Hemispheric Atrophy Research Articles

Botulinum toxin treatment of facial myoclonus in suspected Rasmussen encephalitis

Patients with Rasmussen encephalitis (RE) may develop a variety of involuntary movements. We report a 26-year-old woman who presented with a 3-year history of progressive, continuous myoclonus of the left side of the face and left arm as well as left spastic hemiparesis. Magnetic resonance imaging of the brain showed right hemisphere and basal ganglia atrophy, and 24-hour electroencephalogram demonstrated diffuse slowing with random sharp waves in both hemispheres. An 18-fluoro-deoxy-glucose positron emission tomography scan indicated hypometabolism of the right cerebral hemisphere, including basal ganglia and thalamus. We successfully treated her myoclonus with injections of botulinum toxin A into the left zygomaticus muscle.

Peroxisome proliferator-activated receptor-γ-agonist, rosiglitazone, promotes angiogenesis after focal cerebral ischemia

Peroxisome proliferator-activated receptor-γ (PPAR-γ) agonist, rosiglitazone, not only improves insulin resistance in patients with type II diabetes but also exerts a broad spectrum protective effects in variable animal models of neurologic or cardiovascular diseases. We studied the effect of rosiglitazone on angiogenesis and neurological recovery after focal cerebral ischemia. Rosiglitazone (3 mg/kg or 0.3 mg/kg, p.o.) was administered for 7 days prior to and 3 days after the induction of focal ischemia (total 10 days) in adult rats. The rosiglitazone-treated group showed the enhanced neurologic improvement, the reduced infarction volume compared to the ischemia-vehicle group with dose dependency, and the reduced hemispheric atrophy. Rosiglitazone treatment reduced TUNEL +/activated caspase-3 + cells, MPO +/Ox-42 + inflammatory cell infiltrations, caspase-3 activity, and Bax + cells, as compared to the ischemia-vehicle group. The vascular surface area, the vascular branch points, the vascular length, and the number of BrdU + endothelial cells were significantly increased in the rosiglitazone group compared with the ischemia-vehicle group. Rosiglitazone increased eNOS expression around the ischemic margin with downregulation of FasL. Here, we show that rosiglitazone treatment enhances angiogenesis and functional recovery with dose-dependent induction of ischemic tolerance.

Utility of Ictal SPECT in the Presurgical Evaluation of Rasmussen's Encephalitis

Rasmussen's encephalitis is a progressive disease characterized by severe progressive neurological and cognitive deficits. It is a difficult condition to treat with conventional medical treatment and requires, in most of the cases, surgical intervention. We present the case of a patient with Rasmussen's encephalitis who presented with epilepsia partialis continua, who did not respond to antiepileptic treatment and was evaluated for possible surgical intervention. The electroencephalographic studies revealed bihemispheric involvement during seizures, although magnetic resonance imaging (MRI) showed a marked right hemispheric atrophy. Hyperperfusion over the right hemisphere was seen with the performance of ictal single photon emission computed tomography (SPECT), which finally guided a right functional hemispherectomy and subsequent seizure control. Ictal SPECT could be a useful tool in the armamentarium of tools used for the localization of the epileptogenic focus in patients with Rasmussen's encephalitis and other localization-related epilepsies.

A case of Dyke-Davidoff-Masson syndrome in Korea

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare condition characterized by asymmetry of cerebral hemispheric growth with atrophy on one side, ipsilateral compensatory osseous hypertrophy, and contralateral hemiparesis. We experienced a 17 month-old male who presented with left focal clonic or tonic-clonic seizures accompanied by left hemiparesis and developmental delay. Brain MRIs demonstrated progressive atrophy of the right cerebral hemisphere with dilatation of the lateral ventricle, expansion of the ipsilateral frontal sinus with calvarial thickening, and elevation of the petrous pyramid and orbital roof. Brain SPECT showed a decreased volume of the right hemisphere with reduced blood flow. We therefore report a case of DDMS with a review of the literature.

Embriopatía por ácido valproico con malformaciones graves del sistema nervioso central

Embryogenetic disorders are one of the most serious problems in the life of an epileptic. Over the last few decades many antiepileptic drugs, including valproic acid, have been shown to have teratogenic properties. Embryopathy due to valproate, also known as fetal valproate syndrome, is a well-known and documented example of these conditions. We report the case of a preterm newborn infant who, at birth, exhibited a syndrome characterised by facial dysmorphia, gingival hyperplasia, neurological hyperexcitability and multiple malformations, the most striking of which was the presence of predominantly temporal atrophy in the left brain hemisphere. The most significant event in the medical history of the case was the mother's taking valproate in monotherapy throughout the entire period of gestation as treatment for generalised idiopathic epilepsy that was diagnosed during adolescence. Screening precluded the most common metabolic, hereditary or infectious causes that can cause embryopathies. The mother's history of taking valproic acid and the specific findings that coincided in the peculiar embryopathy of this patient enabled us to link them.

False Lateralization of Mesial Temporal Lobe Epilepsy by Noninvasive Neurophysiological Examinations-Case Report-

Mesial temporal lobe epilepsy (mTLE) is the most common form of symptomatic localization-related epilepsy and is surgically remediable. Lateralization of the seizure onset is particularly important to determine from a surgical perspective. A 39-year-old woman with intractable mTLE first exhibited seizure at the age of 3 years. She experienced epigastric sensation and placed her right hand on her abdomen before falling backward. Although interictal scalp electroencephalography (EEG), sphenoidal scalp ictal EEG, and magnetoencephalography showed right temporal side focus, computed tomography and magnetic resonance imaging showed atrophy of the left cerebral hemisphere. Single photon emission computed tomography with technetium-99m ethyl cysteinate dimmer and I-123 iomazenil showed obscure focus on the left side. As a discrepancy existed between the results of neurophysiological examinations and imaging, we performed subdural electrode implantation on the bilateral temporal lobe. Although a bemegride-induced seizure arose from the right side during the subdural recording, the onset of 5 habitual seizures was observed in the left hippocampus. On the basis of these results, the seizure was diagnosed as left mTLE, and left anterior temporal lobectomy and amygdalohippocampectomy were performed. The patient has been free from the seizures for more than 1.5 years of follow up. Bilateral subdural electrode measurement of habitual seizures is indispensable for clarifying the actual focus when a discrepancy exists between neuroimaging and noninvasive neurophysiological examinations.

Atypical case of hemiconvulsions‐hemiplegia‐epilepsy syndrome revealing contralateral focal cortical dysplasia

Hemiconvulsions‐hemiplegia‐epilepsy syndrome (HH/HHE) is a rare epileptic syndrome consisting of a prolonged unilateral convulsion producing a persisting hemiplegia, sometimes followed by epilepsy. We report on a 13‐month‐old male who presented with febrile left‐sided HH syndrome with right hemispheric unilateral cytotoxic oedema followed by hemispheric atrophy on magnetic resonance imaging (MRI). Six months later the child progressively developed refractory focal epilepsy, including right hemiclonic seizures, and nearly continuous left frontal rhythmic spikes, suggesting the presence of a focal cortical dysplasia (FCD). A repeat MRI at 2 years of age showed left frontal FCD. This unusual case of dual pathology ‐ right HH syndrome and left FCD ‐ suggests that some other factor than the malformation determined the prolonged status and brain atrophy. The kinetics of regional cortical maturation could explain this unusual condition.

Atypical case of hemiconvulsions-hemiplegia-epilepsy syndrome revealing contralateral focal cortical dysplasia

Hemiconvulsions-hemiplegia-epilepsy syndrome (HH/HHE) is a rare epileptic syndrome consisting of a prolonged unilateral convulsion producing a persisting hemiplegia, sometimes followed by epilepsy. We report on a 13-month-old male who presented with febrile left-sided HH syndrome with right hemispheric unilateral cytotoxic oedema followed by hemispheric atrophy on magnetic resonance imaging (MRI). Six months later the child progressively developed refractory focal epilepsy, including right hemiclonic seizures, and nearly continuous left frontal rhythmic spikes, suggesting the presence of a focal cortical dysplasia (FCD). A repeat MRI at 2 years of age showed left frontal FCD. This unusual case of dual pathology--right HH syndrome and left FCD--suggests that some other factor than the malformation determined the prolonged status and brain atrophy. The kinetics of regional cortical maturation could explain this unusual condition.

Surgery: All Ages

Surgery: All Ages

Granulocyte colony-stimulating factor enhances angiogenesis after focal cerebral ischemia

Granulocyte colony-stimulating factor (G-CSF) is a neuroprotective agent and activates endothelial proliferation and bone marrow stem cell mobilization. We studied the effect of G-CSF on angiogenesis and neurological recovery after focal cerebral ischemia. After the induction of transient focal ischemia in rats, G-CSF (50 μg/kg/day, i.p.) or PBS was administered for 3 days. We evaluated the functional recovery, infarct volume, inflammatory infiltration, blood–brain barrier (BBB) disruption, hemispheric atrophy, protein expressions of endothelial nitric oxide synthase (eNOS) and angiopoietins, and the therapeutic time window of G-CSF administration. We then analyzed endothelial cell proliferation, the vascular surface area, the number of branch points, and the vascular length. G-CSF treatment improved behavioral recovery and reduced the infarct volume, the inflammatory infiltration, the BBB disruption, and the hemispheric atrophy. G-CSF injection, starting at 2 h, 1 day, or 4 days after ischemia, resulted in a better functional recovery and a greater reduction in hemispheric atrophy than injection starting at day 7. The vascular surface area, the vascular branch points, the vascular length, the number of BrdU + endothelial cells, and eNOS/angiopoietin-2 expression were significantly increased in the G-CSF group compared with the ischemia-only group. G-CSF injection starting at 1 day induced larger endothelial proliferation compared with injection starting at 7 days. In this study, we provide evidences that G-CSF enhances the angiogenesis and reduces the ischemic damage, which promotes the long-term functional recovery.

Cognitive Affective Psychosis Syndrome in a Patient With Sporadic Olivopontocerebellar Atrophy

Cognitive Affective Psychosis Syndrome in a Patient With Sporadic Olivopontocerebellar Atrophy

Cognitive Affective Psychosis Syndrome in a Patient With Sporadic Olivopontocerebellar Atrophy

Cognitive Affective Psychosis Syndrome in a Patient With Sporadic Olivopontocerebellar Atrophy

Expanding the Spectrum of Acquired Cerebral Hemiatrophy

The authors report a 50-year-old man with progressive left hemispheric atrophy, cognitive decline, infrequent seizures, and spells suggestive of complicated migraine. They discuss the association between migraine and cerebral hemiatrophy and suggest that the spectrum of acquired cerebral hemiatrophy may be broader than what has been commonly recognized.

Parry-Romberg syndrome with fatal brain stem involvement

We report the case of a 4 year-old boy with Parry-Romberg syndrome who had intractable seizures, progressive cerebral hemisphere atrophy, and fatal brain stem involvement.

Primary progressive aphasia: Reversed asymmetry of atrophy and right hemisphere language dominance

Primary progressive aphasia (PPA) is almost always associated with asymmetrical left hemisphere degeneration. The authors report that right hemisphere atrophy in a patient with PPA was associated with atypical right hemisphere dominance for language. This suggests that neuronal damage in PPA is therefore tightly linked to the underlying anatomy of the language network.

Longitudinal Diffusion Changes in Cerebral Hemispheres after MCA Infarcts

Diffusion tensor imaging can be used in vivo to assess the longitudinal and regional microstructural changes occurring after middle cerebral artery (MCA) infarcts in humans. Nine patients were investigated 1 week (D7), 1 (M1), 3 (M3), and 6 months (M6) after the occurrence of an isolated MCA infarction. First, an overall analysis was performed using histograms of mean diffusivity (MD) and fractional anisotropy (FA) in each hemisphere. Thereafter, the regional pattern of diffusion changes was investigated voxel by voxel with statistical parametric mapping 99. In the hemisphere ipsilateral to the infarction, histogram analysis revealed a significant decrease in FA between D7 and M6 associated with a progressive increase in MD from D7 to M3. Remote from the MCA territory, the voxel by voxel analyses detected a significant increase in MD within the thalamus at M3 and M6 and a reduction in FA along the pyramidal tract at M6. In the contralateral hemisphere, between D7 and M6, a significant hemispheric atrophy was observed in association with a global reduction in anisotropy, in the absence of distinctive regional diffusion changes. These results suggest that micro- and macrostructural tissue modifications can be detected with diffusion tensor imaging in regions remote from the ischemic area in both hemispheres.

Selective Atrophy of Left Hemisphere and Frontal Lobe of the Brain in Old Men

In this study, volumes of the whole brain, hemispheres, and frontal lobes of young and elderly adults were quantified by an automated method. Effects of age, sex, and side on absolute and relative volumes of the brain structures were evaluated. Compared with the young group, elderly participants showed a 15% volume loss in the whole brain and hemispheres, and a 22% volume loss in the frontal lobes. The relative volume of the left hemisphere in the elderly group decreased more than that of the right hemisphere. Elderly men showed significantly greater left hemisphere and left frontal lobe volume losses than did elderly women, indicating that the larger left hemisphere relative volume reduction is largely contributed to by selective atrophy of the left frontal lobe volume in elderly men. These results may reflect age- and sex-related functional deterioration in the left brain.

Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: A European consensus statement

Rasmussen encephalitis (RE) is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction and intractable seizures. Recent data on the pathogenesis of the disease, its clinical and paraclinical presentation, and therapeutic approaches are summarized. Based on these data, we propose formal diagnostic criteria and a therapeutic pathway for the management of RE patients.

Neuropathological spectrum of Rasmussen encephalitis

Rasmussen encephalitis (RE) is a chronic epileptic disorder of unknown etiology, and is clinically characterized by progressive neurological deterioration, focal seizures often progressing to intractable epilepsy, cognitive decline and hemispheric atrophy. We report the spectrum of neuropathological changes seen in RE, and discuss the evolutionary mechanisms of this disease. Surgically obtained specimens from RE patients diagnosed during 2002-2004 at this institute were evaluated for the presence and extent of different histopathological features of RE. The H&E and immunohistochemistry stained slides were also evaluated for the type and distribution pattern of inflammatory infiltrates, along with a semiquantitative evaluation for the severity of inflammation. Four cases of RE were admitted during the study period, all of which presented with partial seizures with successive deterioration to intractable epilepsy. The age at onset varied between 5 and 10 years (mean 7.8 years), with three male and one female patients. Subsequently, all four patients underwent hemispherotomy. Histopathological features of perivascular lymphocytic infiltrate, neuronal loss, microglial nodules, and reactive astrocytosis, with or without evidence of neuronophagia confirmed a diagnosis of RE. These cases also had varying degrees of T-cell-rich (CD3-positive) inflammatory infiltrates and CD68-immunopositive microglial proliferation. It was observed that the severity of inflammation had a trend to inversely correlate with the duration of symptoms. It is proposed that an accurate evaluation and histopathological grading of these lesions may possibly have a role in patient prognostication.

Brain atrophy evaluated by computed tomography in independent and institutionalized hip fracture patients

Purpose: To examine if there is an association between brain computed tomography (CT) findings and place of residence in a series of hip fracture patients.Method: The CT scans taken immediately after hip fracture of 215 patients (mean age 81.6 years) living in their own homes or otherwise independently (home-dwelling group) and 95 patients (mean age 82.5 years) permanently institutionalized (institutionalized group) were analysed.Results: The institutionalized patients had significantly more cortical cerebral (frontal, p = 0.004; temporal, p = 0.007; parietal, p < 0.001) and central cerebral (third ventricle width, p < 0.001; frontal horn width, p < 0.001; midbody width, p < 0.001) atrophy than the home-dwelling ones. This was also true of atrophy in the white-matter (WM) area (p < 0.001). The institutionalized patients also had more atrophy of the cerebellar hemisphere (atrophy of the cerebellopontine angle cistern, p = 0.002, greater fourth ventricle width, p = 0.020). No significant difference was seen in the incidence of brain infarcts.Conclusions: Hip fracture patients living in institutions have more brain atrophy than those living independently. The brain atrophy may be one factor in the multiple mechanism underlying their institutional admission.