technetium-tc-99m-bicisate and Spasms--Infantile

We studied the clinical, neuroradiological and EEG characteristics of patients with infantile spasms (IS) who showed focal features to reveal their long-term prognoses and treatment responses.. Subjects included 69 patients with IS who consecutively visited our hospital. We tentatively classified the subjects into focal IS (fIS) and diffuse WS (dIS) groups based on the presence and absence of more than two of the following findings, respectively: (1) epileptic spasms (ES) that were asymmetric, (2) a focal epileptic EEG abnormality, (3) a lateralized neurological abnormality, (4) a focal brain MRI and (5) a focal SPECT abnormality.. We found 23 cases with fIS and 46 cases with dIS. ES responded more frequently in fIS than dIS group (100% vs. 80%; P=0.02) to the initial ACTH trial although the subsequent seizure relapse occurred more frequently in fIS than dIS group (74% vs. 38%; P=0.0006). The second course of ACTH trial brought a short as well as long-term remission in both groups (6/8 cases vs. 5/6 cases). Later in the clinical course, the fIS patients tended to display a focal epileptic EEG abnormality and to develop focal seizures. In our series, approximately one-third of patients with fIS later showed either only a focal epileptic EEG abnormality, a focal epileptic EEG abnormality with focal seizures, or bilateral asymmetric EEG foci with disabling seizures, respectively.. It is useful to classify patients with IS into fIS and dIS groups based on various lateralizing signs because the classification provides practical information regarding the long-term outcome and treatment strategy.

Topics: Adrenocorticotropic Hormone; Brain; Brain Waves; Chi-Square Distribution; Child, Preschool; Cysteine; Electroencephalography; Female; Fluorodeoxyglucose F18; Functional Laterality; Humans; Infant; Infant, Newborn; Longitudinal Studies; Magnetic Resonance Imaging; Male; Organotechnetium Compounds; Positron-Emission Tomography; Spasms, Infantile; Tomography, Emission-Computed, Single-Photon; Tomography, X-Ray Computed

To clarify the mechanism of clustered spasms in West syndrome (WS), we examined ictal SPECT and EEG, as well as those during the interictal period, in three patients with symptomatic WS who had apparent focal cerebral lesions. For ictal SPECT and EEG, we monitored the patients with EEG, and as soon as we noticed the occurrence of clustered spasms clinically and electroencephalographically, [(99m)Tc]ECD was injected intravenously and flushed thoroughly with saline. In these three patients, regional cerebral blood flow (rCBF) increased during ictus and decreased during the interictal period in the area that coincided with the focal cerebral lesion recognized by CT/MRI. The ictal hyperperfusion of bilateral basal ganglia was also detected in two of the three patients. The ictal EEG showed a diffuse slow wave complex corresponding to a clinical spasm. The sharp waves that preceded the diffuse slow wave complex and the spasm appeared in the same area in which rCBF increased during ictus. None of the patients showed partial seizure before or after clustered spasms clinically or electroencephalographically during the ictal SPECT study. Secondary generalization from a cerebral focus may explain the mechanism of spasms in these patients with WS: focal cortical discharge may primarily generate clustered spasms and trigger the brainstem and basal ganglia structures to produce spasms.

Topics: Cerebral Cortex; Cerebrovascular Circulation; Child; Child, Preschool; Cysteine; Electroencephalography; Female; Humans; Infant; Infant, Newborn; Male; Organotechnetium Compounds; Spasms, Infantile; Tomography, Emission-Computed, Single-Photon

To investigate spatial and temporal cortical activity during clusters of naturally occurring epileptic spasms in patients with West syndrome (WS) by using multichannel near-infrared spectroscopy (mNIRS).. Conventional magnetic resonance imaging (MRI) and interictal and ictal single-photon emission computed tomography (SPECT) were carried out in three patients with WS. Thereafter, cortical hemodynamics during naturally occurring epileptic spasms were measured by mNIRS with simultaneous video/electroencephalographic (EEG) monitoring.. Ictal SPECT revealed multiple hyperperfused areas within the cortex. With the use of mNIRS, an increase in regional cerebral blood volume (CBV) was observed in these areas, which is representative of cortical activation. The increase in CBV was accompanied by an increase in the concentrations of both oxy- and deoxyhemoglobin. The following heterogeneous regional changes in CBV during ictus were observed: (a) transient increases that were synchronized with spasms; (b) a gradual increase during an ictal event that fluctuated in synchrony with spasms; and (c) a combination of transient and gradual increases. An increase in regional CBV occurred in multiple areas that were activated either simultaneously or sequentially during an ictal event. Topographic changes in CBV were closely correlated with the phenotype of the spasm.. During ictal events, multiple cortical areas were activated simultaneously or sequentially. The pattern of cortical activation closely affected the phenotype of the spasm, which suggested that the cortex was involved in the generation of spasms.

Topics: Brain Mapping; Cerebral Cortex; Cysteine; Electroencephalography; Female; Gestational Age; Humans; Infant; Magnetic Resonance Imaging; Male; Monitoring, Physiologic; Organotechnetium Compounds; Regional Blood Flow; Spasms, Infantile; Spectroscopy, Near-Infrared; Tomography, Emission-Computed, Single-Photon; Videotape Recording

We evaluated the ictal and interictal single photon emission computed tomography (SPECT) of 9 patients with West syndrome (WS). In this group, we noted two clear patterns of cortical hyperperfusion and subcortical hyperperfusion in the ictal SPECT. Both patterns were different from the previously documented ictal patterns for complex partial seizures (CPS) or secondarily generalized seizures. Our results suggest that the tonic spasms of WS do not always have a single neurophysiological basis; e.g., patients with hemihypsarrhythmia and focal hypsarrhythmia did not show ictal hyperperfusion of the lesion with hypsarrhythmia. These findings indicate that the origin of hypsarrhythmia as an EEG feature and the origin of tonic spasms may be different in such patients. In particular, hypsarrhythmia appears to originate from cortical lesions, whereas the subcortical structures may be primarily responsible for the tonic spasms. Our report is the first published study of ictal SPECT in patients with WS.

Topics: Adolescent; Brain; Cerebral Cortex; Child; Child, Preschool; Cysteine; Diagnosis, Differential; Electroencephalography; Female; Functional Laterality; Humans; Male; Organotechnetium Compounds; Prognosis; Radiopharmaceuticals; Regional Blood Flow; Spasms, Infantile; Tomography, Emission-Computed, Single-Photon