Symptoms Of Brainstem Dysfunction Research Articles

Symptoms associated with headache in youth.

To determine the underlying relationships between a broad range of headache-associated symptoms and how they relate to headache burden. Symptoms associated with head pain inform classification of headache disorders. However, many headache-associated symptoms are not included in the diagnostic criteria, which is largely based on expert opinion. Large symptom databases can assess headache-associated symptoms irrespective of pre-existing diagnostic categories. We conducted a large single-center cross-sectional study on youth (6-17 years old) assessing patient-reported outpatient headache questionnaires between June 2017 and February 2022. Multiple correspondence analysis, an exploratory factor analysis, was applied to 13 headache-associated symptoms. 6662 participants (64% female; median age 13.6 years) were included. Multiple correspondence analysis dimension 1 (25.4% of the variance) captured the absence or abundance of headache-associated symptoms. A greater number of headache-associated symptoms correlated with greater headache burden. Dimension 2 (11.0% of the variance) revealed three symptom clusters: (1) cardinal features of migraine (light, sound, and smell sensitivity, nausea, and vomiting), (2) nonspecific global neurologic dysfunction symptoms (lightheadedness, trouble thinking, blurry vision), (3) vestibular and brainstem dysfunction symptoms (vertigo, balance problems, ear ringing, double vision). Assessing a broader range of headache-associated symptoms reveals clustering of symptomatology and a strong relationship with headache burden.

Care management and contemporary challenges in spina bifida: a practice preference survey of the American Society of Pediatric Neurosurgeons.

Neurosurgical management preferences related to myelomeningocele (MMC) care demonstrate significant variability. The authors sought to evaluate variability in practice patterns across a group of senior pediatric neurosurgeons. The purpose of this study was to identify the extent of variability and of consensus with regard to neurosurgical management of MMC and associated hydrocephalus, Chiari II malformation, and tethered spinal cord. A 43-question survey was distributed electronically to the members of the American Society of Pediatric Neurosurgeons (ASPN). The survey covered domains such as clinic case volume, newborn management, hydrocephalus management, transition to adulthood, clinical indications for shunt revision, Chiari II malformation decompression (C2MD), and tethered cord release (TCR). Ninety responses were received from 200 active ASPN members, for an overall response rate of 45%. The majority (58%) of respondents closed 5-15 new cases of open MMC per year. Nearly all (98%) respondents perform back closure within 48 hours of birth, with the majority imbricating the placode and striving for a 3- to 4-layer closure. The most consistent indications for surgical intervention in early hydrocephalus were CSF leak from the back (92%), progressive ventricular enlargement (89%), and brainstem symptoms, including apnea/bradycardia (81%), stridor (81%), and dysphagia (81%). Eighty percent of respondents indicated that spina bifida care is delivered through multidisciplinary clinics, with neurosurgery, orthopedic surgery, urology, physical therapy, and social work as the most common disciplines included. One-third of clinics see both pediatric and adult patients, one-third offer a formal transition program to adult care, and one-third have no transition program. The vast majority of respondents offer prenatal counseling (95%), referral for in utero closure (66%), and endoscopic third ventriculostomy/choroid plexus cauterization (72%). Respondents were more willing to perform shunt revision for symptoms alone than for image changes alone. An asymptomatic broken shunt without ventricular enlargement produced responses evenly divided between observation, intervention, and further investigation. Operative shunt exploration was always performed before C2MD by 56% of respondents and performed sometimes by 40% of respondents. Symptoms of brainstem dysfunction were the strongest clinical triggers reported for C2MD, while declines in urinary continence, leg strength or sensation, or ambulation were the most consistent thresholds for TCR. Significant disparities exist surrounding key areas of decision making regarding treatment for patients with MMC, though there are central areas of agreement among ASPN members. Additionally, there is significant variation in the clinical management of chronic hydrocephalus, C2MD, and TCR, underscoring the need for further research into these specific areas.

The Management of Birth-Related Posterior Fossa Hematomas in Neonates

Symptomatic posterior fossa hematoma in the term newborn is rare. To report on the management and outcome of posterior fossa subdural hematoma (PFSDH) in neonates. A retrospective analysis of the department database and clinical notes of neonates admitted since 1985 with a PFSDH was performed together with a literature review of similar case series. Sixteen patients were included. The median gestational age was 40 weeks with a high proportion of primiparous mothers (n = 9) and forceps delivery (n = 9). Nine neonates had symptoms of brainstem dysfunction within the first 24 hours of life, whereas the other 7 had a delayed presentation (median 4 days) with signs of raised intracranial pressure due to hydrocephalus. Each patient had a cranial ultrasound followed by computed tomography scan that showed the PFSDH. Eleven neonates required surgical evacuation of the PFSDH, whereas hydrocephalus was managed by transient external ventricular drainage in 2 further patients. Eventually, 2 neonates required a permanent ventriculoperitoneal shunt. Five neonates had no operative intervention. With a mean follow-up of 7.8 years, 2 patients had mild developmental delay and 1 had severe developmental delay. The 13 other patients had a normal development. In neonates with a PFSDH, surgery can be safely performed in those who have clinical and radiological signs of brainstem compression or hydrocephalus. A small number of neonates require a ventriculoperitoneal shunt in the long term. Initial aggressive resuscitation should be performed even in cases of initial severe brainstem dysfunction because of the good long-term neurological outcome.

EPILEPSY IN CHILDREN WITH INFANTILE THIAMINE DEFICIENCY

Objective: To report the follow-up findings of 7 children with severe epilepsy as a result of thiamine deficiency in infancy caused by a defective soy-based formula. Methods: The medical records of 7 children aged 5-6 years with thiamine deficiency in infancy who developed epilepsy were reviewed and their clinical data, EEG tracings, and neuroimaging results were recorded. The clinical course and present outcome of these children, now 5 years after exposure to thiamine deficiency, are described. Results: All infants displayed seizures upon presentation, either tonic, myoclonic, or focal. Six infants had an EEG recording at this stage and all showed slow background. Five of them had no epileptic activity and only 1 displayed focal activity. Following a seizure-free period of 1-9 months, the seizures recurred, and all 7 children displayed either myoclonic or complex partial seizures. Multifocal or generalized spike wave complexes were recorded on the EEGs of all 7 patients, and the tracings of 3 children evolved into hypsarrhythmia. The seizures were refractory to most antiepileptic drugs, and 4 children remain with uncontrolled seizures. All children have mental retardation and motor disabilities as well as symptoms of brainstem dysfunction. Conclusions: Our findings indicate that severe infantile thiamine deficiency may result in epilepsy.

Somatosensory evoked potentials in Arnold–Chiari malformation

Nearly all patients with repaired myelomeningoceles have an Arnold–Chiari (AC) malformation and about 20% of these patients develop clinical signs of brainstem dysfunction. The management of symptomatic AC malformation is still controversial and techniques are needed to provide an objective assessment of brainstem function. We recorded somatosensory evoked potentials (SEPs) in 52 patients aged between 8 months and 20 years (median 7.3 years) with AC malformation, to determine whether the SEPs discriminate patients with symptomatic AC malformation from those without symptoms. The subcortical far-field components P13, P14 and N18, which are generated within the brainstem, were recorded with non-cephalic reference electrodes and the cortical N20 with a frontal reference. Fourteen patients (27%) had signs and symptoms of brainstem dysfunction, which were related to the AC malformation. Abnormal SEPs were mainly recorded in symptomatic patients (sensitivity 0.7, specificity 0.9). The SEPs were particularly useful in patients from 4 years of age (sensitivity 0.9, specificity 0.9), but not in the younger age group. Abnormal somatosensory conduction reflects dysfunction of the brainstem or the upper cervical cord and may be clinically useful to assess patients with late onset symptomatic AC malformation.

Brainstem cranial-nerve lesions in an infant with hypoxic cerebral injury

Recent studies have revealed that the brainstem is characteristically involved in hypoxic encephalopathy in infants. However, few reports have described clinical and cranial magnetic resonance findings in detail because most patients with brainstem lesions die during delivery or soon afterward. Reported here is a patient who manifested selective brainstem injury caused by perinatal hypoxic cerebral injury and exhibited palsy of the lower-brainstem cranial nerves. Magnetic resonance imaging was used to demonstrate lesions of brainstem cranial nerves, which were consistent with symptoms of brainstem dysfunction.

Malformación de Chiari tipo II con disfunción paroxística de tronco. ¿Qué se puede hacer?

The Chiari type II malformation is the main cause of death in children with myelomeningocele, usually due to respiratory dysfunction, and unfortunately there is no effective cure. Episodic symptoms of brain stem dysfunction are frequent. We report a child with Chiari type II malformation and brain stem dysfunction who required tracheotomy and who was fed through a nasogastric tube from the age of 1.5 months. He first presented episodic symptoms at the age of 8 months and died at 9 months due to respiratory dysfunction despite intensive care. We also report our experience in the last 12 years with four patients with Chiari type II malformation and episodic symptoms of brain stem dysfunction. Both patients who required mechanical ventilation died. Neither surgical decompression nor intensive care prevented the fatal outcome, which was unpredictable and inevitable. Prognosis may be more positive in less severe cases, because the two patients that did not require mechanical ventilation show favorable outcome to date.