Reference Range of Cerebrospinal Fluid Opening Pressure in Children: Historical Overview and Current Data

Avery, R. A.

doi:10.1055/s-0034-1376202

Cited by 48 publications

(36 citation statements)

References 19 publications

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“…In another retrospective observational study, the highest opening pressure in pediatric IIH was between 20 and 24 cmH 2 O in 11% of the patients while it was between 25 and 39 cmH 2 O in 48% and more than 40 cmH 2 O in 42% of the patients [16]. In a newer study, opening pressure ≥ 28 cmH 2 O is mandatory for diagnosis of intracranial hypertension for obese patients and 25 cmH 2 O if the patient is not obese [13].…”

Section: Discussionmentioning

confidence: 93%

“…The clinical presentation of IIH is variable and the diagnostic criteria have changed over time, but currently, Dandy criteria and its modifications which involve symptoms and signs of increased intracranial pressure with elevated CSF pressure without laboratory or radiological evidence of intracranial lesion, infection, or hydrocephalus are used for diagnosis of IIH in adult patients [2,4,5,12]; however, there is no agreement for specific diagnostic criteria in pediatric IIH due to controversies about the diagnostic value of CSF opening pressure [13][14][15][16], in addition to the difference in the presentation and clinical finding between children and adolescent patients [2,16].…”

Section: Introductionmentioning

confidence: 99%

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Idiopathic intracranial hypertension in pediatric and adolescent patients

et al. 2019

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Objectives: Pediatric idiopathic intracranial hypertension is a rare condition, but inappropriate diagnosis and management may lead to devastating outcome with loss of vision and lifelong handicap. Dandy criteria are used for diagnosis of idiopathic intracranial hypertension (IIH) in adult, but these criteria cannot be applied in all pediatric patients. The aim of this study is to evaluate the diagnostic criteria and outcome of management of IIH in pediatric patients. Methods: Nineteen patients with IIH and age less than 16 years old have been evaluated for the diagnostic criteria and outcome of management. The patients were classified according to the secondary sexual criteria into adolescent IIH and pediatric IIH. Full neurological and ophthalmological evaluation was completed in all patients. The patients were managed and followed up for a mean period of 12 ± 8.6 months at Zagazig University hospitals or Kingdom Hospital in the time period from 2009 to 2018. Results: Nineteen patients (16 females and 3 males) had been diagnosed and treated with idiopathic intracranial hypertension, their age is between 4 and 15 years, and the patients have been divided into two groups. Group I (pediatric IIH) involved 9 patients (6 females and 3 males), and their age is ranging between 4 and 9 years with mean age 5.56 ± 1.9 years while group II (adolescent IIH) involved 10 patients, all of them are females and their age ranges between 12 and 15 years with mean age 13.5 ± 1.3. Diagnostic criteria of the patients are papilledema, symptoms and signs of intracranial hypertension, and elevated CSF opening pressure with normal MRI. Headache, delayed school performance, and sixth nerve palsy are the most common clinical finding in the patients of group I while headache is the most common presenting symptom in group II. Anemia and otitis media are the most common associated risk factors in group I while obesity and female gender were the most common associated risk factors in group II. The minimum value for opening pressure in group I is 180 mmH 2 O while the minimum value in group II is 250 mm. 16 patients improved after the first lumbar puncture and drainage of CSF; two patients improved after repeated lumbar puncture while lumbo-peritoneal shunt was inserted for two patients, both of them were in group II. Recurrence was diagnosed in four patients (21%), one of them (11.1%) was in group I while three of them (30%) were in group II. Conclusion: Pediatric idiopathic intracranial hypertension can be classified into two subtypes: pediatric type and adolescent type according to the secondary sexual criteria. Modified Dandy criteria can be applied for the diagnosis of adolescent type, but pediatric type is not associated with obesity, has no female predominance, and usually responds to the initial lumbar puncture with a low rate of recurrence.

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Section: Discussionmentioning

confidence: 93%

Section: Introductionmentioning

confidence: 99%

Idiopathic intracranial hypertension in pediatric and adolescent patients

et al. 2019

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“…Avery, et al suggested that the reference range for CSF opening pressure in children should be reconsidered, based on a study of cerebrospinal fluid opening pressure in 472 healthy children between the ages of 1 and 18 years that determined the CSF opening pressure 90th percentile in the healthy study population was 28 cm of water [43]. In our present qMRI study, CSF opening pressure rose above 20 cm and progressed well above 28 cm in all infantile patients for whom sequential measurements were obtained.…”

Section: Discussionmentioning

confidence: 99%

Distinct progression patterns of brain disease in infantile and juvenile gangliosidoses: Volumetric quantitative MRI study

Nestrašil

Ahmed

Utz

et al. 2018

Molecular Genetics and Metabolism

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Background GM1-gangliosidosis and GM2-gangliosidosis (Tay-Sachs disease and Sandhoff disease) are unrelenting heritable neurodegenerative conditions of lysosomal ganglioside accumulation. Although progressive brain atrophy is characteristic, longitudinal quantification of specific brain structures has not been systematically studied. Objectives The goal of this longitudinal study has been to quantify and track brain MRI volume changes, including specific structure volume changes, at different times in disease progression of childhood gangliosidoses, and to explore quantitative brain MRI volumetry (qMRI) as a non-invasive marker of disease progression for future treatment trials. Methods Brain qMRI studies were performed in 14 patients with gangliosidoses (9 infantile, 5 juvenile) yearly. Cerebellar cortex and white matter, caudate, putamen, corpus callosum, ventricles, total brain, and intracranial volumes were measured, as well as total brain volume. Age-matched controls were available for the patients with the juvenile phenotype. Results The infantile phenotype of all gangliosidoses showed a consistent pattern of macrocephaly and rapidly increasing intracranial MRI volume with both (a) brain tissue volume (cerebral cortex and other smaller structures) and (b) ventricular volume (P < 0.01 for all). In contrast to apparent enlargement of the total brain volume, and chiefly the enlarged cerebral cortex, a subset of smaller brain substructures generally decreased in size: the corpus callosum, caudate and putamen became smaller with time. The volume of cerebellar cortex also decreased in patients with infantile GM1-gangliosidosis and juvenile GM1- and GM2-gangliosidosis; however, infantile GM2-gangliosidosis cerebellar cortex was the exception, increasing in size. Elevated intracranial pressure (estimated by lumbar spinal pressure) was a common finding in infantile disease and showed continued increases as the disease progressed, yet lacked MRI signs of hydrocephalus except for increasing ventricular size. Notably, in patients with juvenile gangliosidosis, macrocephaly and elevated intracranial pressure were absent and total brain volume decreased with time compared to controls (P = 0.004). Conclusions The disease course of infantile versus juvenile gangliosidoses is clearly distinguished by the rate of brain disease progression as characterized by qMRI. Assessments by qMRI represent a robust non-invasive method for monitoring CNS changes in the clinical course of gangliosidoses and is ideally suited to monitor effects of novel CNS-directed therapies in future clinical trials.

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“…analyzed the OP of 197 children and found a mean OP of 19.6 cm H 2 023. Furthermore, given the 10 th /90 th percentage of Avery’s data was 11.5 and 28 cm H 2 O, it has been suggested that an abnormal OP should be consider that of >28 cm H 2 O2324. Six of 25 MPS-IH patients had OP greater than 30 cm H 2 O.…”

Section: Discussionmentioning

confidence: 99%

Elevated cerebral spinal fluid biomarkers in children with mucopolysaccharidosis I-H

Raymond

Pasquali

Polgreen

et al. 2016

Sci Rep

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Mucopolysaccharidosis (MPS) type-IH is a lysosomal storage disease that results from mutations in the IDUA gene causing the accumulation of glycosaminoglycans (GAGs). Historically, children with the severe phenotype, MPS-IH (Hurler syndrome) develop progressive neurodegeneration with death in the first decade due to cardio-pulmonary complications. New data suggest that inflammation may play a role in MPS pathophysiology. To date there is almost no information on the pathophysiologic changes within the cerebral spinal fluid (CSF) of these patients. We evaluated the CSF of 25 consecutive patients with MPS-IH. While CSF glucose and total protein were within the normal range, we found a significantly mean elevated CSF opening pressure at 24 cm H2O (range 14–37 cm H2O). We observed a 3-fold elevation in CSF heparan sulfate and a 3–8 fold increase in MPS-IH specific non-reducing ends, I0S0 and I0S6. Cytokine analyses in CSF of children with MPS-IH showed significantly elevated inflammatory markers including: MCP-1 SDF-1a, IL-Ra, MIP-1b, IL-8, and VEGF in comparison to unaffected children. This is the largest report of CSF characteristics in children with MPS-IH. Identification of key biomarkers may provide further insight into the inflammatory-mediated mechanisms related to MPS diseases and perhaps lead to improved targeted therapies.

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Reference Range of Cerebrospinal Fluid Opening Pressure in Children: Historical Overview and Current Data

Cited by 48 publications

References 19 publications

Idiopathic intracranial hypertension in pediatric and adolescent patients

Idiopathic intracranial hypertension in pediatric and adolescent patients

Distinct progression patterns of brain disease in infantile and juvenile gangliosidoses: Volumetric quantitative MRI study

Elevated cerebral spinal fluid biomarkers in children with mucopolysaccharidosis I-H

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