Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Jul 2004
Case ReportsSpectroscopy and serial diffusion MR findings in hGH-Creutzfeldt-Jakob disease.
This report describes the magnetic resonance imaging (MRI) findings in two patients fulfilling the diagnostic criteria for human growth hormone Creutzfeldt-Jakob disease, who initially had pronounced bilateral DWI/FLAIR (diffusion weighted imaging/fluid attenuated inversion recovery) hyperintensities in the basal ganglia, with decreased apparent diffusion coefficient (ADC) values (range, 58-82% of normal). MRI spectroscopy, obtained in one case, showed decreased N-acetyl aspartate/creatine (NAA/Cr) ratio in the atrophic vermis (0.79; normal: mean, 1.20; SD, 0.13), despite the lack of DWI/FLAIR signal changes, whereas NAA/Cr was normal in the putamina (1.6; normal: mean, 1.56; SD, 0.17), despite striking DWI signal changes and decreased ADC values (60% of normal). Serial DWI, obtained in the other case, showed a progressive disappearance of DWI hypersignal of the basal ganglia replaced by pronounced atrophy. Data from these two patients suggest that restricted diffusion associated with a normal NAA value might indicate spongiform changes of still viable cells, and that any subsequent regression of the DWI signal changes, atrophy, or decreased NAA values could be related to progressive neuronal death.
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J. Neurol. Neurosurg. Psychiatr. · Jul 2004
Case ReportsAdult onset ornithine transcarbamylase deficiency: an unusual cause of semantic disorders.
Ornithine transcarbamylase deficiency (OTCD) is the most common urea cycle disorder. This condition usually presents in neonates or children. ⋯ After recovery from the coma, she presented very unusual neuropsychological disorders involving memory and the meaning of certain words, suggesting a semantic deficit. The discovery of OTCD in adulthood is rare and the neuropsychological consequences may be unique.
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J. Neurol. Neurosurg. Psychiatr. · Jul 2004
Case ReportsClinically evidenced unilateral dissociation of saccades and pursuit eye movements.
A patient affected by an ischaemic lesion of the right medial thalamic nucleus presented with a uniocular dissociation of upward vertical saccades and pursuit movement, with absent upward vertical saccades in the left eye. Clinical observations were confirmed by magnetic field scleral search coils analysis. During the vertical eye movement the patient denied any diplopia, thus suggesting a transient visual suppression in the left eye.
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J. Neurol. Neurosurg. Psychiatr. · Jul 2004
Survival and quality of life outcome after mechanical ventilation in elderly stroke patients.
Mortality is high and functional outcome poor in mechanically ventilated stroke patients. In addition, age >65 years is an independent predictor of death at 2 months among these patients. Our objective was to determine survival rates, functional outcome, and quality of life (QoL) in stroke patients older than 65 years requiring mechanical ventilation. ⋯ Although only 40% of elderly patients intubated in the acute phase of stroke survived at least 6 months, one in four survivors recovered to a good functional outcome with a reasonable QoL. Elderly stroke patients need to be selected carefully for intensive care treatment, but elective intubation to allow diagnostic procedures should not be withheld primarily based on their age.
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J. Neurol. Neurosurg. Psychiatr. · Jul 2004
Schizophrenia-like psychosis arising de novo following a temporal lobectomy: timing and risk factors.
To clarify risk factors for the development of schizophrenia-like psychotic disorders following temporal lobectomy, and to explore the possibility that the early postoperative period is a time of high risk for the onset of such chronic psychotic disorders. ⋯ Temporal lobectomy for medically intractable epilepsy may precipitate a schizophrenia-like psychosis. Patients with bilateral functional and structural abnormalities, particularly of the amygdala, may be at particular risk for the development of such psychoses.