1/6. Right-sided anarchic (alien) hand: a longitudinal study.A patient with a bilateral frontal vascular lesion, encroaching upon the mesial cortex on the left and damage of the corpus callosum showed the 'alien hand' phenomenon on the right. The various hypotheses as to the nature of the lesion for the alien hand phenomenon to appear are discussed. It is proposed that an acute clinical condition, following a lesion of the corpus callosum only, should be differentiated from a chronic condition resulting from the additional fronto-mesial lesion.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/6. Dysequilibrium/ataxic diplegia with immunodeficiency.A girl with purine nucleoside phosphorylase (PNP) deficiency is described. The nature of the motor disorder is similar to other children since found to have PNP deficiency. It is suggested that the diagnosis be considered in any child with unexplained dysequilibrium/ataxic diplegia. Other previously unreported features are intracytoplasmic neutrophil inclusion bodies and an improvement in the neutropenia after intravenous immunoglobulin.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/6. Thalamic astasia: inability to stand after unilateral thalamic lesions.Inability to stand in the absence of motor weakness or marked sensory loss is usually considered to reflect midline cerebellar disease. However, the 15 patients reported here had astasia related to unilateral thalamic lesions, documented by autopsy and computed tomography in 2 patients and by computed tomography in 13. The lesions, including infarction (6), hemorrhage (7), and tumor (2), involved primarily the superoposterolateral portion of the thalamus, but spared the rubral region. Alert, with normal or near-normal strength on isometric muscle testing and a variable degree of sensory loss, the patients could not stand and 7 of them could not sit up unassisted. They fell backwards or toward the side contralateral to the lesion. They appeared to have a deficit of overlearned motor activity of an axial and postural nature. In the vascular cases, the deficit improved in a few days or weeks. However, these patients had a tendency to sustain falls during the rehabilitation period.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/6. Psychomotor seizures, arterio-venous malformation and the olfactory reference syndrome. A case report.A case history is reported of a 44-year-old man with a 6-year history of psychomotor seizures. For the past year he had described persistent olfactory hallucinations of an unpleasant nature which he referred to himself. In many respects these symptoms conform to the pattern observed in the olfactory reference syndrome, a recently described but apparently quite discrete psychiatric disorder; there were, however, certain atypical features. At a later stage unilateral anosmia was noted. Radiological examination then demonstrated an arterio-venous malformation in the right frontal lobe. The relationship between the malformation and the psychomotor seizures, and the implications that each has for the development of an olfactory reference syndrome are fully discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/6. Nonverbal perceptual organization output disability and schizophrenia spectrum symptomatology.patients with neurodevelopmental syndromes often receive numerous psychiatric diagnoses before the true nature of their disorder becomes apparent. We present a case in which the neuropsychological evaluation played a significant role in reconceptualizing a patient who had received, at various times, diagnoses of schizotypal personality disorder, schizoaffective disorder, and schizophrenia. The identification of specific cognitive deficits in executive functioning, perceptual organization, visual-spatial problem solving, and abstraction led to: 1) a diagnosis of nonverbal perceptual-organization-output disabled; 2) the adoption of a rehabilitative treatment model; and 3) a greater understanding of the way in which the patient's social deficits represented adaptations to her cognitive impairments. research data and theoretical models relating cognitive deficits to psychiatric symptoms are discussed, and evidence is presented that schizophrenia and certain neurodevelopmental syndromes may share commonalities of pathophysiology. Diagnostic issues arising from similarities between these disorders are discussed. It is suggested that direct comparisons between these groups can aid in clarifying the specific nature of cognitive deficit-symptom relationships, as well as leading to improvements in the understanding, diagnosis and treatment of schizophrenic and neurodevelopmental syndromes.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
6/6. Serial MRI and neurophysiological studies in late-infantile Krabbe disease.We report serial clinical, radiological, and neurophysiological findings of a patient with late-infantile Krabbe disease. At age 13 months, the patient was hospitalized for sudden stiffness and irritability and a diagnosis of spastic diplegia was made. At age 24 months, he was readmitted because of further psychomotor deterioration; neurologically, he manifested severe spastic tetraplegia with optic atrophy. MRI disclosed diffuse high intensity in the cerebral white matter on T2-weighted images. Nerve conduction velocity and evoked potential studies were markedly abnormal, as were the EEG and the EMG. Assay of galactocerebroside beta-galactosidase activity in leukocyte culture disclosed a marked deficiency of the enzyme, confirmatory of the diagnosis of late-infantile Krabbe disease. Serial MRI and neurophysiological studies performed every 6 months for 18 months demonstrated the progressive nature of the disorder, correlating with the clinical deterioration.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |