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Pediatric Oncology
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Stephen Lowis, Rachel Cox, John Moppett, Helen Rees
The clinical presentation of children with CNS tumors varies with age, development, and the site of origin of the tumor. Infratentorial (brain stem and cerebellar) tumors may present with disturbances of truncal steadiness, upper extremity co-ordination and gait, and cranial nerve function.Supratentorial tumors may present with features of raised intracranial pressure, irritability, seizures, regression of developmental milestones, and upper motor neuron signs such as hemiparesis.Optic chiasm tumors may result in visual field defects such as a bitemporal hemianopia, nystagmus, and head tilt.Hypothalamic tumors may give rise to the diencephalic syndrome (failure to thrive, euphoria, and hyperactivity), and endocrine disorders such as diabetes insipidus, hypogonadism, and precocious puberty.
Craniopharyngioma
Published in David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack, Brain and Spinal Tumors of Childhood, 2020
Hermann L. Müller, Claire Alapetite, Jeffrey Wisoff
Even though hypothalamic obesity is a frequent sequela in childhood-onset adamantinomatous craniopharyngioma,257 diencephalic syndrome leading to severe weight loss and cachexia can also occur as a rare hypothalamic disturbance of body composition in childhood-onset craniopharyngioma.262,263 Hoffmann et al.263 analyzed the incidence of diencephalic syndrome, its clinical manifestations before and after diagnosis of childhood-onset craniopharyngioma, and outcome in 485 patients recruited in the German childhood craniopharyngioma registry. Only 4.3% of all childhood-onset craniopharyngioma patients presented with low weight (BMI <–2 sd) at time of diagnosis. Initial significant differences between patients with low weight at the time of diagnosis and normal-weight patients at diagnosis are usually observed at 5 years of age. Within the first 2 years after diagnosis of childhood-onset craniopharyngioma, the BMI of diencephalic syndrome patients and normal-weight patients converge to a similar level. Hoffmann et al. concluded from their analysis of patients’ histories that diencephalic syndrome at the time of diagnosis does not preclude subsequent weight gain caused by a childhood-onset craniopharyngioma with hypothalamic involvement.
The role of complement and complement therapeutics in neuromyelitis optica spectrum disorders
Published in Expert Review of Clinical Immunology, 2022
Panos Stathopoulos, Marinos C Dalakas
NMOSD, previously called Devic’s disease, is characterized by recurring episodes of severe unilateral or bilateral optic neuritis with myelitis commonly affecting more than three consecutive vertebral segments (referred to as Longitudinal Extensive Transverse Myelitis-LETM). Optic neuritis and myelitis can occur concurrently, in quick succession, or in temporal isolation [8]. In addition, NMOSD with AQP4 autoantibodies may also present with or concurrently exhibit intractable hiccups, nausea, and vomiting (area postrema syndrome) or with acute brainstem and diencephalic syndrome [2]. The underlying pathology in affected areas is characterized by cellular necrosis, which is clinically reflected in the severity of symptoms; optic neuritis presents with very low visual acuity or even blindness and the myelitis with paraparesis or even paraplegia with involuntary muscle spasms. Recovery may be suboptimal with significant residual deficits and disability, hence the need for preventive immunotherapy to stop recurrent relapses. An acute AQP4 NMOSD episode is considered an immunological emergency requiring immediate intervention and early immunotherapy initiation.
Neuro-Ophthalmic Literature Review
Published in Neuro-Ophthalmology, 2021
David A. Bellows, Noel C.Y. Chan, John J. Chen, Hui-Chen Cheng, Peter W. MacIntosh, Jenny A. Nij Bijvank, Michael S. Vaphiades, Konrad P. Weber, Sui H. Wong
This retrospective study reported the demographics, clinical manifestations, relapses, anti AQP4-IgG status, imaging and treatment of relapses in 30 patients who were myelin oligodendrocyte glycoprotein (MOG) antibody positive seen at an Indian tertiary centre. MOG-IgG related manifestations were monophasic/recurrent/simultaneous optic neuritis (ON), myelitis, recurrent acute disseminated encephalomyelitis (ADEM), brainstem encephalitis and diencephalic syndrome. MRI features suggestive of MOG-IgG disease were confluent ADEM-like lesions, middle cerebellar peduncle fluffy lesions, longitudinally extensive transverse myelitis (LETM), longitudinally extensive optic neuritis (LEON) and non-LEON. Fifty percent patients had a relapsing course. Plasma exchange and intravenous immunoglobulin worked in patients who showed a poor response to intravenous methylprednisolone. Prednisolone, azathioprine, mycophenolate and rituximab were effective attack preventing agents. Both moderate and severe relapses were seen with an overall good response to IVMP. MOG-IgG disease usually responded well to maintenance oral steroids and “gentle” immunosuppression was often adequate to prevent relapses. Some patients were “steroid dependent.”
40th Meeting of the Upper Midwest Neuro-Ophthalmology Group
Published in Neuro-Ophthalmology, 2019
Megan Tuohy, MD, University of Michigan, described two children who began to refuse food in the first year of life and became emaciated. An extensive metabolic evaluation failed to yield an explanation for this life-threatening phenomenon, which was initially attributed to behavioral issues on the part of the children or their parents. Months later, parents noticed ocular oscillations, triggering brain MRI that revealed massive diencephalic tumours, which were pilocytic astrocytomas as part of Russell diencephalic syndrome. Vinca alkaloid-based and platinum-based chemotherapy finally arrested tumour growth and led to the regaining of developmental milestones and disappearance of nystagmus in one child and stabilization of weight loss in the other. Dr Tuohy emphasized the importance of looking for nystagmus, which occurs in most children with this condition. Delayed diagnosis may unfavorably affect outcome, which is often dire.