian symptoms.- - - - - - - - - - ranking = 28836.400383257keywords = rigidity (Clic here for more details about this article) |
7/38. 5: movement disorders I: parkinsonism and the akinetic-rigid syndromes.The major features of akinetic-rigid syndromes are bradykinesia (small, slow movements), rigidity and tremor, often summarised as "parkinsonism". Approximately 80% of akinetic-rigid syndromes are due to Parkinson's disease. Treatment of Parkinson's disease should be determined by level of disability and handicap. The combination of levodopa and peripheral dopadecarboxylase inhibitor is the most efficacious symptomatic treatment for Parkinson's disease. Motor fluctuations appear after 2-3 years of levodopa treatment, and affect at least 50% of patients after five years. Surgery can relieve refractory tremor and dyskinesias, but does not alter disease progression or need for drug therapy.- - - - - - - - - - ranking = 14418.200191629keywords = rigidity (Clic here for more details about this article) |
8/38. Dopa-responsive parkinsonism secondary to right temporal lobe haemorrahage.A 46-year-old man developed a symmetrical parkinsonian syndrome 7 weeks after large right temporal intracerebral haemorrhage resulting from a ruptured arteriovenous malformation. His signs included bradykinesia, rigidity, start hesitation, and poor postural reflexes, without a resting tremor. He also had signs of a Parinaud's syndrome. Computed tomography and magnetic resonance imaging of the brain demonstrated changes in the right temporal lobe associated with the haemorrhage but no abnormality of the basal ganglia or midbrain. levodopa therapy produced a dramatic improvement within a few days of commencement. We postulate that the parkinsonism resulted from midbrain compression secondary to transtentorial herniation. Although parkinsonism is a rare complication of lobar intracerebral haemorrhage, it is important to recognise as it may be potentially treatable.- - - - - - - - - - ranking = 14418.200191629keywords = rigidity (Clic here for more details about this article) |
9/38. clozapine withdrawal symptoms in a Parkinson's disease patient.Abrupt clozapine withdrawal can cause rebound psychosis and severe somatic symptoms in psychiatric patients. We report on the case of an advanced Parkinson's disease patient who developed myoclonus, tremor, rigidity, hyperreflexia, and stupor after abrupt clozapine withdrawal. The patient's symptoms resolved with treatment with cyproheptadine. This clinical picture suggests serotonergic rebound as an explanation for the patient's symptoms, although other pharmacological mechanisms are possible. clozapine should be gradually withdrawn over a period of 1 to 2 weeks when possible, and abruptly discontinued only when necessary.- - - - - - - - - - ranking = 14418.200191629keywords = rigidity (Clic here for more details about this article) |
10/38. risperidone-responsive segmental dystonia and pallidal deep brain stimulation.A 67-year-old man with risperidone-responsive segmental dystonia underwent bilateral deep brain stimulation (DBS) of the globus pallidus internus. Prospectively, the authors assessed the Burke-Fahn-Marsden dystonia Rating Scale in medication (M) and stimulation (S) "on"/"off" states. With DBS at 9 months, the score improved by 86% to 8.5 in M-"on"/S-"on" and 12.5 in M-"off"/S-"on." Studies of the effects of DBS and concomitant medication may be warranted in selected patients treated by DBS for dystonia.- - - - - - - - - - ranking = 1keywords = state (Clic here for more details about this article) |
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