A Pacchionian Puzzle.

A 73-year-old male with a history of chronic ataxia presented with transient facial droop to the Emergency Department. A CT angiogram and MRI with diffusion weighted imaging (DWI) were negative for stroke. However, incidental note was made of numerous giant arachnoid granulation pits in the posterior fossa predominantly involving the left occipital bone (Figure 1). These arachnoid pits demonstrated multiple foci of herniation of the adjacent cerebellar parenchyma into the pits with gliosis of the herniated parenchyma and focal encephalomalacia of the subjacent cerebellar parenchyma. Review of bone windows on a remote CT brain performed almost 13 years earlier confirmed this to be a longstanding abnormality (Figure 2). The patient's physical exam was suggestive of cerebellar ataxia with left-sided dysmetria on finger to nose testing and a wide-based unsteady gait.

The impact of MEG results on surgical outcomes in patients with drug-resistant epilepsy associated with focal encephalomalacia: a single-center experience.

PURPOSE: To analyze the impact of magnetoencephalography (MEG) results on surgical outcomes in patients with drug-resistant epilepsy secondary to encephalomalacia. METHODS: We retrospectively reviewed 121 patients with drug-resistant epilepsy associated with encephalomalacia who underwent MEG followed by resection surgery. Patients were subdivided into concordant MEG group and dis-concordant MEG group for analysis based on whether the MEG results were in concordance with epileptogenic zones or not. RESULTS: 121 patients were included in the present study. The MEG spike sources of 73 (60.33%) patients were in concordance with epileptogenic zones while the MEG spike sources of the other 48 (39.67%) were in dis-concordance with epileptogenic zones. Favorable seizure outcomes were achieved in 79.45% (58 of 73) of patients with concordant MEG results while only 62.50% (30 of 48) of patients with dis-concordant MEG results were seizure free with a follow-up of 2-10 years. The differences of seizure-free rate between patients with concordant MEG results and dis-concordant MEG results were statistically significant. For patients with concordant MEG results, bilateral lesions on MRI are the only independent predictor of unfavorable seizure outcomes. For patients with discordant MEG results, duration of seizures is the only independent predictor of unfavorable seizure outcomes. CONCLUSIONS: Concordant MEG results are associated with favorable seizure outcomes. Bilateral lesions on MRI independently predict unfavorable seizure outcomes in patients with concordant MEG results while longer seizure durations independently predict unfavorable seizure outcomes in patients with dis-concordant MEG results.

Multicystic encephalomalacia: An autopsy report of 4 cases

Multicystic encephalomalacia is varying sized cystic lesions in the brain encountered in developing fetuses or infants. These cysts start at the periventricular area and may extend onto the cortex. The cause of the formation of these cystic lesions is secondary to an ischemic or hypoxic insult, which leads to liquefactive necrosis and subsequent formation of gliotic cyst walls having an admixture of microglia. We discuss four autopsy cases that had multicystic encephalomalacia to highlight the scenarios in which these lesions are encountered.

Surgical outcomes and prognostic factors of drug-resistant epilepsy secondary to encephalomalacia.

OBJECTIVE: To evaluate long-term outcomes and prognostic factors in patients who underwent surgical resection for drug-resistant epilepsy secondary to encephalomalacia. METHODS: A total of 143 patients with drug-resistant epilepsy who underwent surgical resection with a follow-up of at least 5 years were included. Seizure outcomes were evaluated based on the International League Against Epilepsy classification. Univariate analysis and a multivariate logistic regression model in a backward fashion were used to identify the potential predictors of seizure outcomes. RESULTS: Three months after surgery, 102 of 143 (71.3%) patients had achieved favorable seizure outcomes. Five years after surgery, 107 of 143 (74.8%) patients had achieved favorable seizure outcomes. Changes in the postoperative seizure status were observed in 22 of 143 (15.4%) patients during follow-up, but the difference in the seizure-free rate between 3 months and 5 years after surgery was not significant. Univariate and multivariate analyses revealed that only a monthly seizure frequency of >30 seizures (odds ratio = 3.42, 95% confidence interval = 1.19-9.76) and bilateral ictal onset rhythms (odds ratio = 4.46, 95% confidence interval = 1.61-12.39) were independent predictors of unfavorable seizure outcomes. SIGNIFICANCE: Surgical resection is an effective treatment for patients with drug-resistant epilepsy secondary to encephalomalacia. Knowledge of the predictors of seizure outcomes may help during preoperative counseling and selection of optimal candidates for epilepsy surgery among patients with drug-resistant epilepsy secondary to encephalomalacia.

Abnormal parietal encephalomalacia associated with schizophrenia: A case report.

RATIONALE: It is widely believed that structural abnormalities of the brain contribute to the pathophysiology of schizophrenia. The parietal lobe is a central hub of multisensory integration, and abnormities in this region might account for the clinical features of schizophrenia. However, few cases of parietal encephalomalacia associated with schizophrenia have been described. PATIENT CONCERNS AND DIAGNOSES: In this paper, we present a case of a 25-year-old schizophrenia patient with abnormal parietal encephalomalacia. The patient had poor nutrition and frequently had upper respiratory infections during childhood and adolescence. She showed severe schizophrenic symptoms such as visual hallucinations for 2 years. After examining all her possible medical conditions, we found that the patient had a lesion consistent with the diagnosis of encephalomalacia in her right parietal lobe and slight brain atrophy. INTERVENTIONS: The patient was prescribed olanzapine (10 mg per day). OUTCOMES: Her symptoms significantly improved after antipsychotic treatment and were still well controlled 1 year later. LESSONS: This case suggested that parietal encephalomalacia, which might be caused by inflammatory and infectious conditions in early life and be aggravated by undernutrition, might be implicated in the etiology of schizophrenia.

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

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

Magnetoencephalography helps delineate the extent of the epileptogenic zone for surgical planning in children with intractable epilepsy due to porencephalic cyst/encephalomalacia.

OBJECT: Porencephalic cyst/encephalomalacia (PC/E) is a brain lesion caused by ischemic insult or hemorrhage. The authors evaluated magnetoencephalography (MEG) spike sources (MEGSS) to localize the epileptogenic zone in children with intractable epilepsy secondary to PC/E. METHODS: The authors retrospectively studied 13 children with intractable epilepsy secondary to PC/E (5 girls and 8 boys, age range 1.8-15 years), who underwent prolonged scalp video-electroencephalography (EEG), MRI, and MEG. Interictal MEGSS locations were compared with the ictal and interictal zones as determined from scalp video-EEG. RESULTS: Magnetic resonance imaging showed PC/E in extratemporal lobes in 3 patients, within the temporal lobe in 2 patients, and in both temporal and extratemporal lobes in 8 patients. Magnetoencephalographic spike sources were asymmetrically clustered at the margin of PC/E in all 13 patients. One cluster of MEGSS was observed in 11 patients, 2 clusters in 1 patient, and 3 clusters in 1 patient. Ictal EEG discharges were lateralized and concordant with MEGSS in 8 patients (62%). Interictal EEG discharges were lateralized and concordant with MEGSS hemisphere in 9 patients (69%). Seven patients underwent lesionectomy in addition to MEGSS clusterectomy with (2 patients) and without (5 patients) intracranial video-EEG. Temporal lobectomy was performed in 1 patient and hemispherectomy in another. Eight of 9 patients achieved seizure freedom following surgery. CONCLUSIONS: Magnetoencephalography delineated the extent of the epileptogenic zone adjacent to PC/E in patients with intractable epilepsy. Complete resection of the MEGSS cluster along with PC/E can provide favorable seizure outcomes.

The ketogenic diet for medically and surgically refractory status epilepticus in the neurocritical care unit.

BACKGROUND: Refractory status epilepticus carries a high risk of morbidity and mortality despite, and at times as a result of, aggressive pharmacologic interventions. Dietary therapies have been used for almost a century in children for controlling medically refractory seizures and status epilepticus and recent studies suggest efficacy and safety in adults as well. METHODS: Case report and literature review. RESULTS: We describe a case of medically and surgically refractory status epilepticus that was controlled after initiation of the ketogenic diet and maintenance with the modified Atkins diet in an adult in the neurocritical care unit. CONCLUSIONS: Dietary therapy should be considered as a treatment option in adult patients with refractory status epilepticus.

Cerebromalacia with epilepsy and cortical blindness in a laboratory Japanese macaque (Macaca fuscata).

The authors performed a pathological examination of a 5-year-old female laboratory Japanese monkey who developed cortical blindness and epileptic seizures. Generalized, tonic-clonic seizures started to occur during behavioral training to get the animal to enter a carrying cage for future psychological experiments. Blindness was suspected because of a lack of approaching behavior toward foods such as fruits. Although the monkey was extensively treated with anticonvulsants, the clinical signs did not improve. An increased serum creatine phosphokinase (CPK) level and bilateral occipital brain atrophy were detected. Histopathologically, a severe degree of cerebromalacia was detected bilaterally in the occipital lobe, and necrosis and gliosis were seen mainly in the temporal lobe. Focal inflammation was found in the meninges. No other changes were observed in other nervous tissues or organs, and no signs of a parasitic or viral infection were found in the systemic organs. Spontaneously occurring lesions in the central nervous system have been rarely reported in laboratory monkeys. In the present case, the cause of cerebromalacia could not be confirmed, but the relationship between symptoms such as abnormal vision and the presence of brain lesions was distinct. The authors believe that this case is a valuable historical control case for the laboratory Japanese macaque.

Polioencefalomalacia em bovinos: epidemiologia, sinais clínicos e distribuição das lesões no encéfalo / Bovine polioencephalomalacia: epidemiology, clinical signs and distribution of lesions in the brain

Trinta e um casos de polioencefalomalacia (PEM) diagnosticados de 1999-2008 em bovinos do Sul (13 casos) e Centro-Oeste (18 casos) brasileiros foram estudados. As taxas de morbidade (0,04 por cento-6,66 por cento), mortalidade (0,04 por cento-6,66 por cento) e letalidade (50 por cento-100 por cento) foram semelhantes em ambas as regiões estudadas. Não houve uma associação clara entre os casos de PEM e a idade, sexo dos bovinos e sazonalidade. Os casos ocorreram principalmente em bovinos criados de forma extensiva em pastagem. Na Região Sul a doença afetou principalmente bovinos jovens (um ano de idade ou menos), enquanto que principalmente bovinos mais velhos (3 anos de idade ou mais) foram afetados no Centro-Oeste. Os sinais clínicos mais frequentemente observados incluíram cegueira, incoordenação, andar em círculos, opistótono, decúbito e movimentos de pedalagem. A evolução do quadro clínico variou de 12 horas a 8 dias (media 3 dias e meio). Em 11 encéfalos não foram observadas alterações macroscópicas; as principais alterações macroscópicas nos outros casos incluíam congestão com tumefação e achatamento das circunvoluções, amolecimento e amarelamento do córtex telencefálico, focos de hemorragia no tronco encefálico, cerebelo e telencéfalo e herniação cerebelar. As principais alterações histológicas ocorreram no córtex dos lobos telencefálicos occipital, parietal e frontal; no entanto, lesões menos acentuadas e menos frequentemente observadas ocorreram no hipocampo, núcleos da base, tálamo, mesencéfalo e cerebelo. O tipo de lesão microscópica cortical era consistente em todos os casos e incluía necrose neuronal (neurônio vermelho) laminar segmentar, espongiose, tumefação do núcleo das células endoteliais, astrócitos Alzheimer tipo II e infiltração por células gitter. Em 20 por cento dos casos havia um leve infiltrado celular linfo-histiocitário e em 13 por cento dos casos havia leve infiltrado de neutrófilos e eosinófilos. Adicionalmente, ...

Thirty one cases of polioencephalomalacia (PEM) diagnosed from 1999-2008 in cattle from the Southern (13 cases) and Midwestern (18 cases) Brazil were studied. Morbidity (0.04 percent-6.66 percent), mortality (0.04 percent-6.66 percent), and lethality (50 percent-100 percent) rates were similar in both regions studied. There was no clear association between PEM cases and age, sex or seasonality. Cases occurred mainly in cattle raised at pasture; in the Southern the disease affected mainly young cattle (one-year old or less) while mainly older cattle (three-year-old or older) were affected in the Midwest. Clinical signs more frequently observed included blindness, incoordination, circling, opisthotonus, recumbence and peddling movements. Clinical course varied from 12 hours to 8 days (average three days and a half). In 11 cases no gross changes were observed in the brain. Main gross findings in the brain of remaining cases included congestion with swelling and flattening of gyri, softening and yellow discoloration of cerebral cortex, hemorrhagic foci in the brain stem, cerebellum and telencephalon, and cerebellar herniation. The main histopathological changes were in the cortex of occipital, parietal and frontal telencephalic lobes; however less prominent and less frequently found lesions occurred in the hippocampus, basal nuclei, thalamus, midbrain, and cerebellum. The type of microscopic cortical lesions was consistent in all cases and included segmentar laminar neuronal necrosis (red neurons), spongiosis, swollen of vascular endothelial nuclei, Alzheimer type II astrocytes and infiltration of gitter cells. In 20 percent of the cases there was mild lymphohistiocytic cellular infiltrate and in 13 percent of the cases there was mild infiltrate by neutrophils and eosinophils. Additionally, mild to moderate necro-hemorrhagic lesions were observed in 49 percent of the cases in the basal nuclei, in 39 percent of the cases in brain stem and...

Route learning impairment associated with encephalomalasia secondary to traumatic brain injury: a case report.

Topographical disorientation is marked by difficulty finding one's way in familiar or new environments. The present case study reports findings from a 30-year-old male with encephalomalasia of the left parahippocampal region secondary to brain trauma with subsequent difficulty in learning of new routes. His navigation in premorbidly known (familiar) surroundings was intact. Magnetic resonance images revealed left parahippocampal and bilateral occipital encephalomalasia. Neuropsychological screening showed impairment in structuring a representation of the spatial relationships among landmarks with relatively preserved ability to learn visual and verbal information of these landmarks. Decreased visual perception and inappropriate visual inputs due to cervical dystonia and right homonymous hemianopsia also appear to play a role in his disability. The current knowledge about the neuronal systems involved in visual cognition and topographical orientation also are addressed in this report.

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

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

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

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

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

Hyperprolactinemia presenting with encephalomalacia-associated seizure disorder and infertility: a novel application for bromocriptine therapy in reproductive endocrinology.

OBJECTIVES: To describe successful oral bromocriptine therapy for hyperprolactinemia accompanied by seizure disorder and encephalomalacia identified during infertility evaluation. MATERIALS & METHODS: A 32 year-old male with an unspecified seizure disorder was referred for infertility consultation. The initial seizure was at age 12, and was treated sequentially with phenytoin, phenobarbitol, and carbamazepine monotherapy which was maintained for six years and then discontinued. At age 26, a second seizure was experienced and 150mg lamotrigine b.i.d. was initiated. When the patient married it was discovered that erection was impossible; tadalafil was administered without success. At our center, total testosterone was low at 107ng/dl and semen analysis revealed oligoasthenozoospermia. Serum prolactin was 79.3ng/ml; visual field examination was normal. Brain MR showed no pituitary enlargement. However, encephalomalacia of the right occiptal lobe with porencephalic dilation of the occipital horn of the right lateral ventricle was present. RESULTS: The patient was initially placed on 2.5mg/d bromocriptine but after three months only a nominal reduction of serum prolactin was achieved. Serum prolactin normalized (25.4ng/ml) after bromocriptine was increased to 7.5mg/d. Repeat semen analysis showed overall sperm concentration of 85M/ml, 30% motility and 12% normal forms morphology (Kruger strict criteria). Total testosterone level was 191ng/dl after final bromocriptine dose was attained, which was well tolerated. DISCUSSION: Bromocriptine has an established role in the management of hyperprolactinemia, particularly in women. For males with elevated serum prolactin even when associated with focal encephalomalacia and seizure disorder, bromocriptine therapy can offer safe benefits including improved semen parameters and normal serum testosterone levels.

Altered mentation caused by polioencephalomalacia, hypernatremia, and lead poisoning.

Polioencephalomalacia in general and the specific disorders associated with lead toxicosis and sodium imbalances all have commonalities in risk groups and clinical presentations but can be differentiated based on herd history, subtle clinical differences, and diagnostic tests. Treatment also has some overlap, but determining the specific etiology enables more specific treatment and more effective prophylactic measures.