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At least 19 recordsLinked to original sources
PubMed2000

Hemimegalencephaly and intractable epilepsy: complications of hemispherectomy and their correlations with the surgical technique. A report on 15 cases.

Hemispherectomy is required in most cases of hemimegalencephaly in order to control epilepsy refractory to medical treatment. Although there is a general agreement on the effectiveness of the procedure in controlling the seizure disorder, the choice of the surgical technique is still a subject of debate. In particular, anatomical hemispherectomy is blamed to be associated with a higher incidence of surgical complications, namely hydrocephalus and hemosiderosis, than other less ablative operations such as functional hemispherectomies. A series of 15 children with hemimegalencephaly, who had undergone anatomical hemispherectomy (11 cases), functional hemispherectomy (2 cases), and hemidecortication (2 cases) at the Pediatric Neurosurgery Section, Catholic University Medical School, Rome, is reported. Twelve of these patients presented with one or more complications in their postoperative course. Temporary complications, which resolved spontaneously or following medical therapy, included fever, wound breakdown, worsening of preoperative motor deficit, unilateral third cranial nerve deficit, dystonia, and anemia. In 8 patients, postoperative complications led to a second surgical procedure. A CSF shunt was necessary in 5 children, to control a secondary hydrocephalus. Two subjects underwent a toilette of the residual cavity because of persisting chemical abnormalities in CSF parameters. In a child a cranioplasty procedure was necessary as a consequence of an infection of the hemicranial bone flap. There was no apparent correlation between the rate and the type of complications with a specific surgical procedure. On the other hand, the age factor appeared to play an important role in the occurrence of secondary hydrocephalus, as all 5 children with this complication were less than 9 months old at the time of the hemispherectomy. Surgical mortality was nil in this series.

Brain↗
PubMed2002

Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide.

BACKGROUND: Epilepsy affects 80% of patients with Sturge-Weber syndrome; the majority of seizures begin before the age of 1. When seizures are intractable to medications and unihemispheric, hemispherectomy is often advised. OBJECTIVE: To examine the natural history of patients who underwent hemispherectomy and identify the outcomes in terms of seizure reduction, cognition, and motor deficits. METHODS: A questionnaire was mailed to the parents of patients identified by the Sturge-Weber Foundation as having had a hemispherectomy between 1979 and 2001. Forty-six percent (32/70) of the parents responded. RESULTS: The mean age at onset of seizures was 4 months, and the median age at surgery was 1.2 years. Children had failed to respond to 3.7 anticonvulsants prior to surgery and averaged 387 seizures/month. Forty-seven percent had complications (e.g., hemorrhage and hypertension) in the perioperative period; however, 81% are currently seizure-free, with 53% off anticonvulsants. Hemispherectomy type (anatomic versus functional versus hemidecortication) did not influence outcome. Age at onset of seizures did not predict seizure freedom; however, an older age at hemispherectomy was positively correlated. Postoperative hemiparesis was not more severe than before surgery. Cognitive outcome was not related to the age at operation, side of operation, or seizure freedom. CONCLUSIONS: Children undergoing hemispherectomy presented at a young age and had frequent seizures for approximately 1 year but are now mostly seizure-free. Age at surgery did not have an adverse effect on either seizure or cognitive outcomes.

Adolescent↗
PubMed1984

Compensatory motor function of the somatosensory cortex for dysfunction of the motor cortex following cerebellar hemispherectomy in the monkey.

Electrical activities of the motor and somatosensory cortices preceding visually-initiated hand movements were recorded with electrodes chronically implanted on the surface and at 2.5-3.0 mm depth in the cortex of monkeys, and changes in field potentials in these cortices after cerebellar hemispherectomy were observed for many weeks. As previously reported, a unilateral cerebellar hemispherectomy including the lateral and interpositus nuclei eliminates the cerebellar-mediated superficial thalamo-cortical (T-C) responses recorded in the forelimb motor cortex contralateral to the hemispherectomy. These T-C responses normally precede the hand movement, and the operation results in the delay of movement initiation. The electrodes in the forelimb area of the contralateral primary somatosensory cortex showed an enhancement of superficial T-C responses of the somatosensory cortex for 30-40 days after the operation. The enhanced potentials preceded the delayed movement as do the cerebellar-mediated superficial T-C responses of the motor cortex in normal situations. Local cooling of the somatosensory cortex following the cerebellar hemispherectomy disturbed the reaction time movement for a few weeks after the operation. This effect was rarely encountered in normal monkeys. The present study suggests the compensatory motor function of the somatosensory cortex for the dysfunction of the motor cortex in early weeks after cerebellar hemispherectomy.

Adaptation, Physiological↗
PubMed1996

Hemispherectomy for intractable seizures in children: a report of 58 cases.

Fifty-eight children who underwent anatomical, functional, or modified anatomical hemispherectomy for intractable seizures from 1986 to 1995 were evaluated for seizure control, motor function, and complications. Age at surgery ranged from 0.3 to 17.3 years (median 2.8 years). Twenty-seven anatomical, 27 functional, and 4 modified anatomical hemispherectomies were performed. Seizure control and motor function in the 50 patients with more than 1 year follow-up revealed a 90% or better reduction in seizure frequency in 44/50 (88%) overall: 19/22 (86%) anatomical, 23/26 (89%) functional, and 2/2 modified anatomical. Motor function of the preoperatively hemiparetic extremities was improved or unchanged postoperatively in 38/50 (76%) of the patients. Complications included one intraoperative death, one late death from shunt obstruction managed elsewhere, late postoperative seizure breakthrough requiring reoperation and further disconnection in 5/27 functional hemispherectomy patients, mild cerebrospinal fluid infections in 3/27 anatomical hemispherectomy patients, and hydrocephalus requiring shunting in 3/27 functional hemispherectomy patients. A review of the literature and comparison of techniques is presented.

Adolescent↗
PubMed1995

Hemispherical deafferentation: an alternative to functional hemispherectomy.

An alternative technique for so-called functional hemispherectomy has been developed to be used for the classical indications of hemispherectomy or the various modifications of functional hemispherectomy. The technique entails a smaller trepanation, less operation time, and less blood loss, and it leaves more brain tissue in place as compared with other functional hemispherectomy techniques. It starts with either hippocampectomy alone or with hippocampectomy and anterior temporal lobectomy. After this, deafferentation of the white matter of the temporal, occipital, parietal, and frontal lobe, using either a transcortical transventricular approach along the outline of the lateral ventricle or a sylvian key hole approach, is performed. The technique includes a transventricular callosotomy, and it leaves in place only a small portion of the suprainsular cortex and the insular cortex. However, as one modification, removal of the insular cortex can easily be performed, if necessary, and, as a second modification, the entire transventricular deafferentation can be performed through a sylvian key hole. In this report, the technique is described and the surgical experience for the first 13 patients is outlined. The immediate seizure relief with an average follow-up of 12 months was similar to that for patients with functional hemispherectomy, but the follow-up period for these 13 patients is not long enough to allow definite conclusions concerning long-term control of seizures and long-term complications.

Adolescent↗
PubMed1994

The role of hemispherectomy in the treatment of holohemispheric hemimegaloencephaly.

The role of hemispherectomy in treating holohemispheric hemimegaloencephaly, a unilateral brain malformation, is still not well defined. The authors describe the cases of five infants presenting with intractable seizures, progressive neurological deficits, and severe developmental delay. Electroencephalography (EEG) showed generalized polyspikes from the megaloencephalic hemisphere and progressive slowing on the opposite side in all children; contralateral seizure spikes occurred in three children. Three of the five children underwent hemispherectomy for intractable seizures before 2 years of age, after which the seizures subsided completely in two children and improved remarkably in the third. Preoperative Wada testing proved useful in evaluating pharmacologically the effect of hemispherectomy on contralateral polyspikes. Postoperative EEG revealed the absence of polyspikes in the operated hemisphere and decreased slowing on the contralateral side. Psychomotor development in the surgically treated infants exceeded that of the children not undergoing hemispherectomy. Of the two children treated medically, one died at 4 years of age in status epilepticus and the other (now 5 years old) has frequent seizures and severe developmental delay. Based on these results, hemispherectomy appears to be a useful procedure for controlling seizures and improving psychomotor development in children with hemimegaloencephaly involving the entire hemisphere. Surgery in infancy can prevent or minimize seizure foci and encephalopathic changes that may develop in the contralateral hemisphere. Staging the procedure and exercising meticulous hemostasis make surgery relatively safe in infants who otherwise may have significant blood loss associated with increased blood flow to the megaloencephalic hemisphere.

Anticonvulsants↗
PubMed1988

Functional hemispherectomy for treatment of epilepsy associated with hemiplegia: rationale, indications, results, and comparison with callosotomy.

Hemispherectomy is effective in arresting seizures associated with maximal or near maximal hemiparesis. This procedure, however, carries an unacceptable 33% risk of late complications due to cerebral hemosiderosis. Anatomically partial but functionally complete hemispherectomy was devised to avoid these complications. The frontal or occipital lobes, or both, were left in place with the blood supply intact but with connections to commissures and brainstem divided. The central strip and parietal and temporal lobes were removed. Twenty patients were so treated with a follow-up of 4 to 13 years (average, 7.3 years) in 14. Ten of these are seizure free, 1 had a single nocturnal seizure, 1 had occasional focal twitching, and 2 had a worthwhile but lesser reduction in the seizure tendency. None has developed cerebral hemosiderosis, to date. In appropriately selected patients, functional hemispherectomy is an effective procedure preferable to callosotomy or to partial hemispherectomy. When there is no independent ictal discharge from the opposite hemisphere, arrest of seizures may be expected, leading to improvement in cognitive functioning (mean increase, 10 IQ points), social behavior, and a reduction in or discontinuation of anticonvulsant medication. In these patients, gait and hand use remain unchanged.

Adolescent↗
PubMed1995

EEG prior to hemispherectomy: correlation with outcome and pathology.

Hemispherectomy, for the treatment of seizures, is highly successful but has a significant morbidity rate. The procedure is usually restricted to patients with an intractable seizure disorder and hemiparesis. Because of the inherent risk of surgery, patient selection is a critical issue. This report describes the evaluation of background activity and ictal patterns on surface and invasive EEG in 12 children who underwent both anatomical (7) and functional (5) hemispherectomy in order to determine the role of electroencephalography in the selection of patients for hemispherectomy, and to correlate EEG findings with underlying pathology and outcome. A favorable outcome was predicted by an interictal EEG with two or more of the following: suppression over the abnormal hemisphere, absence of contralateral slowing, absence of generalized discharges and absence of bilateral independent spiking; or by unilateral onset of ictal discharges on invasive intracerebral EEG recording. Outcome did not correlate with the underlying pathology. Hemispherectomy can be successful in patients with a variety of predominantly unilateral pathologic entities.

Brain↗
PubMed1996

Phonological dyslexia and phonological dysgraphia following left and right hemispherectomy.

Four adults who had hemispherectomies because of severe epilepsy following infantile of childhood damage to one hemisphere of the brain, are assessed on their reading and spelling abilities in an attempt to see if the two hemispheres are equipotential for these abilities in infancy. The psycholinguistic assessments of language processing in aphasia (PALPA) are used, and the results are interpreted from the viewpoint of hypotheses of "normal" right and left hemisphere reading abilities. Overall, the results suggest that the two hemispheres are equipotential at infancy for developing the skills underlying reading, but the left hemisphere is more specialized for the skills underlying spelling. All participants could read learnt regular and irregular words, and abstract and concrete words, suggesting that the reading lexicon develops in line with a normal left hemisphere lexicon, whichever hemisphere remains intact following hemispherectomy. However, poor reading of non-words suggests that the phonological reading route is severely impaired following left hemispherectomy (phonological dyslexia), and somewhat impaired following right hemispherectomy. The right-hemispherectomized participant is only mildly impaired on spelling real words, in contrast to the left-hemispherectomized participants who are markedly impaired. None of the participants could spell non-words, suggesting that the phonological spelling route is impaired following removal of either hemisphere (phonological dysgraphia).

Adult↗
PubMed1999

The significance of bilateral EEG abnormalities before and after hemispherectomy in children with unilateral major hemisphere lesions.

The rate of seizure relief following hemispherectomy varies between 50 and more than 80%. There has been particular debate concerning the significance of bilateral electroencephalography (EEG) abnormalities in influencing prognosis. This study was set out to determine the frequency of bilateral EEG abnormalities and their relationship to underlying pathology and outcome. We investigated 28 children with unilateral hemisphere lesions, who underwent hemispherectomy. Interictal and ictal EEGs before and after hemispherectomy were reviewed. Post-operative outcome with respect to seizures was noted. Bilateral EEG abnormalities were seen in 75%, but were more common in children with malformations of cortical development than in patients with acquired cerebral lesions, and were found more often in interictal than in ictal records. Post-operative EEG abnormalities were variable and did not consistently predict outcome. Short-term outcome was similar, irrespective of aetiology. With longer term follow-up, only 47% of children with developmental abnormalities were still seizure-free in contrast to 77% of children with acquired abnormalities. Although the incidence of bilateral EEG abnormalities in patients with major unilateral hemisphere lesions is high, these findings alone should not preclude further consideration for hemispherectomy. Our findings emphasise that the aetiology of the lesion plays a major role in determining outcome.

Adolescent↗
PubMed1999

Histopathologic findings in 37 cases of functional hemispherectomy.

Hemispherectomy procedures are performed in patients for whom focal cortical resection would be predicted to produce a significant reduction in seizures. The functional hemispherectomy procedure consists of disconnecting the hemispheres while attempting, in some cases, to preserve parenchyma. This study retrospectively reviews the histopathologic findings in 37 cases of functional hemispherectomy performed between 1990 and 1998 at a major epilepsy center. Procedures were performed in 20 males and 17 females who ranged in age from 3 months to 37 years (mean age, 9.6 years). In all but two cases, more than half or all the material submitted for pathologic testing was examined histologically. Cortical dysplasias or hemimegalencephaly were identified in 14 patients. The most common patterns of dysplasia observed included architectural disorganization (n = 13), increased molecular layer neurons (n = 11), and neuronal cytomegaly (n = 11). One patient was known to have epidermal nevus syndrome. Six patients had Sturge-Weber syndrome. Remote infarct/ischemic damage was identified as the etiology of seizures in six patients; four of these patients had mild associated secondary cortical architectural abnormalities. Three patients demonstrated pathology consistent with Rasmussen's encephalitis; one additional patient had chronic encephalitis changes, not otherwise specified. In two cases, changes consistent with hippocampal sclerosis were identified; additionally, hippocampal neuronal loss and gliosis was focally identified in three patients. Most of these patients had coexistent cortical dysplasia or radiographic evidence of remote infarct. One specimen demonstrated areas of infarct following resection of an arteriovenous malformation. In two specimens, significant histopathologic findings were not identified; both of these patients had radiographic evidence of remote infarct. The spectrum of pathologic conditions that may be encountered in the setting of a functional hemispherectomy is varied and in this study most frequently included cortical dysplasia, Sturge-Weber syndrome remote infarct, and Rasmussen's encephalitis.

Adolescent↗
PubMed2001

Pattern electroretinograms after cerebral hemispherectomy.

Cortically blind patients with brain damage restricted to the optic radiations or primary visual cortex may be able to detect and discriminate visual stimuli presented in their field defects, even though they deny seeing them. In contrast, patients who are hemianopic as a result of cerebral hemispherectomy cannot explicitly discriminate visual stimuli in their field defects, even when forced choice procedures are used. A possible explanation for this difference is that retrograde, transneuronal degeneration of the retina, which affects approximately 85% of wavelength-sensitive ganglion cells (approximately 70% of the total) after damage restricted to striate cortex, could be far more extensive after hemispherectomy, rendering the retina incapable of processing and conveying visual information to the brain. To test this, we assessed retinal ganglion cell function by means of electroretinography in three patients with cerebral hemispherectomy who were functionally blind. Steady-state pattern electroretinograms elicited by achromatic and isoluminant-chromatic (red-green) sinusoidal gratings, whose contrast was temporally modulated, were recorded from both blind and sighted hemiretinae. The electroretinograms were qualitatively indistinguishable from those of a control patient with a unilateral striate cortical lesion with documented visual capacity in his field defect. Within-subject analysis of variance revealed significant differences in the amplitude of the second harmonic (2f(0)) component of the averaged signal (diagnostic of retinal ganglion cell function) with respect to stimulus, but no significant differences between blind and sighted hemiretinae. This indicates that many retinal ganglion cells must have survived in the hemispherectomized patients. Isoluminant chromatic stimuli tended to elicit stronger signals than achromatic stimuli, which was unexpected given that wavelength-opponent Pbeta ganglion cells are far more susceptible than broad-band Palpha ganglion cells to transneuronal degeneration after cortical damage. It suggests that the 2f(0) component of the response to isoluminant chromatic stimuli might not reflect the activity of chromatic processes. Overall, the results show that the absence of residual vision in the blind fields of patients with cerebral hemispherectomy cannot be due to complete degeneration of retinal ganglion cells and, by extension, complete degeneration of their subcortical targets. This supports an alternative explanation, which is that intact extrastriate cortex is required for mediating voluntary responses to visual stimuli presented in the scotoma.

Adult↗
PubMed1996

Delayed complications thirty-six years after hemispherectomy: a case report.

PURPOSE: To describe a late complication of hemispherectomy in a patient in whom symptoms of hydrocephalus developed 36 years after her left-sided hemispherectomy, the longest delay on record. METHODS: Hemispherectomy has been successfully used in the treatment of intractable epilepsy associated with infantile-type hemiplegia for a half century. Of the patients, however, up to 33% have late increased cerebrospinal fluid pressure complications attributed to superficial cerebral hemosiderosis. Through a retrospective case analysis, we describe such complications in a 52-year-old woman with cognitive impairment, gait instability, urinary incontinence, and right hemineglect 36 years after her initial procedure. RESULTS: Quantitative, objective measures of cognition and gait-laboratory testing confirmed the patient's favorable clinical response to ventriculoperitoneal shunting, as well as the complete resolution of her symptoms, including the atypical occurrence of right-sided hemineglect. CONCLUSIONS: This case uniquely demonstrates the clinical features of a late complication of hemispherectomy while documenting the longest reported delay for developing such adverse sequelae. We also emphasize the need for more extensive follow-up studies to assess the extent of posthemispherectomy complications.

Brain↗
PubMed1998

Continuous unilateral epileptiform discharge and language delay: effect of functional hemispherectomy on language acquisition.

PURPOSE: To assess the efficacy of functional hemispherectomy in promoting language acquisition in a child with severe language delay and continuous left hemispheric epileptiform discharge in the absence of clinical seizures. We report a 6-month-old girl who initially presented with a right hemiparesis secondary to a lesion of probable prenatal origin in the distribution of the left middle cerebral artery. Weeks later, she developed infantile spasms. At 1.5 years of age, because of intractable seizures, the patient had fenestration of the left porencephalic cyst and anterior temporal lobectomy. The seizures ceased; however, language development remained limited to 35 words at 3.5 years of age. The EEG showed almost continuous epileptiform activity over the left hemisphere with no independent epileptiform potentials contralaterally. She underwent a functional left hemispherectomy. METHODS: Imaging, electroencephalographic investigation, pre- and postoperative psychological and speech assessment were carried out. RESULTS: Considerable language, speech, and behavior improvement was noted within 2 months of surgery. CONCLUSIONS: Interictal epileptiform discharges can interfere with the development of contralateral hemispheric function in the absence of clinical seizures. Early functional hemispherectomy may have a role in promoting optimal language development in appropriately selected patients. Although the primary indication for functional hemispherectomy is to control intractable seizures, a secondary proposed indication is to reduce functional impairment of the other hemisphere by electrical interference.

Brain↗
PubMed2001

Disconnective hemispherectomy.

Hemispherectomy is a valuable procedure in the management of seizure disorders caused by unilateral hemispheric disease. Modifications to anatomical hemispherectomy have been proposed to reduce the incidence of superficial cerebral hemosiderosis and hydrocephalus while still achieving seizure control. We report on the modification of a previously described disconnective form of hemispherectomy. We used this procedure on 2 children, with the aid of stereotactic navigation in 1 of the 2 cases. This disconnection was achieved via a transventricular route with minimal cortical resection or disruption of the blood supply. Over the 20 months of follow-up, 1 patient achieved complete seizure control, and 1 patient achieved control of previously incapacitating seizures with few minor seizures persisting. Motor function and speech significantly improved in both patients. Blood loss during the two procedures was significantly less than that reported for anatomical hemispherectomy, and so far there have been no signs of postoperative complications. The hospital stay was limited to 7-14 days after surgery.

Cerebral Cortex↗
PubMed2002

Recovery of language after left hemispherectomy in a sixteen-year-old girl with late-onset seizures.

Hemispherectomy is a very effective surgical treatment for intractable seizures that occur in the setting of Rasmussen's syndrome. The decision of when to perform a dominant hemispherectomy depends greatly on how late the surgeon believes some shift in language to the nondominant hemisphere can occur. We report a right-handed patient with Rasmussen's syndrome who underwent a left hemispherectomy at the age of 16 and has had excellent control of her seizures and remarkable language recovery. Our results indicate that dominant hemispherectomy in adolescence in the setting of late-onset seizures and Rasmussen's syndrome in the profoundly aphasic patient does not commit the patient to the prognosis of a fixed language deficit.

Adolescent↗
PubMed1997

Why would you remove half a brain? The outcome of 58 children after hemispherectomy-the Johns Hopkins experience: 1968 to 1996.

PURPOSE: To report the outcomes of the 58 hemispherectomies performed at Johns Hopkins between 1968 and January 1996. METHODS: Charts were reviewed of the 58 hemispherectomies performed at Johns Hopkins Medical Institutions by the Pediatric Epilepsy Group during the years 1968 to 1996. Twenty-seven operations were done for Rasmussen's syndrome, 24 operations for cortical dysplasias/hemimegalencephalies, and 7 for Sturge-Weber syndrome or other congenital vascular problems. Seizure control alone did not seem to adequately describe the outcomes of the procedure. Therefore, a score was constructed that included seizure frequency, motor disability, and intellectual handicap. This burden of illness score better described the child's handicap before and after surgery. RESULTS: Perioperative death occurred in 4 out of 58 children. Of the 54 surviving children, 54% (29/54) are seizure-free, 24% (13/54) have nonhandicapping seizures, and 23% (12/54) have residual seizures that interfere to some extent with function. Reduction in seizures was related to the etiology of the unilateral epilepsy. Eighty-nine percent of children with Rasmussen's, 67% of those with dysplasias, and 67% of the vascular group are seizure-free, or have occasional, nonhandicapping seizures. All operations were considered by the parents and the physicians to have been successful in decreasing the burden of illness. In 44 the procedure was very successful, in 7 it was moderately successful, and in 3 it was minimally successful. Success was related to the etiology, and early surgery was preferable. CONCLUSION: Hemispherectomy can be a valuable procedure for relieving the burden of seizures, the burden of medication, and the general dysfunction in children with severe or progressive unilateral cortical disease. Early hemispherectomy, although increasing the hemiparesis in children with Rasmussen's syndrome, relieves the burden of constant seizures and allows the child to return to a more normal life. In children with dysplasias, early surgery can allow the resumption of more normal development.

Adolescent↗
PubMed1992

Functional hemispherectomy.

Complete hemispherectomies were performed throughout the 1950's and early 1960's for the treatment of medically refractory seizures associated with hemiplegia. In 1964 a study published by Laine, Pruvet and Ossen reported a late post operative complication; superficial cerebral hemosiderosis. This, with its associated neurological deterioration, hydrocephalus, and at times death, led to disfavor with the procedure. It was replaced by the subtotal hemispherectomy which effectively eliminated the late complication of superficial cerebral hemosiderosis but was less successful in controlling seizures. Results decreased from 85% showing improved seizure tendency to 68%. Dissatisfied with these results, a hybrid operation was designed by Dr. Rasmussen and Dr. Villemure known as functional hemispherectomy. Removing less cortical tissue but disconnecting the remaining tissue provided a functionally complete but anatomically incomplete removal. Patients, having undergone this surgery, have obtained the same degree of seizure reduction without any of the late complications of the complete hemispherectomy. Our presentation will discuss the preoperative, operative and post-operative course of these patients. Criteria for surgery will be reviewed. A description of the surgical procedure will be included so that one can understand why the complications of aseptic meningitis, hydrocephalus, cerebral hemosiderosis and altered motor function will or will not occur postoperatively. Concerns of family and patient will be addressed throughout the presentation.

Cerebral Cortex↗