Headache Characteristics in Children With Pseudotumor Cerebri Syndrome, Elevated Opening Pressure Without Papilledema, and Normal Opening Pressure: A Retrospective Cohort Study

Hamedani, Ali G.; Witonsky, Kailyn F.R.; Cosico, Mahgenn; Rennie, Robert; Xiao, Riu; Sheldon, Claire A.; Paley, Grace; McCormack, Shana E.; Liu, Geraldine W.; Friedman, Deborah I.; Liu, Grant T.; Szperka, Christina L.

doi:10.1111/head.13362

Cited by 18 publications

(17 citation statements)

References 35 publications

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“…Headache is the most common presenting symptom of PTCS, found in up to 98% of children with primary and secondary PTCS, 1,51,52 presenting more often in older children. It is found in nearly all PTCS without papilledema, given that headache is the primary symptom in this rare diagnosis.…”

Section: Headache Versus Migraine Symptomsmentioning

confidence: 99%

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Update in Pediatric Pseudotumor Cerebri Syndrome

Beres

2020

Semin Neurol

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Pseudotumor cerebri syndrome (PTCS) is a rare condition in children presenting with headache and papilledema from increased intracranial pressure that can cause significant morbidity. This can be idiopathic, also known as idiopathic intracranial hypertension or primary intracranial hypertension, or can be secondary to medications and associated medical conditions. Given the threat to vision, early detection and treatment is needed in all age groups. However, identifying papilledema or pseudopapilledema in children presents unique challenges sometimes as a result of differences between prepubertal and postpubertal children, further elucidating the complex pathophysiology. Management requires brain imaging, lumbar puncture, and frequent eye exams with medical and rarely surgical treatment. Visual outcomes in children are favorable if caught early and management can be prolonged over years. Pediatric PTCS is different from adult PTCS in many ways, and this review will focus on the most updated definitions of the disease, theories of pathophysiology, management, and treatment in the pediatric population.

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Section: Headache Versus Migraine Symptomsmentioning

confidence: 99%

“…53 Headache features in children with PTCS had not been studied until recently. Hamedani et al 52 found that episodic and chronic headaches in children were equally present, and the majority of the headaches were rated as severe. The pain was more often localized to the head and neck or shoulders.…”

Section: Headache Versus Migraine Symptomsmentioning

confidence: 99%

Update in Pediatric Pseudotumor Cerebri Syndrome

Beres

2020

Semin Neurol

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“…In contrast, the characteristics of headache in pediatric PTCS are more widely variable. Headache in PTCS may be daily or constant but also may be entirely episodic, and the pain may be diffuse or focal [23,24]. Headache in pediatric PTCS is more likely to involve the neck and shoulders, perhaps related to sensitivity towards distention of the spinal root dural sheaths with increased pressure [23].…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Headache in PTCS may be daily or constant but also may be entirely episodic, and the pain may be diffuse or focal [23,24]. Headache in pediatric PTCS is more likely to involve the neck and shoulders, perhaps related to sensitivity towards distention of the spinal root dural sheaths with increased pressure [23]. Importantly, the "classic" high-pressure headache triad of (i) daily headache; (ii) worsening with Valsalva; and (iii) diffuse nonpulsating pain was found to be present in only 36.6 % of children with PTCS [23,26].…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Headache in pediatric PTCS is more likely to involve the neck and shoulders, perhaps related to sensitivity towards distention of the spinal root dural sheaths with increased pressure [23]. Importantly, the "classic" high-pressure headache triad of (i) daily headache; (ii) worsening with Valsalva; and (iii) diffuse nonpulsating pain was found to be present in only 36.6 % of children with PTCS [23,26]. Consequently, at the risk of missing the diagnosis, the presence or absence of these "classic" headache features cannot be used as a reliable screen for PTCS in children.…”

Section: Clinical Presentationmentioning

confidence: 99%

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Pseudotumor Cerebri Syndrome in Children

Barmherzig

Szperka

2019

Curr Pain Headache Rep

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Pupose of Review: Pseudotumor cerebri syndrome (PTCS) may affect both children and adults, however the risk factors and clinical presentation vary greatly between these populations. This review aims to highlight the entity of PTCS in children and the unique considerations in this population; review the epidemiology and demographics; discuss the clinical presentation, revised diagnostic criteria, and approach to evaluation; review management strategies; and discuss the prognosis and long-term outcomes in children with PTCS. Recent Findings: Clinical presentation can be variable in children and may be less obvious than in their adult counterparts. Papilledema can also be challenging to diagnose in this population. The upper limits for opening pressure on lumbar puncture differ in children, with a cutoff of 25 cm H20 (or 28 cm H2O in a sedated or obese child). Summary: Morbidity related to visual loss, pain, and reduced quality of life lends urgency towards accurately identifying, evaluating, and managing children with PTCS. There are no randomised controlled studies to allow for evidence-based recommendations for the management of PTCS in children. Further studies are needed to clarify and consolidate management approaches in this population.

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Ultrasound-guided initial diagnosis and follow-up of pediatric idiopathic intracranial hypertension

Kerscher,

Zipfel,

Haas-Lude

et al. 2024

Pediatr Radiol

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Background Idiopathic intracranial hypertension in children often presents with non-specific symptoms found in conditions such as hydrocephalus. For definite diagnosis, invasive intracranial pressure measurement is usually required. Ultrasound (US) of the optic nerve sheath diameter provides a non-invasive method to assess intracranial pressure. Transtemporal US allows imaging of the third ventricle and thus assessment for hydrocephalus. Objective To investigate whether the combination of US optic nerve sheath and third ventricle diameter can be used as a screening tool in pediatric idiopathic intracranial hypertension to indicate elevated intracranial pressure and exclude hydrocephalus as an underlying pathology. Further, to analyze whether both parameters can be used to monitor treatment outcome. Materials and methods We prospectively included 36 children with idiopathic intracranial hypertension and 32 controls. Using a 12-Mhz linear transducer and a 1–4-Mhz phased-array transducer, respectively, optic nerve sheath and third ventricle diameters were determined initially and during the course of treatment. Results In patients, the mean optic nerve sheath diameter was significantly larger (6.45±0.65 mm, controls: 4.96±0.32 mm) and the mean third ventricle diameter (1.69±0.65 mm, controls: 2.99±1.31 mm) was significantly smaller compared to the control group, P<0.001. Optimal cut-off values were 5.55 mm for the optic nerve sheath and 1.83 mm for the third ventricle diameter. Conclusions The combined use of US optic nerve sheath and third ventricle diameter is an ideal non-invasive screening tool in pediatric idiopathic intracranial hypertension to indicate elevated intracranial pressure while ruling out hydrocephalus. Treatment can effectively be monitored by repeated US, which also reliably indicates relapse.

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Headache Characteristics in Children With Pseudotumor Cerebri Syndrome, Elevated Opening Pressure Without Papilledema, and Normal Opening Pressure: A Retrospective Cohort Study

Abstract: While missing data and small cohort size are limitations, this study suggests that headache in PTCS is more likely to involve the head along with neck/shoulders, and that headache in PTCS may be episodic or constant. Headache is occasionally absent in PTCS.

Cited by 18 publications

References 35 publications

Update in Pediatric Pseudotumor Cerebri Syndrome

Update in Pediatric Pseudotumor Cerebri Syndrome

Pseudotumor Cerebri Syndrome in Children

Ultrasound-guided initial diagnosis and follow-up of pediatric idiopathic intracranial hypertension

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