Hemispheric Atrophy Research Articles

Granulocyte colony-stimulating factor does not promote neurogenesis after experimental intracerebral haemorrhage.

Hematopoietic growth factors have been suggested to induce neuroprotective and regenerative effects in various animal models of cerebral injury. However, the pathways involved remain widely unexplored. This study aimed to investigate effects of local and systemic administration of granulocyte colony-stimulating factor on brain damage, functional recovery, and cerebral neurogenesis in an intracerebral haemorrhage whole blood injection model in rats. Eight-week-old male Wistar rats (n = 100) underwent induction of striatal intracerebral haemorrhage by autologous whole blood injection or sham procedure and were randomly assigned to either (a) systemic treatment with granulocyte colony-stimulating factor (60 μg/kg) for five-days; (b) single intracerebral injection of granulocyte colony-stimulating factor (60 μg/kg) into the cavity; or (c) application of vehicle for five-days. Bromodeoxyuridine-labelling and immunohistochemistry were used to analyze proliferation and survival of newly born cells in the sub-ventricular zone and the hippocampal dentate gyrus. Moreover, functional deficits and lesion volume were assessed until day 42 after intracerebral haemorrhage. Differences in lesion size or hemispheric atrophy between granulocyte colony-stimulating factor-treated and control groups did not reach statistical significance. Neither systemic, nor local granulocyte colony-stimulating factor administration induced neurogenesis within the dentate gyrus or the sub-ventricular zone. The survival of newborn cells in these regions was prevented by intracerebral granulocyte colony-stimulating factor application. A subtle benefit in functional recovery at day 14 after intracerebral haemorrhage induction was observed after granulocyte colony-stimulating factor treatment. There was a lack of neuroprotective or neuroregenerative effects of granulocyte colony-stimulating factor in the present rodent model of intracerebral haemorrhage. Conflicting results from functional outcome assessment require further research.

Brain atrophy in primary progressive aphasia involves the cholinergic basal forebrain and Ayala's nucleus

Primary progressive aphasia (PPA) is characterized by left hemispheric frontotemporal cortical atrophy. Evidence from anatomical studies suggests that the nucleus subputaminalis (NSP), a subnucleus of the cholinergic basal forebrain, may be involved in the pathological process of PPA. Therefore, we studied the pattern of cortical and basal forebrain atrophy in 10 patients with a clinical diagnosis of PPA and 18 healthy age-matched controls using high-resolution magnetic resonance imaging (MRI). We determined the cholinergic basal forebrain nuclei according to Mesulam's nomenclature and the NSP in MRI reference space based on histological sections and the MRI scan of a post-mortem brain in cranio. Using voxel-based analysis, we found left hemispheric cortical atrophy in PPA patients compared with controls, including prefrontal, lateral temporal and medial temporal lobe areas. We detected cholinergic basal forebrain atrophy in left predominant localizations of Ch4p, Ch4am, Ch4al, Ch3 and NSP. For the first time, we have described the pattern of basal forebrain atrophy in PPA and confirmed the involvement of NSP that had been predicted based on theoretical considerations. Our findings may enhance understanding of the role of cholinergic degeneration for the regional specificity of the cortical destruction leading to the syndrome of PPA.

Dyke Davidoff Masson Syndrome – A Case Report

A 10 year old male child reported to paediatric neurology OPD of BIRDEM General Hospital with the complaints of weakness of left side of body, seizure and facial asymmetry for 2 years. He was on regular anti convulsive therapy and failed to control seizure. General examination revealed no significant abnormality neither delayed mile stones of development happened. Neurological examination revealed left sided spastic hemiparesis, brisk tendon reflexes and extensor planter on left side. With detailed history and examination he was diagnosed as a case of infantile seizure and undergone CT scan of brain followed by MRI scan. Both the reports revealed severe atrophy of right cerebral hemisphere, thinning of cortical gyri, widening of sulci and dilatation of right lateral ventricle with ipsilateral midline shift and was concluded as hemiatrophy of right cerebral hemisphere with suspicion of Dyke Davidoff Masson Syndrome (DDMS), Hemimegalencephaly and Sturge-Weber syndrome. Dyke-Davidoff-Masson syndrome (DDMS) is a rare condition characterized by asymmetric cerebral hemispheric growth with unilateral atrophy, ipsilateral compensatory osseous hypertrophy, hyperpneumatization of the paranasal sinuses and mastoid cells, and contralateral paresis. Varying degrees of hemiparesis, hemiplegia, seizures, mental retardation, and facial asymmetry can be associated with DDMS. Considering clinical history and imaging findings, final Diagnosis was Dyke Davidoff Masson Syndrome. Birdem Med J 2013; 3(2): 196-109 DOI: http://dx.doi.org/10.3329/birdem.v3i2.17215

Dyke-davidoff-masson syndrome associated with epidermoid tumour and arachnoid cyst: a case report.

Dyke-Davidoff-Masson Syndrome (DDMS) is a rarely seen clinical entity which is characterised by cerebral hemiatrophy, contralateral hemiparesis and epilepsy. Radiological features are typical, such as unilateral atrophy of the cerebral hemisphere and associated compensatory bone changes in the skull, like thickening, enlargement of the paranasal sinuses and mastoid air cells. In this article, we report the first case of DDMS associated with epidermoid tumour and arachnoid cyst, who underwent operation for an epidermoid tumour in the inter-hemispheric region. To our knowledge, this is the first report of DDMS associated with multiple intracranial pathologies and this association has not been previously described in the literature. Any patient who receives DDMS in the light of clinical and radiological findings should be investigated for concomitant pathologies. Different sequences of MRI may be useful in the diagnosis of other intracranial lesions.

FIRES and IHHE: Delineation of the syndromes

Idiopathic hemiconvulsion hemiplegia and epilepsy syndrome (IHHE) and febrile infection-related epilepsy syndrome (FIRES) are rare epileptic syndromes characterized by the occurrence of status epilepticus in a previously healthy child during or closely after a febrile episode. In both syndromes, there is no evidence of central nervous system infection (encephalitis) and the etiology remains unclear. Treatment is disappointing, particularly in FIRES, except for a response to ketogenic diet (KD) in half of patients. In IHHS, children develop hemispheric brain atrophy with contralateral hemiplegia, epilepsy, and a variable degree of cognitive deficit. Patients with FIRES develop refractory epilepsy with severe cognitive deficit affecting the temporal and frontal lobe functions. The role of inflammation is hypothesized with a vicious circle involving inflammation and seizure activity facilitated by brain maturation putting them under the concept of "acute encephalopathy with inflammation-mediated status epilepticus."

Hemiconvulsion–hemiplegia syndrome revisited: longitudinal MRI findings in 10 children

Hemiconvulsion-hemiplegia syndrome (HHS) is a rare severe epilepsy of infancy consisting of unilateral convulsive status epilepticus immediately followed by transient or lasting ipsilateral hemiplegia. HHS may occur either in patients with previous brain pathology or without any identified cause, so-called 'idiopathic HHS'. We retrospectively analysed clinical and MRI longitudinal findings of a series of 10 patients (six females, four males) presenting with HHS. Age at the study inclusion ranged from 2 years 6 months to 15 years (mean of 5 y 10 mo, median 4 y 2 mo). After defining magnetic resonance imaging (MRI) features as 'typical', i.e. strictly unilateral involvement, and 'atypical', i.e. bilateral, we compared clinical data from both groups. Cognitive level was assessed using Brunet-Lézine or Wechsler scales. HHS occurred at a mean age of 20.5 months (range 8-48 mo). In all cases, status epilepticus lasted for more than 1 hour and was characterised by unilateral clonic seizures followed by ipsilateral hemiplegia (persistent in five patients). Two patients in this series died: the first from multi-organ failure 2 weeks after the status epilepticus and the other from a second episode of ipsilateral intractable febrile status epilepticus 3 years after the first episode. Early MRI (days 1-7 from status epilepticus) showed hemispheric cytotoxic oedema in all, extending to the contralateral side for one. T2 hyperintensity in the basal ganglia was disclosed in 70% of patients and in the hippocampus in 60%. After 1 month (in intermediate and chronic phases), all surviving patients but one showed hemispheric cortical atrophy corresponding to the regions involved during the early stage. Comparing clinical features of patients presenting with 'typical' features, to those with 'atypical' findings, the second group presented psychomotor delay before status epilepticus. This series underlines the major value of early MRI for the prompt diagnosis of HHS, and shows that involvement of subcortical structures has been underestimated. Hippocampal involvement is not constant.

Extrarolandic electroclinical findings in the evolution of adult-onset Rasmussen's encephalitis

Rasmussen's encephalitis (RE) is a rare immunomediated disorder characterized by unilateral hemispheric atrophy, drug-resistant focal epilepsy, and progressive neurological deficits. Its onset typically occurs in childhood, though it has also been reported in adult age (A-RE) with atypical clinical features. The aim of this study was to describe the electroclinical features in a group of seven patients with A-RE.We retrospectively studied seven women aged 23–43years (mean: 32.1years) with a diagnosis of RE according to commonly accepted diagnostic criteria. All the patients were clinically evaluated and underwent prolonged video-EEG monitoring, laboratory investigations, and high-resolution MRI follow-up.All the patients displayed an ictal electroclinical pattern whose evolution varied. We identified an early phase characterized by polymorphic ictal electroclinical manifestations (temporal semiology in five cases, frontal in one, and parietal in the remaining case) and a late phase clinically characterized by viscerosensitive phenomena followed by somatosensitive signs, experiential symptoms, and motor signs in all the cases. In the late phase, the ictal EEG pattern was characterized by monomorphic, pseudorhythmic, repetitive slow-wave theta activity over the frontal and central regions, with ipsilateral propagation and/or secondary spreading to contralateral perisylvian structures. Patients were treated with a combination of AEDs and immunotherapy (steroids and IVIg); epilepsy surgery was performed in 3 cases.Our results show that A-RE is characterized by early and late clinical- and EEG-different features which may reflect a progressive involvement of a specific “extrarolandic” network in the advanced phase of the disease and may suggest that the electroclinical expression of RE varies according to the different stages of the pathological process.

Pre- and postnatal imaging of early cerebral damage in Sturge-Weber syndrome

A case of prenatal diagnosis of Sturge-Weber syndrome associated with polymicrogyria is reported. The diagnosis was based on a unique association with unilateral hemispheric gyriform calcification, focal hemispheric atrophy and white matter changes on prenatal imaging including ultrasound and MRI. Polymicrogyria, which is exceptionally associated with Sturge-Weber syndrome, is suggestive of and reinforces the hypothesis of early impairment of the cerebral microvasculature related to leptomeningeal angioma, which may lead to abnormal cerebral development as early as the second trimester of pregnancy.

Rotigotine improves executive functions in patients with vascular dementia

background activity but no interictal discharges suggestive of epilepsy. Neuropsychological evaluations with 2-year intervals showed progressivewordfinding difficulties, attentionand memory-deficits. He became nearly mute and apathetic and was unable to continue his work as a process-controller. The MRI was repeated 8-year after symptom-onset showing prominent atrophy of the left hemisphere (figure), consistent with the clinical diagnosis progressive non-fluent aphasia. Case-management was still lacking at referral to our memory clinic. The presence of epilepsy in the presented patient with PPA has led to a long-lasting delay in clinical diagnosis and adequate care. Patients with subtle speech difficulties usually seek medical attention on average 2-3 years after symptom-onset. Primary and secondary care physicians often do not recognise PPA-symptoms adding a further diagnostic delay. Complaints are often attributed to psychogenic factors (burnout, mood or anxiety) or to other comorbid conditions, which even led to a diagnostic delay of 8 years in our case. Conclusions: An atypical age-at-onset, slow disease progression, absence of cognitive or behavioural problems are all factors delaying referral to a memory clinic in PPA. Diagnostic delay is even more pronounced in the presence of comorbid conditions which may affect speech.

Transplantation of bone marrow stromal cells enhances nerve regeneration of the corticospinal tract and improves recovery of neurological functions in a collagenase-induced rat model of intracerebral hemorrhage.

The reorganization of brain structures after intracerebral hemorrhage (ICH) insult is crucial to functional outcome. Although the pattern of neuronal rewiring is well-documented after ischemic stroke, the study of brain plasticity after ICH has been focusing on the enhancement of dendritic complexity. Here we hypothesized that functional restoration after ICH involves brain reorganization which may be favorably modulated by stem cell transplantation. In this study, bone marrow stromal cells (BMSCs) were transplanted into the perilesional sites of collagenase-induced ICH in adult rats one day after ICH injury. Forelimb functional recovery was monitored with modified limb placing and vibrissae-elicited forelimb placement tests. Anterograde and retrograde tracing were used to assess the reorganization of bilateral forelimb areas of the sensorimotor cortex. We found that in rats transplanted with BMSCs after ICH injury, axonal sprouting occurred in the contralateral caudal forelimb area of the cortex, and was significantly higher than in ICH rat models that received only the vehicle (P < 0.01). The number of positive neurons in the ipsilateral rostral forelimb area of the cortex of the BMSC group was 1.5-to 4.5-fold greater than in the vehicle group (P < 0.05). No difference was found between the BMSC and vehicle groups in hemispheric atrophy or labeled neurons in the ipsilateral caudal forelimb area (P = 0.193). Scores for improved functional behavior in the BMSC group were in accord with the results from histology. Neuronal plasticity of the denervated corticospinal tract at bilateral forelimb areas of the cortex in the collagenase-induced ICH rat models was significantly enhanced by BMSC transplantation. BMSC transplantation may facilitate functional recovery after ICH injury.

Disconnective surgery in posterior quadrantic epilepsy: experience in a consecutive series of 10 patients

Outcomes following functional hemispherotomy in patients with drug-resistant epilepsy have been well described. However, studies reporting long-term longitudinal outcomes after subhemispheric disconnective epilepsy surgery are still limited. The authors conducted a retrospective review of prospectively collected data of 10 children who underwent temporoparietooccipital (TPO) disconnective surgery at the Vienna Pediatric Epilepsy Center. There were 3 males and 7 females (median age 8.7 years; range 4.2-22.1 years). The affected hemisphere was the left in 3 patients and the right in 7. The patients' median age at seizure onset was 3.0 years (range 0.2-8.3 years). The median duration of epilepsy before surgery was 5.2 years (range 1.3-17.2 years). The underlying pathology was TPO malformation of cortical development in 5 patients, and venous infarction, posterior hemispheric quadrant atrophy, Sturge-Weber syndrome, cortical involvement of a systemic lupus erythematosus, and gliosis after cerebral tumor treatment in 1 each. In 6 children, a pure TPO disconnection was performed; in 2 patients, the temporal lobe was resected and parietooccipital disconnection was performed. The 2 remaining patients had had previous epilepsy surgery that was extended to a TPO disconnection: disconnection of the occipital lobe (n = 1) and resection of the temporal lobe (n = 1). The authors encountered no complications while performing surgery. No patient needed blood replacement therapy. No patient developed CSF disturbances that warranted treatment. Nine of 10 patients are currently seizure free since surgery (Wieser Class 1a) at a median follow-up time of 2.1 years (range 4 months to 8.1 years). Temporoparietooccipital disconnection is a safe and effective motor-sparing epilepsy surgery in selected cases. Technical adjuncts facilitate a better intraoperative visualization and orientation, thereby enabling a less invasive approach than previously suggested.

Bradicardia en la demencia frontotemporal

IntroductionNumerous regions of the brain, such as the medial frontal cortex, orbitofrontal cortex, insula, and amygdala, participate in the autonomic control of cardiovascular functions such as heart rate. The degenerative process in frontotemporal dementia (FTD) involves the listed anatomical structures and may therefore produce dysautonomic cardiovascular symptoms. AimTo observe whether or not non-cardiogenic bradycardia was more frequent in a group of patients with FTD than in subjects with mild cognitive impairment or dementia of a different aetiology. Patients and methodOnce patients with primary cardiac arrhythmia were excluded, we registered the heart rates of 258 patients with cognitive symptoms (36 with FTD, 22 with Alzheimer disease, 23 with vascular dementia, 10 with other dementias, and 167 with non-dementia cognitive impairment). ResultsBradycardia (<60 beats/minute) was significantly more frequent in patients with FTD. This difference remained significant after excluding subjects undergoing treatment with a potentially bradycardic effect. Bradycardia was more prevalent in behavioural FTD cases than in cases of the aphasic variant, and we detected a trend toward higher frequency among patients with more pronounced right hemisphere atrophy. Moreover, mean systolic blood pressure in FTD patients was lower than in other participants, and systolic hypotension (<120 and <100mm Hg) was more prevalent. ConclusionBradycardia was more frequent in the FTD sample than in other patients with cognitive symptoms. Further investigations will be necessary before we may consider bradycardia to be a sign supporting diagnosis of FTD or its behavioural variant.

Clinico‐pathological investigations of Rasmussen encephalitis suggest multifocal disease progression and associated focal cortical dysplasia

Rasmussen encephalitis is a devastating neurological disorder characterised by seizures, brain inflammation, and progressive hemispheric atrophy. The objective of the current study was to systematically characterise patterns of structural lesions in children with Rasmussen encephalitis, referred for modified anatomical hemispherectomy at the Tsinghua University Epilepsy Center in Beijing. Seven consecutive patients were investigated with a mean age at operation of 4.5 years, who suffered from medically intractable seizures for a mean of 1.6 years. Foci of abnormally increased T2 signal intensity were observed in all patients. With the exception of one child, all patients presented with progressive unilateral cerebral atrophy. FDG-PET imaging revealed extensive regions of hypometabolism within the affected cerebral hemisphere in 3 of 4 patients. Diagnosis of Rasmussen encephalitis was confirmed histologically, demonstrating CD68 positive microglial nodules, as well as CD3 and CD8 positive Tlymphocytes invading the cerebral parenchyma. An intriguing observation was the heterogenous distribution of patterns of lesions throughout the affected hemisphere, suggesting multifocal manifestation and distinct sequences of disease progression, from discrete foci of inflammatory infiltrates (stage 1) to extensive cortical destruction (stage 4). Atypical hippocampal sclerosis (HS), with neuronal cell loss affecting most prominently the CA4 region (HS type 3 or end folium sclerosis), was evident in 5 of 7 cases. Four hippocampi also showed chronic inflammation. In addition, we observed associated focal cortical dysplasia (FCD; ILAE type IIId) in 4 of 7 children, supporting the concept of acquired and postmigratory FCD pathomechanisms. Postsurgical seizure freedom was achieved in all children with a mean follow-up period of 2.7 years and continuous antiepileptic medication.

Patterns of longitudinal brain atrophy in the logopenic variant of primary progressive aphasia

The logopenic variant of primary progressive aphasia (PPA) is characterised by impaired sentence repetition and word retrieval difficulties. Post mortem studies, amyloid imaging and CSF tau/Aβ measurements suggest Alzheimer’s disease (AD) pathology as the underlying cause. Relatively little is known about patterns of progression in patients with the logopenic variant of PPA. 21 patients (3 with post mortem confirmation of AD and 5 with positive amyloid PIB-PET scans) were studied with longitudinal T1-weighted MR imaging (mean interscan interval 1.2years) using volumetric analysis and voxel-based morphometry (VBM). Baseline imaging showed asymmetrical (left greater than right) involvement of the posterior superior temporal and inferior parietal lobes as well as posterior cingulate and medial temporal lobes. The whole brain rate of volume loss was 2.0% per year with a greater rate of left hemisphere atrophy (2.3%/year) than right hemisphere (1.6%/year). Longitudinal VBM analysis showed increasing involvement of other areas in the left hemisphere (temporal, parietal, frontal and caudate) and atrophy of areas in the right hemisphere that had been involved earlier in the disease in the left hemisphere, particularly posterior cingulate/precuneus. With disease progression there was worsening of anomia, sentence repetition and sentence comprehension but consistent with the spread of imaging changes also deficits in single word comprehension, single word repetition and verbal memory. This study shows that the logopenic variant of PPA remains an asymmetrical disease, with spread through the left hemisphere language network but also involvement to a lesser degree of regions in the right hemisphere that mirror the earlier left hemisphere changes.

Abstract TMP15: Contralateral Hemispheric Atrophy After Major Hemispheric Infarction

Objective: Atrophy of the ipsilateral hemisphere, cerebral peduncle, and contralateral cerebellum predict outcome after stroke; little is known of the effect of stroke on the contralateral hemisphere although it plays an important role in functional reorganization. Methods: All patients who underwent decompressive hemicraniectomy for malignant hemispheric infarction at UH-CMC between 1/2003-12/2009 were analyzed with IRB approval. Patients with adequate CT scans both prior to surgery and after reconstructive cranioplasty were included; those with communicating hydrocephalus, confounding lesions or ≥ 10 mm of midline shift were excluded. Brainlab iPlan® software (Germany) was used to manually trace the infarct, non-infarct ipsilateral and contralateral hemisphere on axial, sagittal and coronal slices. Results: Of 30 patients, 9 met inclusion criteria. The inter-rater reliability of volumetric analyses was excellent (r=0.99). The mean contralateral hemisphere volume at follow-up was significantly reduced compared to the baseline study (465.987 ± 78.32 cc vs 493.429 ±76.92 cc, p&lt; 0.0001, Fisher’s two-tailed t-test ). Contralateral hemispheric atrophy was identified in all patients (mean 27.441 cc, range 4.985 - 42.807 cc) and represented a loss of 5.7% (1.2 - 11.16%) of the original volume. Contralateral hemispheric atrophy was significantly related to the volume of the infarct and the time from stroke onset to decompressive hemicraniectomy but not to the extent of midline shift on the post-operative scan nor &lt;10 mm on the pre-operative scan. Contralateral hemispheral atrophy was identified early and not related to the length of follow-up (median 4.5, range 2-43 months). Conclusions: Contralateral hemispheric atrophy occurs within months of major hemispheral infarction and is related to infarct size and the time to decompressive hemicraniectomy.

The Ultimate Stage of Rasmussen?s Encephalitis

Rasmussen encephalitis (RE) is a rare and progressive disease of childhood. We report the case of 14-year-old girl who presented with recurrent partial and generalized epileptic seizures with mental impairment and progressive left sided hemiplegia. EEG showed slowing of the background activity and paroxysmal discharges of slow waves in the right hemisphere. Serial MRI brain scans revealed an important atrophy of the right hemisphere with dilatation of the ipsilateral lateral ventricle. The residual phase, characterized by decrease in seizure frequency and stabilization in the hemiparesis, constitute the ultimate stage of RE.

Experience of using MRI morphometry in Huntington’s disease

One of the most important inherited neurodegenerative disorders, Huntington’s disease (HD), is characterized by cerebral atrophy, the features of which need to be clarified. MRI morphometry allows assessing quantitatively the atrophy of different brain regions, and this method may be regarded as a potential biomarker of neurodegeneration. We used whole-brain voxel-based morphometry (VBM) and region-of-interest (ROI) morphometry in 24 patients with HD, 10 preclinical HD gene carriers and 9 controls. On whole-brain VBM patients had significantly lower grey matter in the caudate, the putamen, and the pre- and postcentral gyri bilaterally compared to controls, while on ROI morphometry the grey matter volume decrease in patients was seen in the caudate, the putamen and the pallidum bilaterally. In clinically unaffected gene carriers the pallidum, the putamen, and the pre-and postcentral gyri bilaterally were larger and the left pallidum was smaller compared to controls. We found more pronounced atrophy of a dominant hemisphere in patients and gene carriers, as well as negative correlation between basal ganglia and cortical structures volumes and the mutation severity, motor and cognitive impairment.

Bilateral Rasmussen's encephalitis associated with type II focal cortical dysplasia: Dormant 'second' epileptogenic zone in contralateral disease.

Rasmussen's encephalitis (RE) is an inflammatory, probably autoimmune disorder manifested by refractory seizures and progressive deterioration of one cerebral hemisphere [1].Here, we describe the unfortunate history of a girl with a progressive disorder which, upon clinical, neuroimaging, and histopathological evaluation, proved to be bilateral RE associated with type II focal cortical dysplasia. Whether the second pathology is relevant for the extent of the disease is discussed.We demonstrated histopathological evidence of RE and type II FCD in the left hemisphere, which led to EPC on the right hemibody at presentation. In addition, there was unequivocal progressive cortical and subcortical atrophy of the right hemisphere, which accounted for the EPC on the left hemibody. This is highly compatible with RE (+/− FCD) in the right hemisphere as well. Although the association of FCD and RE – as well as the occasional occurrence of bilateral RE – has already been reported [3–5], this is the first such case in which bilateral RE and FCD co-occur.

Rasmussen’s Encephalitis: A Case Report

Abstract We report a case of Rasmussen’s Encephalitis. It is a rare, chronic inflammatory neurological disease of unknown origin that usually affects only one hemisphere of the brain. It is common in children under the age of 10 with average age at disease onset around 6 years but uncommon in adults, adult variant that accounts for about 10% of the cases only. Rasmussen’s Encephalitis is characterized by intractable severe seizures, loss of motor skills and speech, paralysis on one side of the body (dysfunctions associated with the affected hemisphere). Our case is a 21 years old female, presented to emergency department JMPC Karachi with complaint of intractable severe seizures, progressive hemiparesis and deteriorated cognition followed by an episode of encephalitis. Her course of illness was focal seizures and right-sided weakness (hemiparesis) for 5 months. In addition to classical clinical presentation of Rasmussen’s Encephalitis, MRI Brain showed hemispheric atrophy of one cerebral hemisphere and old gliotic changes that further supports diagnosis of Rasmussen’s Encephalitis.

Evolución posquirúrgica en pacientes con encefalitis de Rasmussen operados por hemisferotomía

Rasmussen's encephalitis (RE) is a progressive pathology affecting the brain that causes unilateral hemispheric atrophy, neurological dysfunction and refractory epilepsy. Hemispherotomy is considered the most effective treatment today, but some cases present certain peculiarities that can seriously affect the decision to go ahead with this procedure. To evaluate the post-operative progress made by children with RE who have undergone hemispherotomy surgery, and who, in the pre-operative assessment, presented certain characteristics that complicated the decision to perform surgery. The sample selected for study consisted of the cases of RE attended in the Hospital Sao Paulo between 2003 and 2012 who, in the pre-surgery evaluation, presented clinical, electroencephalographic or neuroimaging evidence of involvement of both brain hemispheres, compromise of the dominant brain hemisphere, absence of severe neurological deficit and absence of criteria for refractory epilepsy and atypical crises. The post-operative assessment of the epileptic seizures was evaluated using the Engel scale; motor function was analysed with the Gross Motor Function Classification System and Manual Ability Classification System scales, and language was evaluated clinically. Six cases were selected (four girls), with a mean age at clinical onset of 3.3 ± 1.2 years (range: 2-7 years) and a mean age at hemispherotomy of 6.7 years (range: 2.3-16.5 years). The mean post-surgery follow-up time was three years (range: 0.5-7.2 years). In the post-surgery evaluation of the epileptic seizures, four cases were classified as Engel class I (66%); there was some improvement in motor functioning in five of them, and language improved in all cases. Hemispherotomy must be considered an efficient option for treatment in children with RE.