technetium-tc-99m-bicisate and Syndrome

To describe the ictal technetium-99 m-ECD SPECT findings in polymicrogyria syndromes (PMG) during epileptic seizures.. We investigated 17 patients with PMG syndromes during presurgical workup, which included long-term video-electroencephalographic (EEG) monitoring, neurological and psychiatry assessments, invasive EEG, and the subtraction of ictal-interictal SPECT coregistered to magnetic resonance imaging (MRI) (SISCOM).. The analysis of the PMG cortex, using SISCOM, revealed intense hyperperfusion in the polymicrogyric lesion during epileptic seizures in all patients. Interestingly, other localizing investigations showed heterogeneous findings. Twelve patients underwent epilepsy surgery, three achieved seizure-freedom, five have worthwhile improvement, and four patients remained unchanged.. Our study strongly suggests the involvement of PMG in seizure generation or early propagation. Both conventional ictal single-photon emission computed tomography (SPECT) and SISCOM appeared as the single contributive exam to suggest the localization of the epileptogenic zone. Despite the limited number of resective epilepsy surgery in our study (n = 9), we found a strong prognostic role of SISCOM in predicting surgical outcome. This result may be of great value on surgical decision-making of whether or not the whole or part of the PMG lesion should be surgically resected.

Topics: Adolescent; Brain; Child; Cysteine; Epilepsy; Female; Humans; Magnetic Resonance Imaging; Male; Malformations of Cortical Development; Organotechnetium Compounds; Radiopharmaceuticals; Subtraction Technique; Syndrome; Tomography, Emission-Computed, Single-Photon

The MURCS association is a rare, nonrandom association of müllerian duct aplasia, renal aplasia and cervicothoracic somite dysplasia. The etiology is unknown. Although it is usually a sporadic disorder, familial cases with uterovaginal anomalies have been reported. Occasionally, it may be accompanied by abnormalities involving various other organs or systems. Malformations related to the central nervous system are very rare and the presence of seizures has not been reported previously. We present a 26-year-old female with MURCS association who had late onset, drug resistant partial seizures presumably originating in the frontal lobe.

Topics: Adult; Cysteine; Drug Resistance; Epilepsy, Frontal Lobe; Female; Humans; Kidney Diseases; Magnetic Resonance Imaging; Mullerian Ducts; Organotechnetium Compounds; Radiography; Radiopharmaceuticals; Stellate Ganglion; Syndrome; Tomography, Emission-Computed, Single-Photon

We report a functional neuroimaging study of a 43-year-old woman with Nasu-Hakola disease (NHD). Regional cerebral blood flow (rCBF) images were measured with technetium-99m ethyl cysteinate dimer single photon emission computed tomography (SPECT). rCBF was decreased in the bilateral frontal lobes and thalamus. This finding was consistent with the known underlying neuropathology in patients with NHD. Brain SPECT is useful for demonstrating the pathophysiologic brain region in patients with NHD.

Topics: Adult; Brain; Cerebrovascular Circulation; Chromosome Disorders; Cysteine; Dementia, Vascular; Female; Humans; Lipodystrophy; Memory Disorders; Organotechnetium Compounds; Osteochondrodysplasias; Radionuclide Imaging; Radiopharmaceuticals; Syndrome

We present a 61-year-old male patient with progressive dementia. A brain SPECT with Tc-99m-bicisate was performed for confirmation of clinically suspected Alzheimer-dementia. At the time of the SPECT-investigation marked apraxia and aphasia besides severe dementia were present. Electrophysiological as well as anatomical neuroimaging findings showed non-diagnostic alterations. SPECT revealed distinct perfusion defects, which made Alzheimer Dementia unlikely. The further course of the patient was determined by rapidly progressive deterioration with development of akinetic mutism. Thereafter, increased levels of neuron-specific enolase as well as 14-3-3 proteins were found in the cerebro-spinal fluid (CSF). The patient finally died with signs of cerebral decortication. Due to the clinical course and the CSF-findings the patient's final diagnosis was Creutzfeldt-Jakob-disease, nevertheless no autopsy was performed. The presented case report underscores the clinical utility of perfusion brain SPECT in the differential diagnosis of dementias.

Topics: Brain; Cysteine; Dementia; Diagnosis, Differential; Disease Progression; Humans; Male; Middle Aged; Mutism; Organotechnetium Compounds; Radiopharmaceuticals; Syndrome; Tomography, Emission-Computed, Single-Photon