Signs Of Brainstem Dysfunction Research Articles

СРАВНИТЕЛЬНЫЙ АНАЛИЗ НЕЙРОФИЗИОЛОГИЧЕСКИХ И НЕЙРОВИЗУАЛИЗАЦИОННЫХ ПОКАЗАТЕЛЕЙ У ДЕТЕЙ С ГЕМИПЛЕГИЧЕСКОЙ ФОРМОЙ ДЕТСКОГО ЦЕРЕБРАЛЬНОГО ПАРАЛИЧА В ЗАВИСИМОСТИ ОТ ЛАТЕРАЛИЗАЦИИ ОЧАГА И ПОЛА

Objective: This study aims to analyze the neurophysiological and neuroimaging characteristics of children diagnosed with hemiplegic cerebral palsy (HCP) while considering the impact of gender and laterality of hemiparesis. Methods: We analyzed 125 children between the ages of 7-9 (with a mean age of 8.2±0.9 years) diagnosed with HCP (ICD-10 Code: G80.2). Our analysis focused on the lateralization of cerebral injury. The patients were divided into 2 groups based on the hemispheric focus lateralization. In each group, subgroups were distinguished by gender. After examining the patients, it was found that the most prevalent were male children, children aged eight years, and those with left-sided hemiparesis. Results: The EEG of patients with HCP showed diffuse changes and signs of brainstem dysfunction (disorganization of the α-rhythm at a reduced amplitude level). Furthermore, there were fluctuations in the brain's bioelectrical activity (BEA) of varying degrees of intensity. Residual organic disorders (slowing down of the cortical rhythm) were found in 64 (88.9%) children of group I and in 42 (79.2%) children of group II (p>0.05; χ2 =1.52). MRI data analysis revealed brain pathological changes in all examined patients. In group I, periventricular lesions of the white matter were present in 36.4% (12) and 38.5% (15) of female and male pediatric patients, respectively. In both female and male pediatric patients, atrophic and subatrophic changes were detected in the cerebral cortex with a prevalence of 51.5% (17) and 53.8% (21), respectively. Brain scans revealed that 84.8% (28) of female and 89.7% (35) of male pediatric patients had focal brain lesions. In group II, periventricular lesions of the white matter occurred in 34.8% (8) and 36.7% (11) of female and male pediatric patients, respectively. Gray matter atrophy in both female and male pediatric patients was detected in 47.8% (11) and 53.3% (16) cases, respectively. Focal brain lesions were in 65.2% (1 5) and 76.7% (23) of female and male pediatric patients, respectively. Conclusion: There is no correlation between the neurophysiological and neuroimaging characteristics of children with HCP and their hemiparesis laterality or gender. From the available data, it can be inferred that HCP is likely caused by focal cerebral damage and brain malformations.Keywords: Cerebral palsy, electroencephalogram, brain bioelectrical activity, magnetic resonance imaging, slow sensorimotor rhythm.

Herniation World Federation of Neurosurgical Societies Scale Improves Prediction of Outcome in Patients With Poor-Grade Aneurysmal Subarachnoid Hemorrhage.

Favorable outcomes are seen in up to 50% of patients with World Federation of Neurosurgical Societies (WFNS) grade V aneurysmal subarachnoid hemorrhage. Therefore, the usefulness of the current WFNS grading system for identifying the worst scenarios for clinical studies and for making treatment decisions is limited. We previously modified the WFNS scale by requiring positive signs of brain stem dysfunction to assign grade V. This study aimed to validate the new herniation WFNS grading system in an independent prospective cohort. We conducted an international prospective multicentre study in poor-grade aneurysmal subarachnoid hemorrhage patients comparing the WFNS classification with a modified version-the herniation WFNS scale (hWFNS). Here, only patients who showed positive signs of brain stem dysfunction (posturing, anisocoric, or bilateral dilated pupils) were assigned hWFNS grade V. Outcome was assessed by modified Rankin Scale score 6 months after hemorrhage. The primary end point was the difference in specificity of the WFNS and hWFNS grading with respect to poor outcomes (modified Rankin Scale score 4-6). Of the 250 patients included, 237 reached the primary end point. Comparing the WFNS and hWFNS scale after neurological resuscitation, the specificity to predict poor outcome increased from 0.19 (WFNS) to 0.93 (hWFNS) (McNemar, P<0.001) whereas the sensitivity decreased from 0.88 to 0.37 (P<0.001), and the positive predictive value from 61.9 to 88.3 (weighted generalized score statistic, P<0.001). For mortality, the specificity increased from 0.19 to 0.93 (McNemar, P<0.001), and the positive predictive value from 52.5 to 86.7 (weighted generalized score statistic, P<0.001). The identification of objective positive signs of brain stem dysfunction significantly improves the specificity and positive predictive value with respect to poor outcome in grade V patients. Therefore, a simple modification-presence of brain stem signs is required for grade V-should be added to the WFNS classification. URL: https://clinicaltrials.gov; Unique identifier: NCT02304328.

Outcome of abusive head trauma in children less than 2 years: A single center study from a middle-income country

BackgroundAbusive head trauma (AHT) is a severe manifestation of physical abuse in young children. This study examines the outcome of AHT in children under 2 years and features associated with mortality and disability. MethodsRetrospective chart review of 72 children under 2 years diagnosed to have AHT between 2011 and 2018 at a tertiary teaching hospital in Malaysia. Demographic variables, clinical features, results of neuroimaging and their associations with outcome were explored. ResultsThe median age at presentation was 4 months, 78% were six months or younger and 68% were male. Two-thirds became unwell in the care of an alternative caregiver. Subdural hemorrhage was present in 98%. Mortality was 10%. Forty-nine survivors returned with median interval of 16 months (IQR 5–44 months) between discharge and last follow-up. At least 35% of 65 survivors were disabled with 29% having multiple disabilities. Multiple disabilities (61.1% versus 25.8%) and cognitive impairment (61.1% versus 22.6%) were significantly higher in children 3 years or older at last follow-up (p < 0.05). Signs of brainstem dysfunction at presentation, requirement for ventilation and hypoxic-ischemic injury on neuroimaging were associated with mortality. Needing ventilation, cerebral edema and neurological or visual impairment at discharge were associated with disability. ConclusionsOutcomes of death or disability in 42% make prevention of AHT a public health priority. Survivors require long-term multi-disciplinary follow-up for coordination of rehabilitation and educational support. Prevention should be directed at improving resources and services to support families in the care of young infants under 6 months.

Acute Disorders of Consciousness and Coma - Systematics, Differential Diagnosis and Management

Cerebral diseases such as epileptic seizures, cerebral hemorrhages or meningoencephalitis are the primary cause of approximately 50 % of non-traumatic acute disorders of consciousness. For the differential diagnosis, history and other symptoms are important such as hemiplegia, signs of brain stem dysfunction, meningism or headache. Metabolic, endocrinologic, toxicologic or electrolytic causes of coma usually can be diagnosed by laboratory examinations. Anamnestic informations, body inspection, clinical neurological examination as well as laboratory and imaging findings have to be added and categorized by a multilevel composition to establish a conclusive diagnosis. Simultaneously therapeutic measures for suspected primary cerebral diseases must be initiated, for example a rapid antibiotic treatment in case of a possible bacterial meningitis. A fast and structured diagnostic approach is crucial for ensuring a good prognosis and helps to miss relevant diagnostic steps. Potential diagnostic and therapeutic pitfalls must be kept in mind.

Place of Gamma Knife Stereotactic Radiosurgery in Grade 4 Vestibular Schwannoma Based on Case Series of 86 Patients with Long-Term Follow-Up

Grade IV vestibular schwannoma (Koos classification) is generally considered to be an indication for microsurgical resection or combined radiosurgery-microsurgery. However, the place of Gamma Knife stereotactic surgery (GK-SRS), either as first-line treatment or when progression of residual tumor compresses the brainstem, has not been clearly evaluated. This article reports the results of a large case series of patients with grade 4 vestibular schwannoma treated by GK-SRS. All consecutive patients with grade IV vestibular schwannoma treated by GK-SRS in our department between 1996 and 2011 with a minimum follow-up of 3 years were included in this study. 86 patients were treated by GK-SRS with a minimum follow-up of 3 years. Mean follow-up was 6.2 years (3-16 years). The mean age of the patients at the time of GK-SRS was 54.6 years (range: 23-84) and the sex ratio was 0.6. At the time of radiosurgery, no patient presented brainstem dysfunction prior to GK-SRS. 38 patients had functional hearing before treatment. One patient presented mild trigeminal neuralgia before GK-SRS. Tumor control with no clinical deterioration was obtained in 78 patients (90.7%). No radiation-induced brainstem or cranial nerve toxicity was observed in any of these patients. Functional hearing was maintained in 25 patients. 8 (9.3%) patients presented tumor growth and required microsurgical resection in 7 cases and ventricular shunt in 1 case. On the basis of this large series, GK-SRS appears to be a safe and effective treatment option for grade IV vestibular schwannoma for patients with no signs of brainstem dysfunction.

Multiple sclerosis broke my heart.

We report 5 cases of acute heart failure (AHF) related to multiple sclerosis (MS) relapses. AHF was inaugural in 3 patients, always preceded or accompanied by signs of brainstem dysfunction; it was severe, requiring intensive care management. Echocardiography showed left ventricular hypokinesis. No other cause of AHF has been found. All patients showed a new medullary lesion on brain magnetic resonance imaging. All had rapid and complete recovery of ventricular function after intravenous corticosteroids. We concluded that the cases represent a takotsubo phenomenon. Physicians should be aware of rare cases of takotsubo cardiomyopathy in MS relapses. Ann Neurol 2017;81:754-758.

Fatal Cerebral Edema With Status Epilepticus in Children With Dravet Syndrome: Report of 5 Cases.

Dravet syndrome (DS) is a well-recognized developmental and epileptic encephalopathy associated with SCN1A mutations and 15% mortality by 20 years. Although over half of cases succumb to sudden unexpected death in epilepsy, the cause of death in the remainder is poorly defined. We describe the clinical, radiologic, and pathologic characteristics of a cohort of children with DS and SCN1A mutations who developed fatal cerebral edema causing mass effect after fever-associated status epilepticus. Cases were identified from a review of children with DS enrolled in the Epilepsy Genetics Research Program at The University of Melbourne, Austin Health, who died after fever-associated status epilepticus. Five children were identified, all of whom presented with fever-associated convulsive status epilepticus, developed severe brain swelling, and died. All had de novo SCN1A mutations. Fever of 40°C or greater was measured in all cases. Signs of brainstem dysfunction, indicating cerebral herniation, were first noted 3 to 5 days after initial presentation in 4 patients, though were apparent as early as 24 hours in 1 case. When MRI was performed early in a patient's course, focal regions of cortical diffusion restriction were noted. Later MRI studies demonstrated diffuse cytotoxic edema, with severe cerebral herniation. Postmortem studies revealed diffuse brain edema and widespread neuronal damage. Laminar necrosis was seen in 1 case. Cerebral edema leading to fatal brain herniation is an important, previously unreported sequela of status epilepticus in children with DS. This potentially remediable complication may be a significant contributor to the early mortality of DS.

Brainstem auditory evoked potentials in neurophysiological assessment of brain stem dysfunction in patients with atherostenosis of vertebral arteries

To assess brain stem dysfunction in patients with hemodynamically significant stenosis of vertebral arteries (VA) using short latency brainstem auditory evoked potentials (BAEP). The study group included 50 patients (mean age 64±6 years) with hemodynamically significant extracranial VA stenosis. Patients with hemodynamically significant extracranial VA stenosis had BAEP abnormalities including the elongation of interpeak intervals I-V and peak V latency as well as the reduction of peak I amplitude. After transluminal balloon angioplasty with stenting of VA stenoses, there was a shortening of peak V latency compared to the preoperative period that reflected the improvement of brain stem conductive functions. Atherostenosis of vertebral arteries is characterized by the signs of brain stem dysfunction, predominantly in the pontomesencephal brain stem. After transluminal balloon angioplasty with stenting of VA, the improvement of brain stem conductive functions was observed.

Neuromyelitis Optica Spectrum Disorder: A Case Report

A 66 year old woman suffering from recurrent attacks of neuropathic burning pain over the past 6 years, affecting first the sacral region and then progressively different locations on the face, was referred to our facility. She had severe paraparesis and signs of brainstem dysfunction leading to respiratory failures. After an extensive investigation including brain and spinal cord MRI and laboratory findings, such as positive test for aquaporin-4 immunoglobulin G antibodies (AQP4-IgG), the patient was found to suffer from neuromyelitis optica spectrum disorder (NMOSD) without optic neuritis. She was initially treated with corticosteroids followed by plasmapheresis. She partially recovered regarding her respiratory function but remained paraplegic. She was placed on azathioprine and died a few months afterwards.

Sea Snake Bites Resulted in a Mimic of Brain Death

A 69 year old man with deep coma presented after an alleged history of sea snake bite. Patient presented with irresponsiveness to painful stimuli, complete ptosis, internal and external ophthalmoplegia. He was given continuous ventilator support despite signs of brain stem dysfunction. On admission day 20, the patient was weaned off ventilator and discharged from the hospital. Whenever treating patients who were suspected with a history of snake bite, an emergency physician should always consider severe envenomation in the differential diagnosis of acute neuroparalytic syndrome, which may avert the unfortunate misdiagnoses of brain death.

Heterozygous de-novo mutations in ATP1A3 in patients with alternating hemiplegia of childhood: a whole-exome sequencing gene-identification study

Alternating hemiplegia of childhood (AHC) is a rare neurological disorder characterised by early-onset episodes of hemiplegia, dystonia, various paroxysmal symptoms, and developmental impairment. Almost all cases of AHC are sporadic but AHC concordance in monozygotic twins and dominant transmission in a family with a milder phenotype have been reported. Thus, we aimed to identify de-novo mutations associated with this disease. We recruited patients with clinically characterised AHC from paediatric neurology departments in Germany and with the aid of a parental support group between Sept, 2004, and May 18, 2012. We used whole-exome sequencing of three proband-parent trios to identify a disease-associated gene and then tested whether mutations in the gene were also present in the remaining patients and their healthy parents. We analysed genotypes and characterised their associations with the phenotypic spectrum of the disease. We studied 15 female and nine male patients with AHC who were aged 8-35 years. ATP1A3 emerged as the disease-associated gene in AHC. Whole-exome sequencing showed three heterozygous de-novo missense mutations. Sequencing of the 21 remaining affected individuals identified disease-associated mutations in ATP1A3 in all patients, including six de-novo missense mutations and one de-novo splice-site mutation. Because ATP1A3 is also the gene associated with rapid-onset dystonia-parkinsonism (DYT12, OMIM 128235) we compared the genotypes and phenotypes of patients with AHC in our cohort with those of patients with rapid-onset dystonia-parkinsonism reported in the scientific literature. We noted overlapping clinical features, such as abrupt onset of dystonic episodes often triggered by emotional stress, a rostrocaudal (face to arm to leg) gradient of involvement, and signs of brainstem dysfunction, as well as clearly differentiating clinical characteristics, such as episodic hemiplegia and quadriplegia. Mutation analysis of the ATP1A3 gene in patients who met clinical criteria for AHC allows for definite genetic diagnosis and sound genetic counselling. AHC and rapid-onset dystonia-parkinsonism are allelic diseases related to mutations in ATP1A3 and form a phenotypical continuum of a dystonic movement disorder. Eva Luise and Horst Köhler Foundation for Humans with Rare Diseases.

Hemifacial spasm associated with intraparenchymal brain stem tumor

parenchymal brain stem lesions remains unclear. A 58-year-old woman, who had been experiencing slowly progressive left facial weakness, diplopia, and dysphagia for 7 years, developed intermittent facial twitches around her left orbicularis oculi, lower face, and platysma. On examination, she had severe left facial weakness, left abducens palsy, moderate dysarthria, tongue atrophy, and mild right hemiparesis. Intermittent contractions were visible in left frontalis, orbicularis oculi, and zygomaticus muscles. These occurred as single twitches and as brief series of contractions (Video). Magnetic resonance imaging revealed a nonenhancing lesion (maximum diameter, 22 mm) in the left lower pons and the central and left upper medulla, with increased signal on T2-weighted and proton density images, consistent with a lowgrade pontomedullary glioma or hamartoma. Notably, the lesion involved the facial nucleus and spared the root exit zones of both facial nerves (Fig. 1). Nerve conduction studies showed significantly reduced amplitudes in the left facial nerve, especially in the orbicularis oris and mentalis branches; however, responses were evident in all facial muscles tested. In blink reflex studies, left supraorbital nerve (SON) stimulation produced normal-latency ipsilateral R1 and significantly prolonged ipsilateral and normal contralateral R2 latencies (55.3 and 39.9 ms, respectively). Right SON stimulation produced normal ipsilateral R1 and R2 latencies, but absent contralateral R2 response. These findings suggest interruption of normal inputs to the left facial nucleus. They are also evidence of cross-communication that could originate from the intramedullary portion of the facial nerve or from the facial nucleus. Electromyography revealed evidence of chronic but not acute neurogenic changes in affected facial muscles. Discharges consistent with HFS were recorded from the left orbicularis oris during an episode of repeated spasm-like contractions, typical of HFS, of the left cheek and perioral muscles. The left orbicularis oris had 1þ fasciculations, as well as intermittent single discharges associated with clonic spasms, typical of HFS. No myokymic discharges were observed. Although involuntary facial muscle contractions have been reported in association with intraparenchymal lesions, 4,5 these conditions may have represented ‘‘spastic paretic hemifacial contracture’’ (SPHC), which is characterized by sustained facial muscle contractions and continuous muscle activity. 6,7 In 1 case 4 electromyography showed high-frequency bursts, but clonic spasms were not mentioned. In the other case 5 contractions were sustained, and electrophysiology showed tonic, nearly continuous activity of the facial nerve and high-frequency discharges in its innervated muscles. Both cases may thus have represented SPHC rather than HFS. To our knowledge, therefore, the present report is the first description of facial twitching with the clinical and electrophysiologic hallmarks of idiopathic HFS, associated with an intraparenchymal brain lesion affecting the facial nucleus but not the facial nerve’s root. Legend to Video. Hemifacial spasm and signs of brain stem dysfunction in the patient described in the present report. The video shows intermittent involuntary muscle contractions mainly involving the zygomaticus and to a lesser degree the frontalis and orbicularis oculi on the left side of the face. These occur at variable intervals, at times as single twitches and other times as brief trains of contractions.

Pseudocataplexy and Transient Functional Paralysis: A Spectrum of Psychogenic Motor Disorder

Pseudocataplexy and Transient Functional Paralysis: A Spectrum of Psychogenic Motor Disorder

Un cas d’ophtalmoplégie horizontale bilatérale : le syndrome 1+1

Oculomotor deficiencies in multiple sclerosis (MS) are frequently characterized by internuclear ophthalmpoplegia or isolated abduction or adduction palsies. Complete unilateral conjugate gaze paralysis and the "one and a half" syndrome are rare. Complete bilateral horizontal gaze paralysis has been exceptionally reported. Here, we describe an unusual oculomotor paralysis as a suspected first event of MS. A 24-year-old woman with an uneventful medical history presented for sudden onset of binocular diplopia. On examination, abduction and adduction saccades were impossible, whereas vertical eye saccades and convergence were normal. Oculocephalic reflex failed to improve horizontal eye movement. No nystagmus and no other sign of brainstem dysfunction were observed. Visual acuity was 4/10 in the right eye and 6/10 in the left eye. A sign of Marcus Gunn was noted in the right eye. Blood samples and cerebrospinal fluid were normal, no oligoclonal bands were detected. Visual evoked potentials were significantly impaired in both eyes and argued for bilateral optic neuritis. Brain MRI scans showed white matter T2-hypersignal abnormalities, which fulfill Barkhof criteria for MS. A small symmetric lesion was noted in the posterior part of the medial pontine tegmentum. As a first episode of MS was suspected, treatment with methylprednisolone 1000 mg/d for 3 days was started, and was followed by complete recovery of eye movements and visual acuity after 3 weeks. To our knowledge, only two cases of complete horizontal bilateral ophthalmoplegia have been reported in the literature. Both were associated with peripheral facial nerve palsy as a first event in MS. In our case report, we describe for the first time a complete bilateral horizontal ophthalmoplegia with no other brainstem dysfunction. By analogy with the "one and a half" syndrome, such complete horizontal gaze paralysis could be named a "one and one" syndrome and seems to be specifically related to a first event of MS.

Neurophysiological characteristics of supra-segmental structures in patients with arterial hypertension and chronic headaches

Neurophysiological characteristics of chronic headaches in patients with arterial hypertension were studied using brainstem auditory evoked potentials, visual evoked potentials and blink reflex. In patients with arterial hypertension suffering from chronic headaches, signs of brainstem dysfunction were revealed, which was accompanied by impairments of ascending afferent input and dysfunction of upper structures (thalamus and cortex), including impairment of functioning of the antinociceptive system of supraspinal level. These abnormalities result in the formation of a specific clinical pattern in the studied group of patients.

Physiological abnormalities in experimental allergic encephalomyelitis (EAE): II. Correlation between clinical signs and vestibular hyperreactivity and other signs of brain-stem dysfunction in rats with EAE.

12 Lewis rats were inoculated with a guinea pig spinal cord tissue preparation. They developed experimental allergic encephalomyelitis (EAE) after 12-14 days manifested by weight loss, tail flaccidity, ataxia, hind limb paresis or paralysis and urinary incontinence. Concomitantly with EAE, all animals developed vestibular hyperreactivity (VH) of canal and otolith reflexes. Other signs of brain-stem dysfunction were also observed: abducens paralysis, facial weakness, tachypnoe and mydriasis with defective pupillary light reflex. The vestibular and other abnormalities subsided with some delay after recovery from clinical EAE, whilst histological abnormalities were still present in the CNS.

Auditory double click evoked potentials in multiple sclerosis

Brain stem electric responses (BSER), evoked by acoustic stimuli, were investigated in 29 patients with multiple sclerosis and compared with the responses in a control group of 26 young adults without neurological disease or hearing defect. The procedure included monaural and binaural stimulus presentation of single clicks and pairs of clicks. In the evaluations the vertex-positive Jewett I, III and V response components, and the vertex-negative "FFP7" peak were considered. In the assessment of the BSER components, the FFP7 (the vertex-negative 7 ms far field potential following the JewettV wave) proved to be the best single component with respect to reproducibility in normal subjects. In addition the FFP7 was the single most consistently abnormal component in the patient group. Determination of the latency of the Jewett components and the interpeak conduction times, however, contributed further to the disclosure of the abnormal cases. Five of the patients whose hearing was impaired were considered separately. Of the remaining 24 patients, 20 (83%) had BSER abnormalities. Further analysis did not reveal a clear correlation between BSER abnormalities and clinical signs of brain stem dysfunction.

Dysphagia-Gastroesophageal Reflux Complex: Complications Due to Dysfunction of Solitary Tract Nucleus-Mediated Vago-Vagal Reflex

We report on the complication of gastroesophageal reflux (GER) in four patients with lower brainstem dysfunction. These patients suffered from perinatal asphyxia, cerebellar hemorrhage, or congenital dysphagia of unknown origin and showed facial nerve palsy, inspiratory stridor due to vocal cord paralysis, central sleep apnea, and dysphagia, in various combinations. Naso-intestinal tube feeding was introduced in all of the patients due to recurrent vomiting and aspiration pneumonia resulting from GER. T2-weighted magnetic resonance (MR) imaging revealed symmetrical high intensity lesions in the tegmentum of the lower pons and the medulla oblongata in two of the patients, and pontomedullary atrophy in another patient. In normal subjects, lower esophageal sphincter contraction is provoked by distension of the gastric wall, through a vago-vagal reflex. Since this reflex arc involves the solitary tract nucleus, where the swallowing center is located, the association of dysphagia and GER in the present patients is thought to result from the lesions in the tegmentum of medulla oblongata. We propose the term "dysphagia-GER complex" to describe the disturbed motility of the upper digestive tract due to lower brainstem involvement. In children with brainstem lesions, neurological assessment of GER is warranted, in addition to the examination of other signs of brainstem dysfunction, including dysphagia and respiratory disturbance.

Respiratory obstruction as a sign of brainstem dysfunction in infants with Chiari malformations

Laryngeal respiratory obstruction associated with Chiari malformations was first described in 1932. We studied this type of obstruction in six children with one or several disorders pointing to brainstem dysfunction (failure to thrive, velopharyngeal incompetence, gastroesophageal reflux, or vagal hypertonia). The nature of the laryngeal obstruction was highly variable (vocal cord paralysis, paradoxical vocal cord motion, laryngomalacia) as were the frequency and severity of associated disorders. Chiari malformations should be routinely sought in a child with laryngeal respiratory obstruction occurring at birth or later, whatever the endoscopic diagnosis, especially when signs of brainstem dysfunction are present. The best tool for diagnosing the Chiari malformation is T1- and T2-weighted MRI. Signs of brainstem dysfunction must be treated symptomatically, before treating Chiari malformations by decompressive surgery. This latter approach led to full functional recovery in all five children who underwent the procedure. Palliative surgical treatment should be reserved for patients in whom this procedure is unsuccessful.

Fatal encephalitis due to Nipah virus among pig-farmers in Malaysia

Between February and April, 1999, an outbreak of viral encephalitis occurred among pig-farmers in Malaysia. We report findings for the first three patients who died. Samples of tissue were taken at necropsy. Blood and cerebrospinal-fluid (CSF) samples taken before death were cultured for viruses, and tested for antibodies to viruses. The three pig-farmers presented with fever, headache, and altered level of consciousness. Myoclonus was present in two patients. There were signs of brainstem dysfunction with hypertension and tachycardia. Rapid deterioration led to irreversible hypotension and death. A virus causing syncytial formation of vero cells was cultured from the CSF of two patients after 5 days; the virus stained positively with antibodies against Hendra virus by indirect immunofluorescence. IgM capture ELISA showed that all three patients had IgM antibodies in CSF against Hendra viral antigens. Necropsy showed widespread microinfarction in the central nervous system and other organs resulting from vasculitis-induced thrombosis. There was no clinical evidence of pulmonary involvement. Inclusion bodies likely to be of viral origin were noted in neurons near vasculitic blood vessels. The causative agent was a previously undescribed paramyxovirus related to the Hendra virus. Close contact with infected pigs may be the source of the viral transmission. Clinically and epidemiologically the infection is distinct from infection by the Hendra virus. We propose that this Hendra-like virus was the cause of the outbreak of encephalitis in Malaysia.