Evaluation of the signs and symptoms of pseudotumor cerebri syndrome in pediatric population

One of the prominent causes of pseudotumor cerebri (PTC) syndrome is increased impedance of the venous outflow tracts of the brain. Theoretically, this is a justified mechanism for raised intracranial pressure; yet, there had been few cases of such a scenario reported in the literature, and to the authors' knowledge no case of PTC due to benign osteopetrosis has been reported to date. The present case occurred in a 19-year-old woman with a constellation of signs and symptoms compatible with PTC syndrome, whose radiological and laboratory studies confirmed the diagnosis of osteopetrosis. Magnetic resonance venography demonstrated bilateral jugular foraminal stenosis regarding the underlying disease process. The patient did well after she underwent a lumboperitoneal shunt insertion procedure.

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Therapeutic approaches to pediatric pseudotumor cerebri: New insights from literature data

International Journal of Immunopathology and Pharmacology ◽

10.1177/0394632016681578 ◽

2017 ◽

Vol 30 (1) ◽

pp. 94-97 ◽

Cited By ~ 1

Author(s):

Giovanna Vitaliti ◽

Piero Pavone ◽

Nassim Matin ◽

Omidreza Tabatabaie ◽

Salvatore Cocuzza ◽

...

Keyword(s):

Treatment Guidelines ◽

Controlled Trial ◽

Treatment Strategies ◽

Signs And Symptoms ◽

Pseudotumor Cerebri ◽

Therapeutic Approaches ◽

Childbearing Age ◽

Overweight Women ◽

Cranial Nerve Palsies ◽

Pseudotumor Cerebri Syndrome

The pseudotumor cerebri syndrome (PTCS), also known as idiopathic intracranial hypertension (IIH), is characterized by signs and symptoms of increased intracranial pressure such as headache and cranial nerve palsies, in the absence of any space-occupying mass. This condition commonly affects overweight women of childbearing age, even if it is also frequent in men and children. Children with PTCS may present with atypical signs and symptoms, with a different prognosis compared to adults. However, the treatment is the same for both children and adults, even if there are no strict treatment guidelines in regards. All treatment strategies in children are based on retrospective studies and none has been evaluated in prospective or randomized controlled trial studies. This review focuses on literature data on PTCS treatment in children.

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Prevalence of Signs and Symptoms of Pseudotumor Cerebri Syndrome Before and After Transsphenoidal Surgery for Cushing’s Disease – A Prospective Consecutive Case Series

Experimental and Clinical Endocrinology & Diabetes ◽

10.1055/a-1200-1528 ◽

2020 ◽

Author(s):

Torge Huckhagel ◽

Jörg Flitsch ◽

Roman Rotermund ◽

Volker Knospe

Keyword(s):

Visual Acuity ◽

Cushing’S Disease ◽

Transsphenoidal Surgery ◽

Signs And Symptoms ◽

Pseudotumor Cerebri ◽

Cushing's Disease ◽

Pseudotumor Cerebri Syndrome ◽

Before And After ◽

Biochemical Remission

Abstract Introduction Pseudotumor cerebri syndrome (PTCS) has anecdotally been described after successful treatment of Cushing’s disease (CD), but the prevalence following transsphenoidal surgery has not been determined so far in a prospective study. Patients and Methods 41 consecutive adult CD patients were prospectively screened for signs and symptoms of PTCS, headache-related impairment, and ophthalmological features associated with intracranial pressure elevation before surgery and at follow-up (mean 4 months). Results Biochemical remission was achieved in 36 of 41 (87.8%) patients after surgery. The most frequent preoperative complaints were visual acuity impairment (19 cases; 46.3%), headache (13 cases; 31.7%), and ear noise (9 cases; 22.0%). A significant reduction of visual disturbances was seen at follow-up. Overall, CD patients presented with fairly low headache-related emotional and functional restrictions before and after surgery. One of 34 (2.9%) patients with sufficient ophthalmological follow-up showed new-onset papilledema combined with temporary worsening of visual acuity and scotoma. Conclusion Our results suggest a very low frequency of signs and symptoms of PTCS after surgical treatment for CD in adults. This estimate is in line with previous outcomes from retrospective pediatric CD series. Further large-scale studies are needed to determine the actual prevalence of this condition following biochemical remission of CD.

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Update in Pediatric Pseudotumor Cerebri Syndrome

Seminars in Neurology ◽

10.1055/s-0040-1708847 ◽

2020 ◽

Vol 40 (03) ◽

pp. 286-293

Author(s):

Shannon J. Beres

Keyword(s):

Surgical Treatment ◽

Intracranial Hypertension ◽

Pediatric Population ◽

Age Groups ◽

Rare Condition ◽

Pseudotumor Cerebri ◽

Significant Morbidity ◽

Increased Intracranial Pressure ◽

Pseudotumor Cerebri Syndrome ◽

Eye Exams

AbstractPseudotumor 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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Pseudotumor cerebri syndrome in a patient with narcolepsy type 1

European Journal of Paediatric Neurology ◽

10.1016/j.ejpn.2017.11.002 ◽

2018 ◽

Vol 22 (1) ◽

pp. 194-198 ◽

Cited By ~ 1

Author(s):

Thomas Rossor ◽

Ming Lim ◽

Kirandeep VanDenEshof ◽

Paul Gringras

Keyword(s):

Pseudotumor Cerebri ◽

Pseudotumor Cerebri Syndrome

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Pseudotumor cerebri syndrome: venous sinus obstruction and its treatment with stent placement

Journal of Neurosurgery ◽

10.3171/jns.2003.98.5.1045 ◽

2003 ◽

Vol 98 (5) ◽

pp. 1045-1055 ◽

Cited By ~ 146

Author(s):

Brian K. Owler ◽

Geoffrey Parker ◽

G. Michael Halmagyi ◽

Victoria G. Dunne ◽

Verity Grinnell ◽

...

Keyword(s):

Stent Placement ◽

Pseudotumor Cerebri ◽

Venous Sinus ◽

Stent Insertion ◽

Benign Intracranial Hypertension ◽

Content Type ◽

Venous Sinuses ◽

Pseudotumor Cerebri Syndrome ◽

New Treatment ◽

Fine Print

Object. Pseudotumor cerebri, or benign intracranial hypertension, is a condition of raised intracranial pressure in the absence of a mass lesion or cerebral edema. It is characterized by headache and visual deterioration that may culminate in blindness. Pseudotumor cerebri is caused by venous sinus obstruction in an unknown percentage of cases. The purpose of this study was to investigate the role of cerebral venous sinus disease in pseudotumor cerebri and the potential of endoluminal venous sinus stent placement as a new treatment. Methods. Nine consecutive patients in whom diagnoses of pseudotumor cerebri had been made underwent examination with direct retrograde cerebral venography (DRCV) and manometry to characterize the morphological features and venous pressures in their cerebral venous sinuses. The cerebrospinal fluid (CSF) pressure was measured simultaneously in two patients. If patients had an amenable lesion they were treated using an endoluminal venous sinus stent. Five patients demonstrated morphological obstruction of the venous transverse sinuses (TSs). All lesions were associated with a distinct pressure gradient and raised proximal venous sinus pressures. Four patients underwent stent insertion in the venous sinuses and reported that their headaches improved immediately after the procedure and remained so at 6 months. Vision was improved in three patients, whereas it remained poor in one despite normalized CSF pressures. Conclusions. Patients with pseudotumor cerebri should be evaluated with DRCV and manometry because venous TS obstruction is probably more common than is currently appreciated. In patients with a lesion of the venous sinuses, treatment with an endoluminal venous sinus stent is a viable alternative for amenable lesions.

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