MetaAnalysis of Pseudotumor Cerebri in Prepubertal Children Vs

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

Modified Dandy’s Criteria for the diagnosis of Idiopathic Intracranial Hypertension n n Signs and symptoms of increased intracranial hypertension No localizing neurological sign other than papilledema or abducens nerve palsy. Normal neuroimaging studies other than empty sella. Documented increased intracranial pressure (200 mm of water and higher) with normal fluid contens. Exclusion of structural or systemic cause. Smith JL. Whence pseudotumor cerebri? Clin Neuroophthalmol. 1985 Mar; 5(1): 55 -6.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension

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

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

ADULTS VS CHILDREN

META – ANALYSIS PTC – Children (Scott 1997) Age (years) 3. 5 -18 Number patients M / F 374 175/199 Female % 53. 2 Obesity % 29. 6

Prepubertal children VS Adolescence

Our Data of PTC: 1995 -2005 Age (years) Number patients M / F Female % Obesity N(%) 3 -11 20 13/7 35% 2(10%) 12 -17 15 3/12 80% 6(40%) TOTAL 35 16/19 54% 8(23%)

LITERATURE REVIEW OF PTC IN CHILDREN

Author Age (years) Number patients Shofer 1997 2 -11 12 -16 11 6 Grace 1998 Paul 1998 2 -11 0 -11 12 -17 3. 5 -8 9 -14 2 -11 12 -16 Balcer 1999 Sotiris 2000 Kesler 2002 M / F Female % Obesity N(%) 8/3 4/2 27% 33% NR 10 6/4 40% 1(10%) 17 18 14 26 17 19 13 14 8/9 7/11 7/7 2/24 12/5 8/11 8/5 5/9 53% 61% 50% 92% 29% 58% 38% 64% 4(23. 5%) 9(50%) 6(43%) 22(85%) 7(20%) NR 6(46%) 10(72%)

META – ANALYSIS: Age (years) Number patients M / F Female % Obesity N(%) 0 -11 148 79/69 47% 19(26%) 12 -18 137 36/101 74% 47(54%) Total 285 115/170 53% 66(41%)

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

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

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

Symptoms at presentation. Infants n n Irritability Apathy Somnolence Increasing head size

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

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