Dr. Jacob Genizi Dr. Jacob Genizi Dr Eli ShaharDr Eli Shahar

Child Neurology UnitChild Neurology Unit Meyer Children Hospital,Meyer Children Hospital,Rambam Medical Center, Rambam Medical Center,

Haifa, Israel.Haifa, Israel.

Meta-Analysis of Pseudotumor Cerebri Meta-Analysis of Pseudotumor Cerebri in Prepubertal Children Vs adolescents: in Prepubertal Children Vs adolescents:

Differences in Sex DistributionDifferences in Sex Distribution and Obesity Rateand Obesity Rate

Modified Dandy’s Criteria for the diagnosis of Modified Dandy’s Criteria for the diagnosis of Idiopathic Intracranial HypertensionIdiopathic Intracranial Hypertension

Signs and symptoms of increased intracranial hypertensionSigns and symptoms of increased intracranial hypertension No localizing neurological sign other than papilledema or No localizing neurological sign other than papilledema or

abducens nerve palsy.abducens nerve palsy. Normal neuroimaging studies other than empty sella.Normal neuroimaging studies other than empty sella. Documented increased intracranial pressure (200 mm of Documented increased intracranial pressure (200 mm of

water and higher) with normal fluid contens.water and higher) with normal fluid contens. Exclusion of structural or systemic cause.Exclusion of structural or systemic cause.

Smith JLSmith JL.. Whence pseudotumor cerebri? Whence pseudotumor cerebri?

Clin Neuroophthalmol. 1985 Mar;5(1):55-6Clin Neuroophthalmol. 1985 Mar;5(1):55-6..

Pseudotumor CerebriPseudotumor Cerebri

Idiopathic Intracranial Idiopathic Intracranial HypertensionHypertension

Etiology (PTC)Etiology (PTC)

Metabolic and endocrine disordersMetabolic and endocrine disorders::• Hypoparathyroidism.Hypoparathyroidism.• Hypothyroidism.Hypothyroidism.• vitamin D deficiency. vitamin D deficiency. • Addison, Congenital adrenal Addison, Congenital adrenal

hyperplasia. hyperplasia. • Menarche. Menarche. • Treatment or cessation of Treatment or cessation of

corticosteroidscorticosteroids..

Etiology (PTC)Etiology (PTC)• Otitis media. Otitis media. • Mastoiditis.Mastoiditis.• Sinus vein thrombosisSinus vein thrombosis..

Hematologic disturbances:Hematologic disturbances:• Iron deficiency Anemia.Iron deficiency Anemia.• Hemolysis.Hemolysis.• Polycytemia. Polycytemia.

Etiology (PTC)Etiology (PTC)

MedicationMedication Nalidixic acidNalidixic acid CiprofloxacinCiprofloxacin Tetracycline, minocyclineTetracycline, minocycline Vitamin A.Vitamin A. GH therapy.GH therapy. Thyroid replacement Thyroid replacement

therapy.therapy.

Etiology (IIH)Etiology (IIH)

Miscellaneous or Miscellaneous or Risk FactorsRisk Factors ??? ???

• Women of reproductive age.Women of reproductive age.• Obesity. Obesity.

ADULTSADULTSVS VS

CHILDRENCHILDREN

הבדלים עיקריים בין ילדים הבדלים עיקריים בין ילדים לבין מבוגריםלבין מבוגרים

מבוגרים ילדים ותינוקות מבוגרים ילדים ותינוקות נשים בד”כ בנים = בנות נשים בד”כ בנים = בנותמגדר מגדר

לא מהווה גורם סיכון לא מהווה גורם סיכון "גורם סיכון" חשוב "גורם סיכון" חשוב השמנת יתרהשמנת יתר

משמעותי משמעותי10-2010-20 פי פי ביטוי קליני כאבי ראש אי-שקט, אפטיה, ישנוניותביטוי קליני כאבי ראש אי-שקט, אפטיה, ישנוניות

,,C.N. 6C.N. 6 חולשה של חולשה של ,,C.N. 7C.N. 7פזילה, חולשה של פזילה, חולשה של

כאבי גב וצואר, כאבי ראש. כאבי גב וצואר, כאבי ראש.PapilledemaPapilledema כן כשמרפסים או סוטורות כן כשמרפסים או סוטורות

קרניאליות פתוחים, בד”כ קרניאליות פתוחים, בד”כ אין פפילאדמה. אין פפילאדמה.

Age Age (years)(years)

Number Number patients patients

M / FM / F FemaleFemale

%%

ObesityObesity

%%

3.5-183.5-18 374374 175/199175/199 53.253.229.629.6

META – ANALYSISMETA – ANALYSIS

PTC – Children (Scott 1997)PTC – Children (Scott 1997)

Prepubertal childrenPrepubertal childrenVSVS

AdolescenceAdolescence

Our Data of PTC: 1995-2005Our Data of PTC: 1995-2005

Age Age (years)(years)

Number Number patients patients

M / FM / F FemaleFemale

%%

ObesityObesity

NN(%)(%)

3-113-11202013/713/735%35%22((10%10%))

12-1712-1715153/123/1280%80%66((40%40%))

TOTALTOTAL353516/1916/1954%54%88((23%23%))

LITERATURE REVIEW OFLITERATURE REVIEW OF

PTC IN CHILDRENPTC IN CHILDREN

AuthorAuthorAge Age (years)(years)

Number Number patients patients

M / FM / F FemaleFemale

%%

ObesityObesity

NN)%()%(

Shofer Shofer 19971997

2-112-1111118/38/327%27%NRNR

12-1612-16664/24/233%33%NRNR

Grace Grace 19981998

2-112-1110106/46/440%40%11((10%10% ) )

Paul Paul 19981998

0-110-1117178/98/953%53%44((23.5%23.5%))12-1712-1718187/117/1161%61%99((50%50%))

Balcer Balcer 19991999

3-113-1114147/77/750%50%66((43%43%))12-1712-1726262/242/2492%92%2222((85%85%))

Sotiris Sotiris 20002000

3.5-83.5-8171712/512/529%29%77((20%20%))9-149-1419198/118/1158%58%

Kesler Kesler 20022002

2-112-1113138/58/538%38%66((46%46%))12-1612-1614145/95/964%64%1010((72%72%))

META – ANALYSISMETA – ANALYSIS::

Age Age (years)(years)

Number Number patients patients

M / FM / F FemaleFemale

%%

ObesityObesity

NN(%)(%)

0-110-1114814879/6979/6947%47%1919((26%26%))

12-1812-1813713736/10136/10174%74%4747((54%54%))

TotalTotal 285285115/170115/17053%53%6666((41%41%))

PathophysiologyPathophysiology

Adults - Sex hormones and lipids' Adults - Sex hormones and lipids' regulation in the female may play a regulation in the female may play a role in the induction of PTC.role in the induction of PTC.

Children - Disordered CSF dynamics.Children - Disordered CSF dynamics.

Symptoms at presentationSymptoms at presentationAdultsAdults

• 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 eyesPainful eyes

Symptoms at presentation- Symptoms at presentation- ChildrenChildren

• Incidental – Asymptomatic. Incidental – Asymptomatic. • AtaxiaAtaxia• PhotophobiaPhotophobia• MyalgiaMyalgia• TinnitusTinnitus• Limb numbnessLimb numbness

Symptoms at presentation- Symptoms at presentation- InfantsInfants

IrritabilityIrritability ApathyApathy SomnolenceSomnolence Increasing head sizeIncreasing head size

TreatmentTreatment

Acetazolamide (Diamox)Acetazolamide (Diamox) Cortico-steroidsCortico-steroids Lumboperitoneal (LP) shuntingLumboperitoneal (LP) shunting Optic nerve sheath fenestrationOptic nerve sheath fenestration

Conclusions:Conclusions:

• Pseudotumor cerebri among prepubertal Pseudotumor cerebri among prepubertal children bares different characteristics children bares different characteristics compared with adolescents:compared with adolescents:

• Boys affected more frequently than girls.Boys affected more frequently than girls.• Concurrent obesity is significantly less Concurrent obesity is significantly less

common in pre-pubertal children.common in pre-pubertal children.