A Closer Investigation of the Synchronous Bilateral Pattern of MRI Lesions in Acute Necrotizing Encephalopathy Type 1.

We observed a lesion pattern in a series of 4 cases of RANBP2-mutation-linked acute necrotizing encephalopathy, which appears to be specific for this condition. The setting of synchronous bilateral mammillary, amygdaloid, and lateral geniculate lesions, along with claustro-parahippocampal lesions, can serve as a diagnostic tool in this condition. We add several further details to the MR imaging features of the typical brain lesions encountered in this disease.

Neurolymphomatosis as a late relapse of non-Hodgkin's lymphoma detected by 18F-FDG PET/CT: a case report / Neurolinfomatosis como recaída de un linfoma no Hodgkin detectada con 18F-FDG PET/TAC: a propósito de un caso

Neurolymphomatosis is a rare condition defined as an infiltration of nerves, nerve roots or nervous plexuses by haematological malignancy. Its diagnosis may sometimes be difficult with conventional imaging techniques. This paper aims to emphasize the importance of this entity and the role of 18F-FDG PET/CT in this indication. We present the case of a 53-year-old male who complained of sharp pain in his right hip and right leg paresthesia after 2 years of complete remission from Non-Hodgkin's lymphoma. Physical examination and CT scan were negative and the lumbar MRI showed protrusion of L5-S1 disc. Physiotherapy, nonsteroid antiinflammatory drugs and steroids were inefficient. PET/CT was performed four months after the onset of the symptoms, revealing focal FDG uptake in the right S1 nerve root and linear FDG uptake along the right sacral plexus suggesting relapse. This was confirmed by histology (AU)

La neurolinfomatosis es una entidad rara definida por la infiltración de los nervios, raíces o plexos nerviosos por un proceso hematológico maligno, siendo en ocasiones difícil de diagnosticar mediante técnicas de imagen convencionales. La finalidad del caso es llamar la atención sobre su importancia y el papel de la 18F-FDG PET/TAC. Presentamos el caso de un varón de 53 años con dolor en la región de la cadera derecha y parestesias en la pierna derecha tras 2 años de remisión completa de un linfoma no Hodgkin. El examen físico y la TAC fueron negativos, mostrando la RM lumbar una protrusión discal en L5-S1. El tratamiento con fisioterapia y con antiinflamatorios no esteroideos y esteroideos fue ineficaz. La PET/TAC realizada a los 4 meses reveló una captación focal de FDG en la raíz del nervio S1 derecho y una captación lineal a lo largo del plexo sacro derecho sugestivo de recaída del linfoma, lo que fue confirmado por la histología (AU)

Neurolymphomatosis as a late relapse of non-Hodgkin's lymphoma detected by 18F-FDG PET/CT: a case report.

Neurolymphomatosis is a rare condition defined as an infiltration of nerves, nerve roots or nervous plexuses by haematological malignancy. Its diagnosis may sometimes be difficult with conventional imaging techniques. This paper aims to emphasize the importance of this entity and the role of (18)F-FDG PET/CT in this indication. We present the case of a 53-year-old male who complained of sharp pain in his right hip and right leg paresthesia after 2 years of complete remission from Non-Hodgkin's lymphoma. Physical examination and CT scan were negative and the lumbar MRI showed protrusion of L5-S1 disc. Physiotherapy, nonsteroid antiinflammatory drugs and steroids were inefficient. PET/CT was performed four months after the onset of the symptoms, revealing focal FDG uptake in the right S1 nerve root and linear FDG uptake along the right sacral plexus suggesting relapse. This was confirmed by histology.

Clinical, radiological and genetic aspects of leukodystrophies.

The authors summarize the pathomechanism of the myelination process, the clinical, radiological and the genetical aspects of the leukodystrophies, as in 18q deletion syndrome, adrenoleukodysrtophy, metachromatic leukodystrophy, Pelizaeus-Merzbacher leukodystrophy, Alexander disease and olivo-ponto-cerebellar atrophy (OPCA).

Progressive multifocal leukoencephalopathy in an HIV-infected child.

Progressive multifocal leukoencephalopathy is an infection of the immunosuppressed, especially of AIDS, patients. The disease is caused by the JC virus and is exceptionally rare in children. The diagnosis is based on MRI and on the detection of JC virus DNA in the cerebrospinal fluid. Progression is relentless in most cases. The only treatment of proven benefit is restoration of the immune system by highly active antiretroviral therapy. We report the case of a 15S-year-old HIV-infected boy. After several months of fatigue he developed apathy, head tilt, diplopia, motor apraxia and unsteady gait. Physical examination revealed mild cerebellar signs. MRI showed a 30-mm large, non-enhancing, hyper-intense area in the right cerebellar hemisphere and the middle cerebellar peduncle. JC virus DNA was detected in the cerebrospinal fluid. Two weeks later the MRI showed progression. The patient's condition rapidly worsened and he died four months after the onset of the disease. Autopsy revealed widespread lesions of the cerebellar hemispheres and the brainstem. The case presented is peculiar owing to the young age of the patient, the unusual localization and the unifocal nature of the lesion.

Bilateral striatal lesion associated with varicella.

Bilateral striatal lesion is characterised by a specific clinical syndrome (encephalopathy with rigidity, irritability, variable pyramidal, and extrapyramidal symptoms, speech abnormalities) and symmetrical lesion of the basal ganglia including the caudate nucleus, the putamen, and occasionally other nuclei. We report three cases in whom bilateral striatal lesion developed in association with varicella. Each patient recovered completely and showed no signs of cognitive deficiency, chorea or hyperkinetic syndrome, all of which have been reported as sequelae of BSL associated with other conditions. These cases suggest that bilateral striatal lesion may be an immune-mediated complication of varicella.

Bilateral epilepsy surgery in a 4-year-old child.

OBJECTIVE: Reports on bilateral epilepsy surgical interventions are anecdotal because of the possible neurological deficits caused by them. METHODS: We report on a four-year-old amaurotic child with catastrophic epilepsy due to bilateral occipital cortical dysplasia. After video-EEG monitoring and intraoperative electrocorticography he underwent a two-step bilateral occipital lobectomy. RESULTS: The first resection resulted in only temporary seizure cessation; however, he became seizure-free after the second operation (follow-up: 20 months). CONCLUSION: Patients with catastrophic epilepsy due to bilateral epileptogenic lesions but without a high risk of additional postsurgical deficit may be good candidates for epilepsy surgery.

Clinical differences in patients with unilateral hippocampal sclerosis and unitemporal or bitemporal epileptiform discharges.

PURPOSE: To investigate factors determining the presence of bilateral interictal epileptiform discharges (IEDs) in temporal lobe epilepsy (TLE) with unilateral hippocampal sclerosis (HS). METHODS: We analysed data of 243 TLE patients with unilateral HS who had long-term video-EEG. Eighty-one patients (33%) had bitemporal IEDs. RESULTS: We categorised patients into a unilateral group (UG), a bilateral group (BG) according to presence of bitemporal IEDs. We found no difference between UG and BG regarding epilepsy duration, secondarily generalised seizures, and history of febrile seizures. Mean seizure frequency was significantly higher in the BG (UG: 7.7+/-14.7 seizures/month; BG: 13+/-35 seizures/month, P=0.01). We found a significant correlation between late epilepsy onset and the presence of bitemporal IEDs. The mean age at epilepsy onset in UG was 10.1+/-7.9 years, while in BG it was 13+/-9.2 years (P=0.02). CONCLUSIONS: The traditional concept of the evolution of mirror focus cannot apply for humans because the duration of epilepsy does not influence the evolution of bitemporal IEDs. Other factors, i.e. age at onset and seizure frequency may play a role in this process. The association between the higher seizure frequency and mirror foci indicates that the development of mirror focus depends on seizures and not on a progressive 'interictal' epileptogenesis.

Right hippocampal sclerosis is more common than left after febrile seizures.

Temporal lobe epilepsy (TLE) is frequently associated with hippocampal sclerosis (HS) and a history of febrile convulsions (HFC). The authors investigated 292 patients with TLE due to HS. Left HS occurred more frequently (57%) than right HS (43%, p = 0.01). Forty-seven percent of the patients had HFC. In patients with right HS, HFC occurred in 59.6%, whereas in patients with left HS, HFC was present in 37.5%, showing a highly significant lateralization difference.

Automatisms with preserved responsiveness and ictal aphasia: contradictory lateralising signs during a dominant temporal lobe seizure.

The 25-year-old right-handed woman suffering from temporal lobe epilepsy (TLE) was referred to our centre for presurgical evaluation. MRI showed a right-sided hippocampal sclerosis. During video-EEG-recorded seizures, abdominal aura was followed by oral automatisms, during which she was completely reactive to external stimuli, although she was unable to speak. Ictal EEG showed right temporal seizure pattern, without contralateral propagation. She had abnormal speech postictally. Speech-activated functional transcranial Doppler sonography revealed right-sided speech dominance. She has become seizure free after a right-sided amygdalo-hippocampectomy. In our patient, contradictory clinical ictal lateralising signs (automatisms with preserved responsiveness vs. ictal and postictal dysphasia) occurred during right-, speech-dominant-sided seizures. This is the first report when automatisms with preserved consciousness occurred during a seizure originating and involving the speech-dominant hemisphere.

Rhombencephalosynapsis: clinical findings and neuroimaging in 9 children.

Rhombencephalosynapsis is a rare congenital abnormality characterised by dorsal fusion of the cerebellar hemispheres, agenesis or hypogenesis of the vermis, fusion of dentate nuclei and superior cerebellar peduncles. We describe 9 children, aged 1.5 to 6 years, with rhombencephalosynapsis. Isolated rhombencephalosynapsis was found in 2 patients, hydrocephalus in 3 children and another 3 children had ventriculomegaly. Additional supratentorial abnormalities were documented in 5 patients. Clinical findings ranged from mild truncal ataxia and normal cognitive abilities to severe cerebral palsy and mental retardation. No correlation between clinical findings and magnetic resonance imaging could be established so far.

Misleading lateralization by ictal SPECT in temporal lobe epilepsy-- a case report.

A 28-year-old woman with temporal lobe epilepsy underwent presurgical evaluation. Scalp-EEG showed non-localizing seizure patterns. MRI revealed a right hippocampal sclerosis. Ictal HMPAO-SPECT showed a marked left temporal hyperperfusion. Video-EEG monitoring with foramen ovale electrodes (FOE) showed an initial seizure pattern which appeared in the right FOE and which shifted to the left 8 s after clinical onset. Three years after a right temporal lobectomy, the patient is seizure-free. In conclusion, although the ictal SPECT suggested a left temporal seizure focus, the intracranial EEG and the postoperative seizure-freedom confirmed the right-sidelocation of the epileptogenic region. A rapid right-left seizure spread explains the mechanism of falsely lateralizing ictal SPECT.

Hypertension and clusters of risk factors in different stroke subtypes (an analysis of Hungarian patients via Budapest Stroke Data Bank).

Our aim was to determine the frequency of hypertension in the Hungarian stroke population, and to compare it with the data of other stroke registries. We attempted to find characteristic cluster-like associations between hypertension and another nine risk factors in different stroke subtypes and to ascertain the role of hypertension in leukoaraiosis, in early mortality, in stroke recurrence and in the case fatality rate up to 10 years. Risk factor profile of 500 unselected acute stroke cases of the Budapest Stroke Data Bank were analysed. We compared data of hypertensive stroke patients to those of unaffected ones. LIFEREG procedure of the SAS software package, cluster analysis, logistic regression, Pearson's correlation coefficient and Student's t-test were used as statistical methods. Hypertension was documented in 75% of the patients. The largest clusters were formed by the following groups: atherosclerotic stroke, hypertension with ischaemic heart disease; lacunar stroke and haemorrhage, hypertension with elevated serum cholesterol, cardiogenic embolism, ischaemic heart disease with atrial fibrillation. The case fatality rate was significantly higher in the group of hypertensive patients with ischaemic heart disease relative to those without it during the 10 years follow-up period. In the leukoaraiosis-group, systolic blood pressure was significantly higher than in the non-leukoaraiosis group. The rate of hypertension was higher than in other stroke registries. Hypertension appears to be the most frequent risk factor in stroke patients but case fatality rate is determined by presence or absence of ischaemic heart disease.

Orgasmic aura originates from the right hemisphere.

The authors present a patient with right mesiotemporal epileptogenic region who experienced orgasmic epileptic aura. Twenty-two similar published cases were also evaluated. Among 15 patients with unilateral EEG foci, 13 (87%) had right and 2 (13%) had left focus. All of the nine patients who had sufficient data on ictal onset area had right-sided seizure onset. The authors suggest that orgasmic aura is an ictal lateralizing sign to the right hemisphere.

[Surgically treatable epilepsy--a review]. / Mútéttel gyógyítható epilepszia--összefoglaló tanulmány.

20-25% of epileptic patients do not become seizure free on adequate drug therapy. In 25-50% of patients with intractable epilepsy, the brain area responsible for seizures is well localizable and does not involve eloquent regions. In these patients, the surgical excision of the epileptic focus may lead to relief from seizures. In Hungary, there may be 5-6000 patients who needs an epilepsy surgery, but till now only 200 patients with chronic epilepsy underwent a surgical procedure. In the surgically remediable epilepsies, the operation is not a "ultima ratio". Concerning these syndromes, if 2-3 adequate antiepileptic drugs do not lead to seizure freedom within 1-3 years after the epilepsy onset, then a presurgical evaluation is necessary. The most common surgically remediable epilepsy is the temporal lobe epilepsy in which 60-90% of drug-resistant patients could be surgically cured. In lesional neocortical epilepsies 50-80% of patients become postoperatively seizure free. In childhood hemispheric epilepsies, the surgery could lead to seizure freedom in 70-80% of patients. The basic tools of the presurgical evaluation are the detailed history, the high resolution-MRI, the video-EEG monitoring, and the neuropsychological assessment. These investigation methods are usually enough to evaluate the necessity of the surgery and the postoperative outcome as well as to plan the localization and the extension of the resection. In some cases, ictal SPECT, PET, or video-EEG monitoring with intracranial electrodes could also be necessary in order to localize the epileptic focus.

[Risk factors for early death and recurrence in stroke]. / A stroke korai halálozását és az ismételt stroke kialakulását meghatározó tényezók.

The early case fatality rate (28-day mortality) in 500 acute stroke patients of Budapest Stroke Data Bank was 17% and it was 25% within the first year with an increase between 1-3% annually. In the group with early death significantly higher age (p < 0.0001), more serious neurological symptoms, impaired consciousness (p < 0.0001), and higher volume of the lesions (p < 0.0001) have been detected. In this group significantly more stroke in the territory of vertebrobasilar system, a higher number of ischemic heart disease, and atrial fibrillation have been registered. According to the stroke subtypes there were significantly more cardiogenic embolisms, and hemorrhages, but less lacunar infarcts. The level of blood sugar at admission, and the rate of low platelets were higher among patients, who died within 28 days. Significantly higher percentage of the regions in the right frontal, right parietal, right occipital lobes and right thalamus have been affected in the early death group than in the survivors' group, however no such difference have been found in the lesions of the left homological structures. Severity of recurrent strokes have been more serious, and among patients with repetitive stroke significantly more ischemic heart diseases have been demonstrated. Atherosclerosis is the main risk for a new cerebrovascular accident. The case recurrent rate in the first 28 days is 7%, and it is 16% within the first year. The annual increase was between 1-5%. 71% of the recurrent strokes had the same mechanism as the first one.

Can intravascular lymphomatosis mimic sinus thrombosis? A case report with 8 months' follow-up and fatal outcome.

We report a case of intravascular lymphomatosis of the brain with 8 months' follow-up and fatal outcome. Several MRI investigations revealed variegated, rapidly changing infarct-like lesions and invasion of the walls of the superior sagittal sinus and deep veins. When disturbances of the venous outflow are detected with multifocal infarct-like lesions, intravascular lymphomatosis should be considered in the differential diagnosis. Brain biopsy may ensure the proper diagnosis ante mortem, but failure of biopsy is frequent, as in our case.

Kallmann syndrome in three unrelated women and an association with femur-fibula-ulna dysostosis in one case.

We describe three unrelated women with hypogonadotropic hypogonadism and anosmia; that is, Kallmann syndrome. Absence of olfactory bulbs and tracts and different degrees of asymmetric dysplasia of olfactory sulci were demonstrated by MRI. Both the father of Case 1 and the maternal aunt of Case 3 had anosmia, thus autosomal dominant inheritance seems to be likely. Patient 2 had Kallmann syndrome and FFU (femurfibula-ulna) dysostosis as a sporadic occurrence in her family.