Experience in treating children with ocular dyskinesia and hemifacial spasm secondary to pontine tumours adjacent to the fourth ventricle and systematic review.

OBJECTIVE: To investigate the treatment plan and prognosis of children with ocular dyskinesia and hemifacial spasm secondary to pontine tumours adjacent to the fourth ventricle. METHODS: In this retrospective study, the clinical information of 10 consecutively collected children with ocular dyskinesia and hemifacial spasm secondary to pontine tumours adjacent to the fourth ventricle was analyzed. All 10 children underwent pontine tumour resection through a trans-cerebellomedullary fissure approach; 4 children underwent preoperative diffusion tensor imaging scans to determine the relationship between the tumour and facial nerve nucleus, and the other 6 children underwent intraoperative deep electroencephalography (EEG) tumour monitoring, in which the tumour electrical discharge activity of the tumour was recorded. A voxel distribution map was established to describe the distribution of the tumour location, and patient prognosis was evaluated through clinical and imaging follow-up. RESULTS: All 10 children achieved total tumour resection; 9 tumours were pathologically suggested to be ganglioglioma (WHO grade I), and 1 was a hamartoma. The symptoms of the original ocular dyskinesia and hemifacial spasm disappeared immediately after the operation. The children were followed up for 4-75 months, and none of the symptoms recurred; four cases with preoperative diffusion tensor imaging showed that the tumour was close to the facial nerve. Four in six intraoperative electrophysiological monitoring showed that the tumour had electrical discharge behaviour, and the tumour distribution map indicates a high density of tumour presence in the facial nerve nucleus and the nucleus of the abducens nerve. CONCLUSIONS: In paediatric patients, the facial symptoms are related to the location and abnormal electrical discharge of the tumour. There is no significant correlation between ocular dyskinesia and the location of the tumour. Conventional antiepileptic therapy for this disease is ineffective, and early surgical intervention for total tumour resection can achieve a clinical curative effect.

Brainstem cavernous hemangioma with improvement of Holmes tremor on excision.

An 8-year-old boy presenting with left-angle paralysis, tremor in upper and lower extremities, and diplopia was diagnosed with hemorrhage from a mesencephalic cavernous hemangioma. He underwent hemangiomectomy through the occipital transtentorial approach 4 weeks post-hemorrhage, after which Holmes tremor (HT) markedly reduced. A year later, hemangioma has not recurred; he is now independent in his daily activities. Early intervention in the subacute stage allows for the complete removal of brainstem cavernomas (BSCs), with minimal risk of complications or sequelae. Proper timing and surgical approach for BSCs can prevent re-bleeding and improve HT after an initial hemorrhage, without any lasting negative consequences.

Diencephalic syndrome-like presentation of brainstem tumor: a series case-based review.

PURPOSE: Russel described a rare clinical entity known as diencephalic syndrome (DS) in 1951, which was traditionally caused by a neoplasm in the hypothalamic-optic chiasmatic region. DS is characterized by severe emaciation despite adequate or slightly reduced caloric intake, locomotor hyperactivity, euphoria and other minor features. Current evidence suggests that a rare population of children with a similar phenotype may have their tumor located in the posterior fossa instead, defining the DS-like presentation, a rare entity with few cases reported in the literature. METHODS: A thorough search of three databases (PubMed, Ovid Medline, and Ovid Embase) was conducted to identify relevant papers reporting children with DS associated with brainstem tumors. To our knowledge, only seven cases have been documented in the literature. Moreover, we present four of our own cases, focusing on the unusual clinical presentation, the diagnosis process, and the lag time between the initial symptoms and the definitive diagnosis. RESULTS: In this review, the mean lag time between the onset of symptoms and diagnosis was 20.9 months (median: 16 months; range: 1.5-72 months), whereas in our series of cases, the time was 32.5 months (median: 33 months; range: 7-57 months). CONCLUSION: Despite recent significant advances in neuro-oncology diagnostic tools, this mean lag time did not improve when compared with the previous literature review from 1976. Throughout these data, we aim to raise awareness in the hopes of detecting intracranial neoplasms earlier in cases of children with profound emaciation of unknown cause.

Trigeminal trophic syndrome in a pediatric patient with diffuse intrinsic pontine glioma.

A 13-year-old girl with a history of diffuse intrinsic pontine glioma (DIPG) suffered from progressively worsening facial ulcerations secondary to paresthesia-induced self-excoriation. She was diagnosed with trigeminal trophic syndrome (TTS) induced by DIPG and struggled to heal her lesions in the background of this excoriation disorder. A multidisciplinary approach that included mood disorder management with sertraline and amitriptyline helped diminish paresthesia, improve her quality of life, and promote healing of the ulcers despite the progression of her DIPG. This case highlights the multifactorial complexity of TTS in pediatric patients and the need for successful management strategies.

Glioblastoma multiforme with oculomotor nerve involvement: case report and literature review.

Gliomas involving the cranial nerves III-XIII are rare. Even rarer are glioblastomas multiforme (GBMs) with only 10 cases previously reported. Oculomotor nerve involvement was described in only 2 patients. The mechanisms proposed so far include an origin from the nerve itself or an extension within the nerve of a midbrain tumor. We report the case of a 69-year-old man who presented with an isolated left oculomotor nerve palsy. He was found to have a left temporal GBM extended to the frontal lobe. Diagnostics and intraoperative and pathological findings clearly demonstrated a massive infiltration of the cisternal portion of the left oculomotor nerve. We suppose this could be the first case of direct oculomotor nerve invasion by exophytic spread of a supratentorial GBM or by subarachnoid seeding from a temporal tumor. Less probably, it could be the first case of an oculomotor nerve GBM with a temporal lobe invasion.

Brainstem tumors may increase the impairment of behavioral emotional cognition in children.

PURPOSE: It remains unclear as to whether patients with brainstem tumor experience complex neuropsychiatric problems. In this cohort study, we specifically investigated behavioral, emotional and cognitive symptoms in pediatric patients with brainstem glioma and healthy individuals. METHODS: A total of 146 patients with pediatric brainstem tumors (aged 4-18 years old) and 46 age-matched healthy children were recruited to assess their behaviors and emotions examined by the Child Behavior Checklist. A variety of clinical factors were also analyzed. RESULTS: There were significant differences in most behavioral and emotional symptoms between pediatric patients and healthy subjects. Moreover, patients with pons tumors exhibited significantly higher scores than patients with medulla oblongata tumors (p = 0.012), particularly in concerning the syndrome categories of Withdrawn (p = 0.043), Anxious/depressed symptoms (p = 0.046), Thought Problems (p = 0.004), Attention deficits (p = 0.008), Externalizing problems (p = 0.013), and Aggressive behavior (p = 0.004). A tumor body located in the pontine (p = 0.01, OR = 4.5, 95% CI = 1.4-14.059) or DIPG in the midbrain (p = 0.002, OR = 3.818, 95% CI = 1.629-8.948) appears to act as a risk factor that is associated with more problems in patients with neuropsychiatric symptoms. CONCLUSIONS: Pediatric patients with brainstem tumors exhibit severe behavioral and emotional problems. Tumor invades the pontine and midbrain act a risk factor with more problems. It suggests that structural and functional abnormalities in the brainstem will cause prolonged behavioral problems and emotional-cognitive dysfunctions in young children.

Ketogenic diet treatment in recurrent diffuse intrinsic pontine glioma in children: A safety and feasibility study.

BACKGROUND: The mean overall survival rate of children with diffuse intrinsic pontine glioma (DIPG) is 9-11 months, with current standard treatment with fractionated radiotherapy and adjuvant chemotherapy. So far, novel therapeutic strategies have not yet resulted in significantly better survival. The main source of energy for glioblastoma cells is glucose. Therefore, metabolic alterations induced by the use of the extremely carbohydrate-restricted ketogenic diet (KD) as adjuvant therapy are subject of interest in cancer research. PROCEDURE: This study explores the safety and feasibility of the KD in children with recurrent DIPG and no remaining treatment options. Safety was defined as the number of adverse effects. Feasibility was defined as the number of patients who were able to use the KD for three months. Coping of patients and parents was measured with questionnaires. RESULTS: Three of 14 children referred to our hospital between 2010 and 2015 were included. Two patients completed the study, and one died before the end of the study. Hospitalizations were needed for placing a nasogastric tube (n = 1) and epileptic seizures (n = 1). Adverse effects related to the diet were mild and transient. Parents were highly motivated during the study. CONCLUSION: Use of KD is safe and feasible, but the effect on survival has to be proven in a larger cohort of children who start the KD earlier after diagnosis, preferably as adjuvant therapy to fractionated radiotherapy.

Unilateral hyperhidrosis secondary to brainstem meningioma producing mass effect.

Unilateral hyperhidrosis of neurological origin has been associated with head trauma, cerebral palsy, spinal cord injury, peripheral neuropathy, lesions of the hypothalamus, and cerebral or brainstem strokes. In this report, we describe a 61-year-old man with isolated sweating on the left side of his entire body. A right-sided brainstem meningioma producing mass effect is suspected as the underlying etiology.

Recurrent Bell's palsy following ventriculoperitoneal shunt insertion: an unusual case to face.

We present the case of a 15 year old boy who developed transient lower motor neuron facial weakness on two separate occasions shortly after ventriculoperitoneal shunt insertion. Both episodes, each of which occurred on the ipsilateral side to shunt insertion, were transient, self-limiting and were managed medically with a course of oral steroids. We believe this is the first reported case of Bell's palsy after this type of surgery in a child. Potential pathophysiological mechanisms are discussed.

Rebleeding and Outcome in Patients with Symptomatic Brain Stem Cavernomas.

PURPOSE: We sought to evaluate the long-term functional outcomes and identify the potential risk factors for rebleeding in patients with brain stem cavernous malformations (BCMs) who presented with hemorrhages and were surgically or conservatively treated and prospectively monitored. METHODS: From January 1990 to July 2015, we included patients with first hemorrhagic episodes secondary to single BCMs. Modified Rankin score (mRS) was used for neurological status assessment. Univariate and multivariate regression statistics were used to identify the risk factors for rebleeding. RESULTS: A total of 99 patients with BCMs hemorrhages were included (59 [59.6%] women, mean age 37± 13 years). As initial treatments, 37 patients (37.4%) underwent surgery and 62 (62.6%) received conservative treatment. The median follow-up was 3.33 years (interquartile range 1.16-7 years; 408.3 patient/years). The rebleeding rate by patient/year was 10% in conservatively treated patients. Deterioration was significantly more frequent in patients with rebleeding (p = 0.0001). At the end of the follow-up, the mRS were favorable in 49 patients (65.3%) without rebleeding, whereas only 8 (33.3%) with rebleeding evolved to favorable outcomes (p = 0.006). Lesion size >18 mm (hazards ratio, HR 3.34, 95% CI 1.54-7.26; p = 0.0001) and ventral location or crossing the brain stem's midpoint (HR 2.5, 95% CI 1.14-5.46; p = 0.022) were associated with a major risk of rebleeding in the univariate analysis, but only a lesion >18 mm remained statistically significant (HR 2.7, 95% CI 1.2-6.21; p = 0.016) in the multivariate analysis. CONCLUSION: A lesion size >18 mm was the principal factor associated with hemorrhage recurrence. The overall functional outcome was good. However, significant morbidity was attributable to rebleeding.

Tectal glioma presenting with clinical triad of obesity, amenorrhea and central cord syndrome with radiological pentad of hydrocephalus, empty sella, suprapineal diverticula, Chiari and syrinx.

BACKGROUND: Tectal gliomas (TG) are slow-growing tumors and generally present with features of increased intracranial pressure. CASE DESCRIPTION: We describe an unusual case of a young girl who presented with clinical triad of obesity, primary amenorrhea and central cord syndrome. The radiology unveiled a pentad of hydrocephalus, empty sella, suprapineal recess diverticulum, secondary Chiari malformation and cervical syringomyelia, masking a relatively obscure tectal plate glioma. She was subjected to endoscopic third ventriculostomy (ETV). All of her symptoms improved after ETV and the tumor is being followed up. The possible pathogenesis of such a highly atypical clinico-radiological presentation is described. CONCLUSION: This unusual syndromic presentation of tectal glioma without clinical features of increased intracranial pressure is probably due to increased compliance of third ventricular walls, and is relieved with ETV.

Brainstem and Autonomic Nervous System Dysfunction: A Neurosurgical Point of View.

Central autonomic control nuclei and pathways are mainly integrated within the brainstem, especially in the medulla oblongata. Lesions within these structures can lead to central dysautonomia.Central autonomic control structures can be damaged by tumors, during surgery, or by other neurosurgical pathologies. These may elicit clinical or subclinical autonomic complications that can constitute a serious clinical problem.The authors present a broad review of the central autonomic nervous system, its possible dysfunctions, and the relation between neurosurgery and this "not-well-known system". Preliminary results of an autonomic study of brainstem lesions that is currently being carried out by the authors are also shown.

MRI capture of intermittent ventriculomegaly in a patient with ventriculo-peritoneal shunt.

Intermittent change in ventricular size in patients with ventriculo-peritoneal shunts is a recognised complication but definitive imaging evidence is rare. We report a 3 years old boy with a medullary astrocytoma and ventriculo-peritoneal shunt placement who demonstrated intermittent ventriculomegaly during a single MRI scan.

Cognitive outcomes among survivors of focal low-grade brainstem tumors diagnosed in childhood.

Pediatric focal low-grade brainstem tumors are associated with excellent prognosis. Surgical resection and conformal radiation therapy are front-line treatment options; radiation therapy (RT) serves as an excellent treatment for disease progression. Given high survival rates and limited research regarding functional outcomes, the current study examined neurocognitive outcomes in a group of low-grade brainstem glioma survivors. Forty-three survivors of focal low-grade brainstem gliomas underwent neurocognitive assessment (58 % male; median = 6.9 years at diagnosis; median = 14.9 years at latest assessment). Treatment included combinations of surgery, chemotherapy, and RT with 70 % ultimately receiving RT. Neurocognitive outcomes were evaluated through retrospective chart review. Intellectual and academic performance were significantly different from normative expectations (full scale IQ = 86.5 ± 16.8; reading comprehension = 91.3 ± 16.4; math reasoning = 88.2 ± 18.9; reference group = 100 ± 15). Further, the percentage performing below average exceeded the expected 16 % in the normative sample (full scale IQ = 43 %; reading comprehension = 37 %; math reasoning = 50 %). Mean parent ratings did not reflect concerns regarding internalizing and externalizing behaviors or executive functioning (internalizing = 54.9 ± 12.7; externalizing = 51.6 ± 14.6, global executive composite = 57.1 ± 16.0; reference group = 50 ± 10); however, the proportion with clinically elevated scores was higher than the expected 16 % (internalizing = 42 %; externalizing = 26 %; global executive composite = 38 %). Mean performance fell below average for visual-motor coordination (81.8 ± 13.2) and parent ratings of adaptive functioning (73.4 ± 24.2), with 65 and 62 % falling outside the average range, respectively. There were no significant differences between those receiving and not receiving RT. Multiple cognitive domains were significantly different from normative expectations. Despite focal disease and treatment targeting subcortical brain regions, neurocognitive risks exist that may impact treatment planning and caregiver education.

Endoscopic third ventriculostomy for hydrocephalus in brainstem glioma: a case series.

OBJECT: A brainstem glioma is an incurable brain tumor that can be complicated by hydrocephalus. A ventriculoperitoneal (VP) shunt is generally performed for the control of hydrocephalus. This study aimed to reveal the safety and efficacy of an endoscopic third ventriculostomy (ETV) for hydrocephalus in brainstem gliomas. METHODS: Six patients who had pontine glioma with hydrocephalus underwent an ETV between May 2010 and November 2015. In all the cases, there were one or more symptoms of hydrocephalus (headache, nausea, vomiting, or lethargy). Retrospective review of these patients was performed using the medical records and neuroimagings. RESULT: The ETV was performed safely and there were no intraoperative complications in all patients. The mean follow-up period was 12.3 months. An immediate symptomatic relief of hydrocephalus and an adequate control of symptoms were achieved without a VP shunt in all patients. CONCLUSIONS: The ETV is considered to be an effective and safe procedure for the treatment of hydrocephalus in brainstem gliomas. Determining the ventriculostomy site according to the preoperative MRI in each case is considered to be important for the safe procedure.

Surgical Approach to Ponto-mesencephalic Cavernoma.

BACKGROUND AND AIMS: The surgical treatment of intrinsic brainstem lesions remains a major challenge. In this article we present the results of using an infratentorial-supracerebellar (ITSC) approach for the resection of intrinsic ponto-mesencephalic lesions. MATERIALS AND METHODS: The authors reviewed the cases of 16 patients. In seven of them, a paramedian ITSC transcollicular approach was used to resect intrinsic mid-brain lesions, and in the other nine patients, an intermediate or lateral ITSC infra-trochlear approach was used for ponto-mesencephalic lesions. RESULTS: All 16 lesions were completely removed. There was no recurrence of bleeding during the follow-up period, and no mortality. In seven patients with mid-brain lesions, the preoperative ocular symptoms improved in three of them, and the neurological deficits - other than ocular symptoms improved - in five of them. The preoperative modified Rankin Scale score of 1.8 improved to 1.3 postoperatively. In nine patients with a ponto-mesencephalic lesion, the preoperative ocular symptoms improved in four of nine patients, and the neurological deficits other than ocular symptoms improved in five of them, while one patient exhibited venous infarction in a cerebellar hemisphere that caused neurological deterioration. The preoperative modified Rankin Scale score of 3.75 improved to 2.5 postoperatively in these patients. CONCLUSION: The ITSC transcollicular or infra-trochlear approach provide a wide operative entry zone and minimize the functional damage to the surrounding structures for access to ponto-mesencephalic lesions.

A novel treatment-responsive encephalitis with frequent opsoclonus and teratoma.

Among 249 patients with teratoma-associated encephalitis, 211 had N-methyl-D-aspartate receptor antibodies and 38 were negative for these antibodies. Whereas antibody-positive patients rarely developed prominent brainstem-cerebellar symptoms, 22 (58%) antibody-negative patients developed a brainstem-cerebellar syndrome, which in 45% occurred with opsoclonus. The median age of these patients was 28.5 years (range = 12-41), 91% were women, and 74% had full recovery after therapy and tumor resection. These findings uncover a novel phenotype of paraneoplastic opsoclonus that until recently was likely considered idiopathic or postinfectious. The triad of young age (teenager to young adult), systemic teratoma, and high response to treatment characterize this novel brainstem-cerebellar syndrome.

Pediatric tectal plate gliomas: a review of clinical outcomes, endocrinopathies, and neuropsychological sequelae.

Pediatric tectal plate gliomas are indolent slow-growing gliomas that often present with increased intracranial pressure or incidentally on routine brain imaging. We investigated clinical outcomes, endocrinopathies, and neuropsychological sequelae associated with tectal plate gliomas. Twenty-six patients with tectal plate glioma were identified in a 20-year retrospective review. Clinical outcomes, treatments, endocrine function, neuropsychological testing outcomes and radiographic imaging were reviewed for possible signs correlating with tumor progression. Among 26 patients, 19 presented with signs or symptoms of increased intracranial pressure (73 %) versus an incidental finding in 7 (27 %). Median follow-up was 46 months (range 8-143 months). Six of 26 (23 %) experienced progressive disease after diagnosis. Five of 26 (19 %) required more than one surgical procedure due to failure of initial endoscopic third ventriculostomy. Seven of 26 had history of endocrine dysfunction, of which, five presented with endocrine dysfunction (precocious puberty or short stature), 1 developed menstrual irregularities after surgical intervention and 1 had preexisting pan hypopituitarism. Of 12 patients with available neuropsychological testing, eleven had at least one indicator of executive functioning in the low-average to impaired range. While tectal plate gliomas have been considered indolent tumors that are rarely progressive, 23 % of patients in our cohort experienced disease progression and required further therapy. Neurocognitive deficits may occur, while endocrine deficiency is uncommon. Regular multidisciplinary oncology follow-up, routine monitoring with MRI and formal neurocognitive evaluation are imperative to provide early recognition of disease progression or recurrent hydrocephalus and to improve school functioning in this population.

Metastatic pancreatic neuroendocrine tumor to the central nervous system in a patient with von Hippel-Lindau disease: A case report and literature review.

Pancreatic neuroendocrine tumor (NET) is frequently encountered in patients with von Hippel-Lindau disease (VHL) and uncommonly metastasizes to the central nervous system. Here, we present the case of a VHL patient with symptomatic pancreatic NET metastases to both the cervical spinal cord and a preexisting brainstem hemangioblastoma (e.g., tumor- to-tumor metastasis).