Dr. Jacob Genizi Dr Eli Shahar Child Neurology Unit Meyer Children Hospital, Meyer Children Hospital, Rambam Medical Center, Haifa, Israel. Haifa, Israel. Meta-Analysis of Pseudotumor Cerebri in Prepubertal Children Vs adolescents: Differences in Sex Distribution and Obesity Rate

Modified Dandy’s Criteria for the diagnosis of Idiopathic Intracranial Hypertension Signs and symptoms of increased intracranial hypertension Signs and symptoms of increased intracranial hypertension No localizing neurological sign other than papilledema or abducens nerve palsy. No localizing neurological sign other than papilledema or abducens nerve palsy. Normal neuroimaging studies other than empty sella. Normal neuroimaging studies other than empty sella. Documented increased intracranial pressure (200 mm of water and higher) with normal fluid contens. Documented increased intracranial pressure (200 mm of water and higher) with normal fluid contens. Exclusion of structural or systemic cause. Exclusion of structural or systemic cause. Smith JL. Whence pseudotumor cerebri? Clin Neuroophthalmol Mar;5(1):55-6.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension

Etiology (PTC) Metabolic and endocrine disorders: Hypoparathyroidism. Hypoparathyroidism. Hypothyroidism. Hypothyroidism. vitamin D deficiency. vitamin D deficiency. Addison, Congenital adrenal hyperplasia. Addison, Congenital adrenal hyperplasia. Menarche. Menarche. Treatment or cessation of corticosteroids. Treatment or cessation of corticosteroids.

Etiology (PTC) Otitis media. Otitis media. Mastoiditis. Mastoiditis. Sinus vein thrombosis. Sinus vein thrombosis. Hematologic disturbances: Iron deficiency Anemia. Iron deficiency Anemia. Hemolysis. Hemolysis. Polycytemia. Polycytemia.

Etiology (PTC) Medication l Nalidixic acid l Ciprofloxacin l Tetracycline, minocycline l Vitamin A. l GH therapy. l Thyroid replacement therapy.

Etiology (IIH) Miscellaneous or Risk Factors ??? Women of reproductive age. Women of reproductive age. Obesity. Obesity.

ADULTS VS CHILDREN

הבדלים עיקריים בין ילדים לבין מבוגרים מבוגרים ילדים ותינוקות מבוגרים ילדים ותינוקות מגדר נשים בד ” כ בנים = בנות השמנת יתר " גורם סיכון " חשוב לא מהווה גורם סיכון פי משמעותי פי משמעותי ביטוי קליני כאבי ראש אי - שקט, אפטיה, ישנוניות חולשה של C.N. 6, חולשה של C.N. 6, פזילה, חולשה של C.N. 7, פזילה, חולשה של C.N. 7, כאבי גב וצואר, כאבי ראש. כאבי גב וצואר, כאבי ראש. Papilledema כן כשמרפסים או סוטורות קרניאליות פתוחים, בד ” כ קרניאליות פתוחים, בד ” כ אין פפילאדמה. אין פפילאדמה.

Obesity%Female% M / F Number patients Age (years) / META – ANALYSIS PTC – Children (Scott 1997)

Prepubertal children VS Adolescence

Our Data of PTC: ObesityN(%)Female% M / F Number patients Age (years) 2(10%)35%13/ (40%)80%3/ (23%)54%16/1935TOTAL

LITERATURE REVIEW OF PTC IN CHILDREN

ObesityN(%)Female% M / F Number patients Age (years) Author NR27%8/ Shofer 1997 NR33%4/ (10%)40%6/ Grace (23.5%)53%8/ Paul (50%)61%7/ (43%)50%7/ Balcer (85%)92%2/ (20%)29%12/ Sotiris %8/ (46%)38%8/ Kesler (72%)64%5/

META – ANALYSIS: ObesityN(%)Female% M / F Number patients Age (years) 19(26%)47%79/ (54%)74%36/ (41%)53%115/170285Total

Pathophysiology Adults - Sex hormones and lipids' regulation in the female may play a role in the induction of PTC. Adults - Sex hormones and lipids' regulation in the female may play a role in the induction of PTC. Children - Disordered CSF dynamics. Children - Disordered CSF dynamics.

Symptoms at presentation Adults Headache (90%) Headache (90%) Nausea & vomiting (56%) Nausea & vomiting (56%) Double vision (38%) Double vision (38%) Visual loss/ blurred vision (25%) Visual loss/ blurred vision (25%) Sore / stiff neck (9%) Sore / stiff neck (9%) Change in personality (9%) Change in personality (9%) Lethargy / anorexia (6%) Lethargy / anorexia (6%) Dizziness (6%) Dizziness (6%) Painful eyes Painful eyes

Symptoms at presentation- Children Incidental – Asymptomatic. Incidental – Asymptomatic. Ataxia Ataxia Photophobia Photophobia Myalgia Myalgia Tinnitus Tinnitus Limb numbness Limb numbness

Symptoms at presentation- Infants Irritability Irritability Apathy Apathy Somnolence Somnolence Increasing head size Increasing head size

Treatment Acetazolamide (Diamox) Acetazolamide (Diamox) Cortico-steroids Cortico-steroids Lumboperitoneal (LP) shunting Lumboperitoneal (LP) shunting Optic nerve sheath fenestration Optic nerve sheath fenestration

Conclusions: Pseudotumor cerebri among prepubertal children bares different characteristics compared with adolescents: Pseudotumor cerebri among prepubertal children bares different characteristics compared with adolescents: Boys affected more frequently than girls. Boys affected more frequently than girls. Concurrent obesity is significantly less common in pre-pubertal children. Concurrent obesity is significantly less common in pre-pubertal children.