MEG May Reveal Hidden Population of Spikes in Epilepsy With Porencephalic Cyst/Encephalomalacia.

Porencephalic cyst/encephalomalacia is often associated with intractable epilepsy. A limited number of studies reported magnetoencephalography's (MEG's) potential to help construct treatment strategy for epilepsies associated with porencephalic cyst/encephalomalacia. The authors present here simultaneous electroencephalography (EEG) and MEG findings in three adult patients with pediatric-onset epilepsy due to porencephalic cyst/encephalomalacia. There were two types of spikes: one type was detected by MEG only (EEG-/MEG+), and the other detected by both EEG and MEG (EEG+/MEG+). Both types were seen in all three cases. The EEG-/MEG+ spikes all formed tight clusters; in contrast, the majority of EEG+/MEG+ spikes formed loose clusters. These data suggest that MEG may be helpful to better identify spike populations in epilepsy patients with porencephalic cyst/encephalomalacia. If hidden spike populations were to be found by MEG, this information would affect the interpretation of patient's pathophysiology and planning of intracranial electrode placement.

Hemifield slide from traumatic optic chiasmopathy.

A 56-year-old man suffered a high velocity linear acceleration closed head injury, resulting in skull fractures including bone dehiscence at the planum sphenoidale and tuberculum sellae. After regaining consciousness, he reported blurry vision and episodic diplopia. Visual field testing showed a bitemporal hemianopia. Ocular motility testing uncovered no misalignment. Brain MRI revealed post-traumatic encephalomalacia within the optic chiasm, resulting in the visual field defect and subsequent hemifield slide. Normally, cerebral processing of overlap between the visual fields prevents hemifield slide, which is caused by episodic loss of visual field overlap with subsequent slipping or sliding apart of images.

St Theresa's dart and a case of religious ecstatic epilepsy.

OBJECTIVE: : We report a case of ecstatic seizures with religious overtones in a patient whose semiology resembles that of Saint Theresa of Avila (1515 to 1582), and review the anatomical basis of this phenomenon. BACKGROUND: : Seizures with an emotional component are typically associated with negative sensations. Ecstatic seizures, particularly those with a religious character, are rarely reported, although they hold an important place in history, as a number of religious figures have been posited to be epileptic. Here, we present a patient with ecstatic seizures whose semiology and religious overtones resemble those of Saint Theresa of Avila (1515 to 1582), and discuss the anatomical basis of this phenomenon. METHOD: : Case report. RESULTS: : Evaluation of the patient demonstrated right temporal encephalomalacia on magnetic resonance imaging of the brain. Electroencephalogram showed focal slowing and poorly defined sharp waves in the right frontotemporal region. This area of pathology concurs with prior studies that localize ecstatic seizures to the anterior insula or temporal lobes. CONCLUSIONS: : Ecstatic seizures may involve the right temporal region. Although interpretation of ecstatic seizures is subject to individual variation, a similar localization and our patient's religious background may explain the resemblance between the semiology of his seizures, and that of Saint Theresa's visions as described almost 5 centuries ago.

Imaging diagnosis--polioencephalomalacia in a calf.

A 2-month-old mix-breed calf developed acute blindness and ataxia. Serum thiamine concentration was deficient. In antemortem magnetic resonance imaging there were laminar T2-hyperintense regions extending along the cerebral cortex that primarily affected the gray matter. The lesions were relatively symmetric between the left and right hemispheres but no abnormalities were present at the frontal lobes. At necropsy, laminar autofluorescence of the cerebral cortex was observed under ultraviolet exposure at 365 nm, consistent with a diagnosis of polioencephalomalacia. Polioencephalomalacia in the bovine species is compared with that in other species, namely humans, dogs, and cats.

Ipsilateral blinking seizures during left fronto-temporal ictal pattern on scalp EEG.

We report an infant with left eye blinking seizures accompanying a left (ipsilateral) fronto-temporal scalp EEG ictal pattern. The epileptogenic lesion was a left frontal encephalomalacia along the ventriculo-peritoneal shunt tract. The shunt was inserted for treatment of communicating hydrocephalus. This case illustrates the lateralizing value of the ictal blinking. Review of the literature suggests that seizures with unilateral blinking are likely to be produced by activation of ipsilateral trigeminal fibers innervating subdural intracranial structures and pial vessels in temporal and frontal lobes. Ipsilateral blinking could also be produced by activation of the ipsilateral cerebellar hemisphere.

Early destructive lesions in the developing brain: clinical and electrographic correlates.

OBJECTIVE: Early brain insults can cause cavitary lesions including porencephaly (POR) and multicystic encephalopathy (MCE). The objective of this study was to investigate clinical and electrographic correlates associated to these types of destructive brain lesions. METHOD: Patients with POR and MCE were selected and submitted to clinical and Video-EEG monitoring. The following variables were analyzed: demographic data, type of lesion, presence of gliosis, perinatal complications, epilepsy, brain atrophy, and presence and frequency of epileptiform discharges. RESULTS: Twenty patients were included, 65% males, 35% females, ages ranging from 1 to 40 years, 14 with MCE and 6 with POR. Eighteen patients had hemiparesis, 19 had epilepsy (current or in the past), seven of them had refractory seizures, and 16 had epileptiform discharges. All patients with MCE had gliosis while only 2 with POR had it. CONCLUSIONS: No correlation was observed between type of lesion and clinical and electrographical outcome. However, a positive correlation was observed between frequency of discharges and presence of brain atrophy, and between MCE and gliosis.

Early destructive lesions in the developing brain: clinical and electrographic correlates

OBJECTIVE: Early brain insults can cause cavitary lesions including porencephaly (POR) and multicystic encephalopathy (MCE). The objective of this study was to investigate clinical and electrographic correlates associated to these types of destructive brain lesions. METHOD: Patients with POR and MCE were selected and submitted to clinical and Video-EEG monitoring. The following variables were analyzed: demographic data, type of lesion, presence of gliosis, perinatal complications, epilepsy, brain atrophy, and presence and frequency of epileptiform discharges. RESULTS: Twenty patients were included, 65 percent males, 35 percent females, ages ranging from 1 to 40 years, 14 with MCE and 6 with POR. Eighteen patients had hemiparesis, 19 had epilepsy (current or in the past), seven of them had refractory seizures, and 16 had epileptiform discharges. All patients with MCE had gliosis while only 2 with POR had it. CONCLUSIONS: No correlation was observed between type of lesion and clinical and electrographical outcome. However, a positive correlation was observed between frequency of discharges and presence of brain atrophy, and between MCE and gliosis.

OBJETIVO: Insultos cerebrais precoces podem causar lesões cavitárias incluindo porencefalias (POR) e encefalomalacias multicisticas (EMC). O objetivo deste estudo foi investigar correlatos clínicos e eletrográficos associados a estes dois tipos de lesões destrutivas. MÉTODO: Pacientes com POR e EMC foram selecionados e submetidos à avaliação neurológica e monitorização vídeo-eletrencefalográfica, analisando-se as seguintes variáveis: dados demográficos, tipo de lesão, presença de gliose, complicações perinatais, epilepsia, atrofia cerebral, presença e freqüência de descargas epilépticas. RESULTADO: Vinte pacientes foram incluídos, sendo 65 por cento do sexo masculino, 35 por cento do feminino, idades entre 1 e 40 anos, sendo 14 com EMC e 6 com POR. Dezoito pacientes tinham hemiparesia, 19 tinham ou tiveram epilepsia (7 deles refratários ao tratamento medicamentoso) e 16 deles tinham paroxismos epileptiformes. Todos com MCE tinham gliose associada, contra apenas 2 dos pacientes com POR. CONCLUSÃO: Não houve correlação entre tipo de lesão e evolução clínica e eletrográfica. Houve, entretando, correlação positiva entre freqüência de descargas epilépticas e presença de atrofia cerebral, e entre lesão do tipo EMC e presença de gliose.

Cortical reorganization induced by virtual reality therapy in a child with hemiparetic cerebral palsy.

Virtual reality (VR) therapy is a new, neurorehabilitation intervention aimed at enhancing motor performance in children with hemiparetic cerebral palsy (CP). This case report investigated the effects of VR therapy on cortical reorganization and associated motor function in an 8-year-old male with hemiparetic CP. Cortical activation and associated motor development were measured before and after VR therapy using functional magnetic resonance imaging (fMRI) and standardized motor tests. Before VR therapy, the bilateral primary sensorimotor cortices (SMCs) and ipsilateral supplementary motor area (SMA) were predominantly activated during affected elbow movement. After VR therapy, the altered activations disappeared and the contralateral SMC was activated. This neuroplastic change was associated with enhanced functional motor skills including reaching, self-feeding, and dressing. These functions were not possible before the intervention. To our knowledge, this is the first fMRI study in the literature that provides evidence for neuroplasticity after VR therapy in a child with hemiparetic CP.

fMRI reveals large-scale network activation in minimally conscious patients.

BACKGROUND: The minimally conscious state (MCS) resulting from severe brain damage refers to a subset of patients who demonstrate unequivocal, but intermittent, behavioral evidence of awareness of self or their environment. Although clinical examination may suggest residual cognitive function, neurobiological correlates of putative cognition in MCS have not been demonstrated. OBJECTIVE: To test the hypothesis that MCS patients retain active cerebral networks that underlie cognitive function even though command following and communication abilities are inconsistent. METHODS: fMRI was employed to investigate cortical responses to passive language and tactile stimulation in two male adults with severe brain injuries leading to MCS and in seven healthy volunteers. RESULTS: In the case of the patient language-related tasks, auditory stimulation with personalized narratives elicited cortical activity in the superior and middle temporal gyrus. The healthy volunteers imaged during comparable passive language stimulation demonstrated responses similar to the patients' responses. However, when the narratives were presented as a time-reversed signal, and therefore without linguistic content, the MCS patients demonstrated markedly reduced responses as compared with volunteer subjects, suggesting reduced engagement for "linguistically" meaningless stimuli. CONCLUSIONS: The first fMRI maps of cortical activity associated with language processing and tactile stimulation of patients in the minimally conscious state (MCS) are presented. These findings of active cortical networks that serve language functions suggest that some MCS patients may retain widely distributed cortical systems with potential for cognitive and sensory function despite their inability to follow simple instructions or communicate reliably.

Neurologic abnormalities and cerebrospinal fluid changes in horses administered fumonisin B1 intravenously.

The objective of this experiment was to characterize a dose-dependent toxic effect of fumonisin B1 (FB1) and to document initial neurologic signs, clinical progression, and terminal cerebrospinal fluid (CSF) changes in horses administered FB1 IV. Seventeen healthy horses were administered 0.00 (n = 4), 0.01 (n = 3), 0.05 (n = 3), 0.10 (n = 3), or 0.20 mg (n = 4) of purified FB1 IV q24h. When neurologic abnormalities observed by a masked observer became severe, atlanto-occipital CSF taps were performed and CSF pressure, cell count, cytology, protein, albumin and glucose concentrations, and creatine kinase activity were measured. Changes in CSF and number of days to 1st observation of neurologic abnormalities were compared between doses by ANOVA, with the level of significance set at P < .05. Control horses and low-dose horses (0.01 mg/kg) remained neurologically normal. In higher dose FB1-treated horses (n = 10), initial clinical signs (days 4-10) included hindlimb ataxia, delayed forelimb placing, and decreased tongue tone and movement. Hindlimb and trunkal ataxia, depression, hyperesthesia, and intermittent dementia gradually became apparent. When data from all horses with neurologic abnormalities were pooled (0.05-0.20 mg/kg FB1), mild clinical signs (mean day 6.3) occurred significantly earlier than did more severe (mean day 8.9) clinical signs (P = .009). Neurologic horses had high CSF protein, albumin, and IgG concentrations and increased albumin quotients (P < .05). It was concluded that FB1-induced neurologic and CSF changes in a dose-dependent manner, with a no-observable-limit of 0.01 mg FB1/kg IV q24h for 28 days. The neurologic and CSF changes were consistent with vasogenic cerebral edema.

Cardiovascular changes associated with intravenous administration of fumonisin B1 in horses.

OBJECTIVE: To determine whether cardiovascular dysfunction is evident in horses with leukoencephalomalacia experimentally induced by administration of fumonisin B1. ANIMALS: 11 healthy horses of various breeds (body weight, 252 to 367 kg). PROCEDURE: Horses were randomly assigned to 3 groups and administered fumonisin B1 daily. Horses received IV injections of 0 (control horses; n = 4), 0.01 (3), or 0.20 mg (4) of fumonisin B1/kg for 7 to 28 days. Horses were examined daily for evidence of neurologic disease. When neurologic signs consistent with leukoencephalomalacia were evident, horses were anesthetized, and catheters were inserted for evaluation of the cardiovascular system. After recovery from anesthesia, hemodynamic measurements were obtained. RESULTS: Fumonisin-treated horses with clinical signs of neurologic disease had evidence of cardiovascular dysfunction manifested as decreases in heart rate, cardiac output, right ventricular contractility (assessed by measuring the maximal rate of change of right ventricular pressure), coccygeal artery pulse pressure, and pH and base excess in venous blood as well as increases in systemic vascular resistance, compared with values for control horses. Fumonisin-treated horses with and without clinical signs of neurologic disease also had higher serum and right ventricular sphinganine and sphingosine concentrations than control horses. CONCLUSIONS AND CLINICAL RELEVANCE: An association was detected among fumonisin-induced neurologic disease, increased serum and myocardial sphinganine and sphingosine concentrations, and decreased cardiovascular function in horses. Fumonisin-induced decreases in cardiovascular function may contribute to the pathophysiologic development of leukoencephalomalacia in horses.

Occipitoparietal epilepsy, hippocampal atrophy, and congenital developmental abnormalities.

PURPOSE: Diagnostic uncertainty may arise in patients with occipitoparietal epilepsy when there is neuroimaging evidence of a posterior quadrant lesion and coexistent hippocampal abnormalities ("dual pathology"). It is not known whether hippocampal atrophy (HA) in these patients results from seizure propagation to temporolimbic structures or whether it is part of the pathological process underlying the occipitoparietal epilepsy. Clarification of this issue may have a significant bearing on the management of these patients. METHODS: We studied 20 patients with occipitoparietal epilepsy and neuroimaging or pathologic evidence of a congenital developmental abnormality. Normalized hippocampal volumes were obtained in all patients. The medical records and video-EEG recordings were analyzed to correlate the MRI findings with clinical data, seizure semiology, and EEG findings. RESULTS: HA was found in seven patients (35%). Neuroimaging abnormalities concordant with the side of HA were seen in all cases. There was clinical or EEG evidence of temporal spread in 12 patients. There was no correlation between the presence of HA and temporal lobe spread. The only clinical factor associated with HA in this series was a younger age of seizure onset. CONCLUSIONS: HA in patients with occipitoparietal epilepsy due to congenital developmental abnormalities is most likely to be a marker of a more widespread process related to a common pathogenesis during prenatal or perinatal development. HA in these patients is unlikely to be the result of secondary spread from an extrahippocampal focus. Surgical treatment should be tailored toward the primary epileptogenic zone rather the site of seizure spread.

Sulfur-induced polioencephalomalacia in stocker calves.

Calves from 3 farms exhibited blindness, head pressing, and circling before death. Brain lesions confirmed polioencephalomalacia. Excess sulfur was found in the diets on all 3 farms in corn by-products or molasses based supplements. Corn gluten feed and corn steep liquor (by-products of the refinement of corn for ethanol), corn syrup, corn gluten, corn oil, and corn starch have gained popularity as livestock feeds due to their low prices. With this increased usage as livestock feed, increasing number of cases of polioencephalomalacia have been seen.

Resection of frontal encephalomalacias for intractable epilepsy: outcome and prognostic factors.

PURPOSE: Because focal encephalomalacia is an important cause of medically intractable partial epilepsy and few studies have evaluated the efficacy and the safety of resecting focal-encephalomalacias to improve seizure control, we studied a cohort of 17 consecutive patients who underwent resection of encephalomalacias in the frontal lobes as a treatment of their intractable epilepsy. METHODS: We evaluated several factors for their value in predicting postsurgical seizure control. Pre- and postsurgical magnetic resonance imaging (MRI) scans were reviewed independently by 2 blinded investigators. RESULTS: At a median of 3 years of follow-up (range 0.6-7.5 years), 12 patients (70%) were seizure-free or had only rare seizures. The presence of a focal fast frequency discharge (focal ictal beta pattern) at the beginning of seizures on scalp EEG was predictive of seizure-free outcome (p = 0.017), even among patients who had complete resection of their encephalomalacias (p = 0.016). There was no significant differences in outcome with regard to age at the time of the injury that caused encephalomalacia, interval between injury and onset of seizures, duration of presurgical seizure history, presurgical seizure frequency, age at surgery, or the completeness of encephalomalacia resection. The analysis regarding completeness of encephalomalacia resection almost reached significance, suggesting that it may also be an important predictive factor (p = 0.051). CONCLUSIONS: We conclude that surgery is a very effective treatment for intractable frontal lobe epilepsy (FLE) secondary to encephalomalacias. Patients are more likely to become seizure-free if they have a focal ictal beta discharge on their scalp EEG. Complete resection of the encephalomalacia should be attempted, since our results suggest that this may be a favorable predictive factor. Moreover, the operative strategy for our patients entailed, whenever possible, complete resection of the encephalomalacias and of the adjacent electrophysiologically abnormal tissues.

Sulphur-induced polioencephalomalacia in lambs.

An outbreak of polioencephalomalacia affected 16 of 46 Swaledale lambs and five of 25 Scottish blackface lambs 15 to 32 days after they were introduced to an ad libitum concentrate ration containing 0.43 per cent sulphur. The clinical signs were acute and included depression central blindness and head-pressing, but no hyperaesthesia, nystagmus, dorsiflexion of the neck or opisthotonos were observed. Treatment of the affected lambs with vitamin B1, dexamethasone and antibiotics was associated with a prolonged recovery period, though no further cases were identified after vitamin B1 had been given parenterally to all the lambs at risk.

[Multivariate autoregressive analysis of carotid artery blood flow waveform in a newborn with multicystic encephalomalacia].

We analyzed the carotid artery blood flow waveform (CABFW) through multivariate autoregressive analysis in a case with multicystic encephalomalacia (MCE) after neonatal asphyxia and compared the result with those of 35 healthy newborns. The total power of CABFW was at the -2 SD level of the value for 35 healthy newborns, and the power, % power, bio-informing amounts and damping time of component 3 (damping frequency 11.15 Hz) were less than -2 SD of the values in 35 healthy newborns. The Pulsatility Index (PI) of anterior cerebral artery (ACA) was high (0.76). These results suggest that cerebral blood flow decreases because of cerebral vasoconstriction in MCE after neonatal asphyxia.

Oedipism in a patient with frontal lobe encephalomalacia.

There is a growing recognition of the role of the frontal lobes in the aetiology of severe behavioural aberrations. The authors describe a case of Oedipism in a patient who had MRI evidence of frontal lobe encephalomalacia. After discussing the function of the frontal lobes in modulating behaviour the authors suggest that the structural lesion seen on the MRI was in part responsible for the patient's self-destructive act. Treatment issues and the importance of recognizing underlying structural lesions in instances of extreme self-mutilation are discussed.

Computerized electroencephalographic assessment of congenital brain infarction.

We studied 12 children (8 female and 4 male) aged 2.2-14.3 years, whose computed tomographic (CT) examination had shown evidence of malacic and/or porencephalic outcomes of early vascular brain infarction. Topographic spectral electroencephalographic (EEG) analysis was performed in all patients in the awake state. The following spectral EEG variables were studied: topography, absolute and relative power of delta, theta, alpha, beta bands, overall power, and peak alpha frequency asymmetries. The results of topographic spectral EEG analysis were compared with the localization and nature of lesions as detected by CT scans. Depending on the nature of the lesions, we were able to identify two different spectral patterns. Porencephalic cysts were characterized by an increase in delta and theta bands in the areas surrounding the lesion sites, as identified by CT. Spectral EEG patterns of malacic outcomes resulted in a focal increase in theta and delta band power, corresponding to the topography of lesions. Moreover, in 9/12 subjects an asymmetry of alpha rhythm in occipital leads was found homolaterally to the lesion sites, associated with a decrease in power, without any CT evidence of an occipital lesion.

Multicystic encephalomalacia in an adult case of Lennox-Gastaut syndrome: a case report.

It is well known that multicystic encephalomalacia results in a severe neurological deficit and psychomotor retardation. The authors reported the case of a 30-year-old man with Lennox-Gastaut syndrome who had multicystic encephalomalacia in CT and MRI but whose neurological deficit was not serious. It is possible that the forceps delivery caused MCE and that a vaccination against Japanese encephalitis at the age of 9 triggered the Lennox-Gastaut syndrome.