1/186. technetium-99m-HmPAO brain SPECT in infantile Gaucher's disease.The authors report serial technetium-99m hexamethylpropylene-amine-oxime brain single photon emission computed tomography (SPECT) findings in two infants with Gaucher's disease type 2. Detailed neurologic and laboratory examinations, including bone marrow biopsies and enzymatic assays, were described. Serial brain magnetic resonance imaging studies in one patient illustrated the progressive cerebral atrophy in the frontal and temporal lobes. The SPECT in both cases demonstrated positive findings of initial scattered hypoperfusion, with extending to hypoperfusion of the entire cerebrum after 4 months of clinical deterioration. These changes in the SPECT findings may reflect progressive degeneration of the cerebrum in Gaucher's disease type 2. brain SPECT may provide useful information on cerebral flow and metabolic distribution corresponding to the neurologic deficits of neuronopathic Gaucher's disease.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
2/186. Cognitive deterioration associated with focal cortical dysplasia.Surgery for an area of focal cortical dysplasia in a critical region is reported in a right-handed female manifesting intractable focal epilepsy and verbal cognitive deterioration. She developed the first seizure at 2 years of age and was treated with phenytoin and zonisamide, with good control until 10 years of age. Although seizures did not occur at 9 years of age, she manifested dyscalculia, right-left disorientation, and finger agnosia, and N-isopropyl-p-iodoamphetamine single-photon emission computed tomography (SPECT) revealed focal hypoperfusion in the left parietal lobe. At 11 years of age, she developed regular nocturnal seizures and gradually lost the ability to understand the meaning of sentences. Verbal IQ declined from 94 to 63, and the area of hypoperfusion detected by interictal N-isopropyl-p-iodoamphetamine SPECT spread over the left parietotemporal lobes. magnetic resonance imaging revealed focal cortical dysplasia mainly in the left parietal lobe, and ictal technetium-99m-ethyl cysteinate dimer SPECT images demonstrated an area of hyperperfusion around the focal cortical dysplasia, including the left precentral gyrus. Because of the overlap between the epileptogenic and functional cortex, the authors concluded that cortical resection, including focal cortical dysplasia, was inappropriate in this patient.- - - - - - - - - - ranking = 3keywords = lobe (Clic here for more details about this article) |
3/186. posterior leukoencephalopathy syndrome may not be reversible.The association of an acute reversible encephalopathy with transient occipital lobe abnormalities on imaging studies is well known. This condition has been called reversible posterior leukoencephalopathy syndrome. The clinical presentation usually includes seizures, headache, altered mental status, and blindness, often associated with hypertension and immunosuppressants. The authors discuss a two-year-old male with down syndrome who presented 2 months after allogeneic bone marrow transplantation with severe oculogyric crisis, without other complaints. The patient was being treated for hypertension and was receiving cyclosporine for prophylaxis of graft-vs-host disease. A computed tomography scan of the head revealed marked bilateral lucencies mainly involving the white matter of the occipital lobes, with a few foci of punctate hemorrhage. The condition improved when cyclosporine was discontinued, but an area of leukomalacia was identified on follow-up magnetic resonance imaging. To the authors' knowledge, oculogyric crisis as a presentation of reversible posterior leukoencephalopathy has not been previously described. Recognizing this association is important, because patients receiving cyclosporine are often receiving other medications that can potentially cause dystonic eye movements, possibly leading to a delay in diagnosis and treatment, which can result in an irreversible neurologic deficit.- - - - - - - - - - ranking = 2keywords = lobe (Clic here for more details about this article) |
4/186. Interictal change in cardiac autonomic function associated with EEG abnormalities and clinical symptoms: a longitudinal study following acute deterioration in two patients with temporal lobe epilepsy.The purpose of the present study was to investigate the ictal and interictal changes in cardiac autonomic function (CAF), and the relationship between the interictal change in CAF to the electroencephalogram (EEG) and clinical findings. In two patients with temporal lobe epilepsy (TLE) showing acute deterioration, a quantitative evaluation of their interictal CAF based on heart rate variability and their EEG using spectral analysis was conducted, and the findings compared with repeated clinical evaluations during the recovery period. The ictal heart rate changes and their temporal relationship to ictal discharge were investigated using simultaneous EEG/electrocardiogram (ECG) monitoring in one of the patients. Interictal parasympathetic function was decreased during the period of acute deterioration, but was increased in association with improvements in the EEG and clinical findings. In contrast, the sympathetic function showed no specific changes. The ictal discharges were preceded by a brief bradycardia, with a long delay of up to 40s. The results demonstrated that this decrease in parasympathetic function was closely related to the interictal changes in central nervous system function. On the other hand, the ictal discharges in one of the patients were thought to have caused a transient elevation of parasympathetic function. It is strongly suggested that patients with TLE have interictal as well as ictal changes in CAF that are mediated mainly through the parasympathetic nervous system.- - - - - - - - - - ranking = 5keywords = lobe (Clic here for more details about this article) |
5/186. Pituitary involvement by Wegener's granulomatosis: a report of two cases.We describe two cases of pituitary involvement by Wegener's granulomatosis. At initial presentation, or during subsequent disease "flares," a pattern of pituitary abnormality was suggested. During periods of remission, we found the pituitary returned to a nearly normal appearance. Loss of the normal posterior pituitary T1 hyper-intensity matched a clinical persistence of diabetes insipidus, suggesting there is permanent damage to this structure by the initial disease process.- - - - - - - - - - ranking = 107.07169207246keywords = pituitary (Clic here for more details about this article) |
6/186. An ACTH-producing pituitary carcinoma developing Cushing's disease.An autopsy case of an ACTH-producing pituitary carcinoma in a 59-year-old man who developed Cushing's disease is reported. The surgically removed pituitary tumor was diagnosed as chromophobe adenoma, however, pulmonary metastases appeared 2 years after the operation. autopsy revealed a residual pituitary tumor in the sella turcica with systemic metastases to the lungs, liver, pulmonary lymph nodes, hypothalamus, dura mater, and the subarachnoid space of the midbrain and spinal cord. immunohistochemistry revealed ACTH positivity in the tumor cells. Further immunohistochemical study showed positive high expression of Ki-67 in the tumor removed at surgery as well as in the autopsied tumor. Ki-67 labeling index provided valuable information about the invasive and proliferative potential compared to noninvasive benign pituitary adenoma.- - - - - - - - - - ranking = 214.14338414492keywords = pituitary (Clic here for more details about this article) |
7/186. Rapid evolution of chronic viral hepatitis into hepatocellular carcinoma after beta-interferon treatment.A 62-year-old man, affected by Chronic Active hepatitis (discovered in 1993) and treated with interferon, referred to our department with increased abdominal volume, persistent abdominal pain, continuous-remittent fever and jaundice. CT scan of the liver revealed a hypodense, not capsulated, infiltrative, solid formation in the right lobe. US guided biopsy showed multinucleated giant cells, with eosinophilic cytoplasm and pleomorphism of the nuclei, arranged in several thick trabecula lined by endothelial cells or formed bile containing acini. In our case, the rapid evolution of chronic viral hepatitis towards HCC calls for a careful evaluation of the role of IFN therapy, since this drug is widely used in chronic liver diseases.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
8/186. Progressive dysarthria. case reports and a review of the literature.Two patients presenting with progressive dysarthria as the single initial manifestation of a neurodegenerative condition are described. The nature of the dysarthria as well as the additional symptoms that developed in the course of the disorder are very different in these two cases. Nevertheless, neuroimaging findings are strikingly similar and suggest bilateral involvement of posterior inferior frontal lobe structures, mainly in the dominant cerebral hemisphere. The clinical syndrome of these patients can therefore be considered an example of frontotemporal degeneration presenting without dementia or compartmental alteration, at least in the early stages. This broadens the clinical spectrum of frontotemporal degeneration and demonstrates the need for a syndromal subclassification of this nosological entity.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
9/186. Primary progressive aphasia: a case report.We report a 69-year-old male patient whose motor aphasia started at the age of 61. The language disability remained isolated and progressed over a period of eight years without any additional cognitive deficits. Computed tomography (CT) and magnetic resonance imaging (MRI) showed moderate cortical atrophy with frontal dominance. Single photon emission tomography (SPECT) showed hypoperfusion in the frontotemporoparietal region, positron emission tomography (PET) demonstrated a global cortical reduction of glucose utilization with a lesser decrement in the occipital lobes. The clinical symptoms and the neuropsychological findings fit the diagnosis of primary progressive aphasia.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
10/186. epilepsy surgery in children with pervasive developmental disorder.Pervasive developmental disorder (PDD) is occasionally associated with medically intractable complex partial seizures. The outcome of PDD was explored in three males and two females who underwent epilepsy surgery at 32 months to 8 years of age (mean = 4 years) after onset of epilepsy at 1 week to 21 months of age (mean = 11 months). Four children had temporal lobe resections (three right, one left; two for focal cortical dysplasia, and two for tumors), and one had a right temporoparieto-occipital resection (for focal cortical dysplasia). Each child underwent repeated evaluations by a pediatric neuropsychologist and psychiatrist. Fourteen to 47 months (mean = 23 months) after operation, one child with persistent seizures had moderate developmental and behavioral improvement, three children (two seizure free, one with rare staring spells) had mild developmental and behavioral improvement, and the remaining child (seizure free) experienced a worsening of her PDD. The four children with mild-to-moderate improvement in postoperative cognitive and behavioral development still demonstrated persistent delay. Cognitive gains were confirmed by neuropsychologic testing in the oldest patient but were not reflected in test results from the three younger children, who had more modest improvement. The child with worsening of her PDD had cognitive and emotional deterioration to babbling, echolalia, aggressiveness, decreased social interaction, and increased mouthing of objects beginning several months postoperatively. These results suggest that families should be counseled that PDD symptoms in children with focal epileptogenic lesions may or may not improve after epilepsy surgery, even if the surgery is successful with respect to seizure control.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
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