Multicystic Encephalomalacia: The Neuropathology of Systemic Neonatal Parechovirus Infection.

The Neuropathology of Human Parechovirus (HPeV) is not widely described due to the relatively recent discovery of the virus combined with a limited number of autopsy case reports. We report the case of an infant boy born at 38 weeks who, six days after birth, presented with fever and severe neurological dysfunction. Human Parechovirus Type 3 (HPeV3) RNA was detected in his cerebrospinal fluid (CSF) and blood. He died five days after his initial presentation. Neuropathologic examination demonstrated multicystic encephalomalacia (ME). This case report confirms that white matter pathology is dominant in HPeV3 infection. A unique feature, of HPeV encephalomalacia is absence of CSF pleocytosis and minimal inflammation in the meninges. The findings permit comment on the pathogenesis of brain injury by this virus.

Huge chronic subdural haematoma formation in an encephalomalacia stroke cavity.

A patient suffering from a cerebrovascular ischaemic stroke may present similar symptoms to a patient with a chronic subdural haematoma (CSDH). Head CT imaging of an old extensive hemispheric infarction may appear hypodense in a similar fashion as CSDH. We described a 46-year-old man with a 2-week history of mild headache and worsening right lower extremity hemiparesis. Eight years prior, he suffered a left middle cerebral artery territory infarct. The head CT scan showed a huge, slightly hypodense area on the left brain, causing a significant mass effect. A new stroke was of concern versus a chronic subdural haematoma inside the old encephalomalacia stroke cavity. Only three previously reported cases of CSDH occupying an encephalomalacic cavity had been reported. This rare presentation should be considered in the differential diagnosis in patients with a history of cerebrovascular stroke. MRI is useful in making a correct diagnosis.

Pseudofeeders on fetal magnetic resonance imaging predict outcome in vein of Galen malformations.

OBJECTIVE: Although vein of Galen aneurysmal malformations (VGAM) can be diagnosed in the fetus, the challenge is predicting the occurrence of its 2 major complications: cardiopulmonary failure and encephalomalacia. This study attempts to determine which fetal brain magnetic resonance imaging (MRI) features might be used to predict the development of these complications at birth. METHODS: The cohort was extracted from a prospectively assembled database of VGAM cases managed at a single referral center from 2000 to 2014. Of 251 patients with VGAM, 83 cases were diagnosed prenatally. A total of 58 patient charts having at least 1 fetal MRI were reviewed. Patterns of brain parenchyma, hydrocephalus, and so-called middle cerebral artery (MCA) "pseudofeeders" were correlated with cardiac failure, pulmonary hypertension, and encephalomalacia at birth. RESULTS: The median gestational age at fetal MRI was 32.3 weeks of pregnancy (±2.3). Nine fetuses (16%) had encephalomalacia. Thirty-one fetuses (53%) had MCA pseudofeeders. Twenty-six fetuses (45%) had prenatal hydrocephalus. Prenatal MCA pseudofeeders were a risk factor for encephalomalacia at birth (p = 0.001). MCA pseudofeeders and hydrocephalus were risk factors for both severe cardiac failure (p = 0.01 and p = 0.04, respectively) and severe pulmonary hypertension (p = 0.014 and p = 0.05, respectively) at birth. INTERPRETATION: MCA pseudofeeders are the result of impaired cerebral blood flow, and are thus a risk factor for further brain melting at birth. Their presence can be used for informing parents and as an aid in management decisions. Ann Neurol 2017;81:278-286.

Cerebral Disorders of Calves.

Neurologic diseases of the cerebrum are relatively common in cattle. In calves, the primary cerebral disorders are polioencephalomalacia, meningitis, and sodium toxicity. Because diagnostic testing is not always readily available, the practitioner must often decide on a course of treatment based on knowledge of the likely disease, as well as his or her own clinical experience. This is particularly true with neurologic diseases in which the prognosis is often poor and euthanasia may be the most humane outcome. This article reviews the most common diseases affecting the cerebrum of calves with a focus on pathophysiology, diagnosis, and treatment.

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.

Bilateral cystic encephalomalacia following multiple intrauterine transfusions for anti-Kell isoimmunisation.

Fetal and neonatal haemolytic diseases result from maternal allo-immunisation to fetal antigens. Maternal antibodies cross the placenta causing red cell haemolysis, resulting in fetal anaemia and, in severe cases, hydrops and perinatal death. Intravascular intrauterine blood transfusion (IUT) has markedly reduced perinatal mortality and is now a standard procedure. IUT is considered to be a safe procedure with fetal loss rate reported to be less than 5% and no reported increase in the rate of neurodevelopment impairment. In this report, we are presenting a case of bilateral cystic encephalomalacia following fetal anaemia secondary to anti-Kell iso-immunisation treated with multiple IUTs. Such a significant adverse outcome following IUT for anti-Kell iso-immunisation has not been reported in the literature. This case highlights the need for appropriate parental counselling and routine postnatal head ultrasound in all babies delivered following multiple IUTs.

Development of a de novo arteriovenous malformation after severe traumatic brain injury.

Arteriovenous malformations (AVMs) are typically considered congenital lesions, although there is growing evidence for de novo formation of these lesions as well. The authors present the case of an AVM in the same cerebral cortex that had been affected by a severe traumatic brain injury (TBI) more than 6 years earlier. To the best of the authors' knowledge, this is the first report attributing the formation of an AVM directly to TBI.

Bovine diseases causing neurological signs and death in Mexican feedlots.

The number of large feedlot operations, similar to that of USA and Canada, has notably increased in Mexico in the last three decades. Clinical and laboratory diagnoses of neurological diseases in feedlot cattle are crucial in Mexico and Central America because of the high incidence of bovine paralytic rabies (BPR). Because of its zoonotic potential, BPR must be promptly diagnosed and differentiated from other bovine neurological diseases such as thrombotic meningoencephalitis (TME), polioencephalomalacia (PEM) and botulism. More recently, BPR and botulism have been diagnosed with increasing frequency in Mexican feedlots. Neither BPR nor botulism has relevant gross lesions, thus post-mortem diagnosis without laboratory support is impossible. Herein, we describe five outbreaks of neurological diseases in Mexican feedlots in which BPR, botulism and PEM were diagnosed either independently or in combination. A diagram illustrating the most conspicuous pathologic findings and ancillary laboratory test required to confirm the diagnoses of these neurological diseases in feedlot cattle is proposed.

Severe brain damage in premature infants associated with postnatal infection.

AIM: This study examined clinical features and neuroimaging characteristics of severe brain damage in premature infants with postnatal infection. METHODS: We retrospectively analyzed clinical data of two preterm infants who developed extensive encephalomalacia secondary to postnatal infection. RESULTS: Two premature boys experienced serious postnatal infection at about 3 weeks after birth; the infection was characterized by lethargy, apnea, increased CRP, severe bilateral pneumonia, positive sputum culture for multidrug-resistant bacteria, and mild changes of cerebrospinal fluid. Both infants required ventilation and antibiotic therapy. While both infants survived, a very extensive encephalomalacia was documented by serial cranial ultrasound, MRI and CT scans 3 to 4 weeks after postnatal infection. Their mothers had no premature ruptures of membranes and no signs of antenatal infection, suggesting potential postnatal infection in the infants. CONCLUSION: More studies are needed to better understand the underlying mechanism of encephalomalacia associated with postnatal infection. To facilitate early diagnosis and effective treatment, cranial ultrasound scans should be done routinely in premature infants with serious postnatal infection.

Assessment of ruminal hydrogen sulfide or urine thiosulfate as diagnostic tools for sulfur induced polioencephalomalacia in cattle.

To determine if ruminal hydrogen sulfide, urine thiosulfate, or blood sulfhemoglobin could be used as diagnostic indicators for sulfur-induced polioencephalomalacia, 16 steers (8 cannulated, 368 ± 12 kg; 8 unmodified, 388 ± 10 kg; mean ± standard error) were fed 1 of 2 dietary treatments. Diets consisted of a low sulfate (0.24% S; control) wheat midd-based pellet or the control pellet with sodium sulfate added to achieve a high-sulfate (0.68% S) pellet. As designed, intake did not differ (P = 0.80) between treatments. At 8 hr postfeeding, ruminal hydrogen sulfide was not affected by cannulation (P = 0.35) but was greater (P < 0.01) in high S (6,005 ± 475 mg/l) than control (1,639 ± 472 mg/l) steers. Time of day of sampling affected (P = 0.01) ruminal hydrogen sulfide, with peak concentrations occurring 4-12 hr after feeding. Urine was collected prefeeding (AM) and 7-9 hr postfeeding (PM). Urine thiosulfate concentrations of high S steers sampled in the PM were greater (P > 0.01) than in the AM. However, there was no difference due to time of sampling for control. In both the AM and PM, urine thiosulfate concentrations of high S were greater (P > 0.01) than control. Although hydrogen sulfide and thiosulfate were elevated by increased dietary S intake, a concentration at which polioencephalomalacia is likely to occur could not be determined. Sampling urine for thiosulfate or rumen gas for hydrogen sulfide of nonsymptomatic pen mates 4-8 hr after feeding may be useful to assess sulfur exposure and differentiate between causes of polioencephalomalacia.

Nigropallidal encephalomalacia in horses grazing Rhaponticum repens (creeping knapweed).

Nigropallidal encephalomalacia was diagnosed in two horses in northern Victoria that had a history of long-term pasture access to a dense growth of Rhaponticum repens. The region in which the affected horses lived had received well above average rainfall for several months preceding the poisoning. Affected horses had sudden onset of subcutaneous oedema of the head, impaired prehension and mastication, dullness, lethargy and repeated chewing-like jaw movements. Diagnosis was confirmed at necropsy, with characteristic malacic lesions in the substantia nigra and globus pallidus of the brain. This is the first documented case of nigropallidal encephalomalacia in Australian horses associated with R. repens.

Severe enteroviral encephalitis complicated by encephalomalacia--case presentation.

Enteroviral encephalitis is a rare neuroinfection more often diagnosed in children within the context of enteroviral epidemic outbreaks. It has pleomorphic clinical features, variable severity and a definite potential to cause neuropsychological sequelae especially in infants. Some subtypes are extremely severe with a mortality rate of up to 25% by affecting the brain stem. These subtypes usually come with highly specific findings on neuroimaging. We present a paediatric case of diffuse encephalitis most likely enteroviral in nature, with some particular features concerning the severe clinical form, the neuroimaging aspects and the neuropsychological sequelae due to the rarely described evolution towards encephalomalacia.

Parkinsonism with multiple cysts in the bilateral striata.

The present paper reports on a 68-year-old man with a 10-year history of parkinsonism who developed hallucinations and delusions after admission to an intensive care unit for the treatment of organophosphate intoxication. His initial diagnosis was delirium. On the basis of brain computed tomography findings and clinical symptoms, we diagnosed drug-induced psychosis in parkinsonism with multiple cysts in the bilateral striata.

Clinical application of readout-segmented- echo-planar imaging for diffusion-weighted imaging in pediatric brain.

BACKGROUND AND PURPOSE: RS-EPI has been suggested as an alternative approach to EPI for high-resolution DWI with reduced distortions. To determine whether RS-EPI is a useful approach for routine clinical use, we implemented GRAPPA-accelerated RS-EPI DWI at our pediatric hospital and graded the images alongside standard accelerated (ASSET) EPI DWI used routinely for clinical studies. MATERIALS AND METHODS: GRAPPA-accelerated RS-EPI DWIs and ASSET EPI DWIs were acquired on 35 pediatric patients using a 3T system in 35 pediatric patients. The images were graded alongside each other by using a 7-point Likert scale as follows: 1, nondiagnostic; 2, poor; 3, acceptable; 4, standard; 5, above average; 6, good; and 7, outstanding. RESULTS: The following were the average scores for EPI and RS-EPI, respectively: resolution, 3.5/5.2; distortion level, 2.9/6.0; SNR, 3.4/4.1; lesion conspicuity, 3.3/5.9; and diagnostic confidence, 3.2/6.0. Overall, the RS-EPI had significantly improved diagnostic confidence and more reliably defined the extent and structure of several lesions. Although ASSET EPI scans had better SNR per scanning time, the higher spatial resolution as well as reduced blurring and distortions on RS-EPI scans helped to better reveal important anatomic details at the cortical-subcortical levels, brain stem, temporal and inferior frontal lobes, skull base, sinonasal cavity, cranial nerves, and orbits. CONCLUSIONS: This work shows the importance of both resolution and decreased distortions in the clinics, which can be accomplished by a combination of parallel imaging and alternative k-space trajectories such as RS-EPI.