Rapidly progressive dementia.
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Biomedical subjects
Publications and source records attributed to N Heye.
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We report the case of a 40 year-old man with a severe lesion of the anterior rami of the left spinal nerves L5 and S1 who showed hypertrophy of the leg and atrophy of the intrinsic foot and gluteal muscles. In the biopsy of the hypertrophied gastrocnemius muscle, perivascular inflammatory infiltrates were observed, apart from atrophied and hypertrophied muscle fibres. Electromyography revealed no pathologic spontaneous activity but chronic neurogenic changes. The precise site of the lesion was predicted by electrophysiologic investigations. The lesion was caused by two perineural cysts in the region of the upper sacral plexus, as demonstrated by MRI and CT of the small pelvis and confirmed at operation. Three years earlier, when almost only L5 muscles were affected, an intervertebral disc prolapse L5/S1 had been suspected on myelography and CT but could not have been confirmed at operation.
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The Motor Performance Test Series (MPTS) is widely used for treatment control in Parkinson's disease (PD). To elucidate the possible influence of depression on the fine motor skills in PD, 54 patients with idiopathic PD were investigated with the MPTS. 27 patients with major intensity of depression were compared to 27 age and motor disability matched patients with minor symptoms of depression, evaluated by the Zung depression scale. As determined by the subtest aiming, a significant lower ability for precise, quick complex arm-hand movements in depressed Parkinsonian patients was found. This result may be explained partly by motivation deficits in depressed patients with PD. On the other hand impairment of special motor loops including frontal lobe projections to specific thalamic subnuclei or to the caudate nucleus may cause disturbances of the subtest aiming in depressed Parkinsonian patients. On the basis of these findings impaired aiming may be explained by diminished ability for complex, semivoluntary movements in depressive Parkinsonian patients. The influence of psychiatric comorbidity on MPTS subtest aiming has to be considered in further therapy studies using evaluation of motor deficits by MPTS.
INTRODUCTION: Etiology of depression in Parkinson's disease (PD) is associated with serotonergic dysfunction. Previous studies, supporting this hypothesis, were performed on patients treated with antiparkinsonian drugs. To eliminate the influence of parkinsonian drug therapy and to elucidate significance of different biochemical pathways in PD associated with depression we determined levels of biogenic amines in cerebrospinal fluid (CSF) of 26 untreated "de novo" Parkinsonian patients. MATERIAL AND METHODS: Patients were scored with the Hamilton depression scale (HD) and subdivided into groups with HD score > or = 18 and HD score < 18. Diagnosis of depression was made according to DSM III R. Both groups were matched for age and motor disability. RESULTS: In both groups no significant differences appeared between CSF levels of dopamine, noradrenaline, 3,4-dihydroxyphenylacetic acid, homovanillic acid, 3-methoxy-4-hydroxyphenylglycol and 5-hydroxyindole acetic acid, determined by high-performance liquid chromatography. DISCUSSION: In contrast to previous studies on treated Parkinsonian patients no sign of altered serotonin metabolism especially in context with severity of depression in early stages of PD was found. Due to our results, we suggest, that biochemical markers of depression in CSF of PD may be influenced by antiparkinsonian therapy and that depression in PD may respond to serotonin reuptake inhibitors mainly in later stages of PD.
BACKGROUND AND PURPOSE: We investigated the distribution and frequency of microthromboemboli (MTE) in acute infarcts in humans and determined whether MTE in the contralateral circulation resulted in histological changes. METHODS: Forty patients dying within the first month after unilateral infarct were investigated. Infarct etiology was determined mainly on the pathological findings. Whole brain sections from the region of maximal necrosis were stained for fibrin. Fibrin-containing MTE were transferred to a schematic drawing and counted. Sections from 20 patients without infarcts served as controls. RESULTS: Infarct sections had significantly more MTE than controls. Infarcts of thrombotic (n=6) and thromboembolic (n=21) origin had more MTE than infarcts of embolic origin (n=13). Thromboembolic infarcts had the highest number of MTE within the region assumed to be the ischemic penumbra, other arterial territories, and the contralateral hemisphere. Patients with large infarcts and those with short clinical courses had a higher number of MTE. Sixteen patients had recent micronecroses in the contralateral hemisphere. CONCLUSIONS: There seems to be a pattern of MTE in acute infarcts that is dependent on cause, size, and clinical duration. Our findings of contralateral micronecroses emphasize that acute infarcts may result in more widespread cerebral injury than clinically expected. Given the many variables influencing stroke and death in humans, the results have to be interpreted with caution.
STUDY DESIGN: This is a report of a patient with acute spinal epidural hematoma taking aspirin, 250 mg/day. OBJECTIVE: To describe the association of spinal epidural hematoma and aspirin. SUMMARY OF BACKGROUND DATA: In about 50% of patients, the cause of acute spinal epidural hematoma is obscure. Analysis of possible mechanisms is necessary. METHODS: Diagnostic lumbar puncture, suboccipital myelography, and surgical removal of the hematoma was performed. RESULTS: Increased bleeding was noticed during these procedures. Bleeding time was normal 6 days after discontinuance of aspirin. CONCLUSION: Because aspirin is widely used, its role in causing spinal epidural hematoma will remain conjectural. For the present patient, however, it was postulated that aspirin had a major impact on the extent of the epidural bleeding. There should be awareness of a possible link between aspirin and spinal epidural hematoma.
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The purpose of this open study was to find out whether botulinum toxin A (BTX-A) relieves the signs and symptoms of chronic limb spasticity. The study comprised 40 patients, aged 12-82 years, with moderate to severe spasticity of the upper (13) or lower limbs (27) refractory to conventional physical and medical treatments. Outcome measures were clinical and blinded videotape assessments of spasticity and motor function. Electromyography guided BTX-A injections were given in one or two sessions at total doses averaging 175 U in the upper limb (range 70-270 U) and 221 U in the lower limb (range 100-500 U). Thirty four patients (85%) derived worthwhile benefit, with improved limb posture and increased range of passive motion in 31, pain reduction in 28 of 31 with pain, and improved function in 16. Side effects were limited to local and usually mild discomfort from the injections (19), symptomatic local weakness (one), and local infection (one). Preliminary experience indicates that BTX-A is a promising adjunctive treatment for selected patients with spasticity.
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Wernicke's encephalopathy (WE) is a thiamine deficiency disorder and is characterized clinically by the triad of ocular abnormalities, ataxia and disturbances of consciousness. We report on 3 patients with WE, of whom 2 had insufficient thiamine substitution. In the first patient symptoms disappeared during thiamine substitution. In the second patient acute WE was the terminating event in the sequence of parenteral nutrition, lactic acidosis and cardio-pulmonary decompensation. Possibly due to hereditary deficits WE developed in the third patient despite sufficient thiamine substitution. Attention to thiamine deficiency should be paid in all patients with history of alcoholism, malnutrition, malabsorption, tumors, inflammation, other severe diseases and in parenteral hyperalimentation. In order to prevent WE thiamine should be substituted with at least 100 mg/day i.v. or i.m.
The case of a 67-year-old woman with terminal renal insufficiency, who developed extensive encephalopathy with predominant involvement of the white matter is reported. The encephalopathy was the consequence of preexisting hypertensive alterations, acidosis, hypoxia, ischemia, bacteremia and varicella-zoster meningoencephalitis. The vasculitic alterations associated with meningoencephalitis had a major influence on the development and the extent of the leukoencephalopathy.
We report on four patients with a histologically proven diagnosis of arteritis temporalis and clinical and/or neuroradiological evidence of severe focal cerebral ischemia due to intracranial vasculitis. While one patient suffered from a transient ischemic attack, CCT and MRI scans of the other patients showed multiple lacunar infarctions, combined with territorial infarctions in two cases. Necropsy in one patient demonstrated generalized giant cell vasculitis in large and small cerebral vessels. We suppose that the cerebral involvement was provoked by insufficient steroid therapy of arteritis temporalis in two patients. In one case, remission could be achieved by a combination of high-dose steroids and cyclophosphamide; one further patient remitted under lower steroid dosage. Steroid therapy was ineffective in two patients, one of whom died due to secondary complications. We conclude that central nervous system affection is a rare but dangerous complication of arteritis temporalis and may present as cerebral micro- and macroangiopathy.
PURPOSE: We used MR imaging to analyze retrospectively the pattern of hyperostosis occurring concomitantly with recurrent sphenoid wing meningiomas. METHODS: Bone involvement was compared in 12 corresponding CT and MR studies of 10 female patients with sphenoid wing meningiomas recurrence after earlier surgical treatment. Four of these had histologically confirmed meningiomatous infiltration of the bone. RESULTS: All patients had CT findings of localized hyperostosis of parts of the sphenoid wings. MR revealed inhomogeneous areas of slightly increased signal intensity in hyperostotic bone on T2-, proton density- and T1-weighted sequences. In nine of 10 patients, segments of the hyperostotic bone showed different degrees of gadolinium enhancement. CONCLUSIONS: Because earlier studies have revealed high incidences of meningiomatous bone infiltration in sphenoid wing meningiomas, and because infiltration was confirmed in four of our patients, we postulate that the gadolinium enhancement in the area of hyperostosis may be related to meningiomatous bone infiltration.