Objective:Pseudotumor Cerbri (PTC) is a clinical syndrome characterized by increased Intra-Cranial Pressure (ICP) without any evidence of a mass lesion or any obstructive process. The incidence is 0.9-2 per 100.000 people. It is more frequent in adults in the 20-30 years age group, especially obese women, and less common in pediatric age groups; 11-16 years old children may however suffer from PTC, with no difference in the rates of occurrence in either sex.Material & Methods:In this descriptive retrospective study we reviewed the files of 32 children with diagnosis of PTC admitted during the past 15 years in the neurology ward of the Mofid Children's Hospital (25) and Ali Asghar Children Hospitals (7) between the years 1988 and 2003.Results:The results of this study revealed that children in the 5-10 years age group, girls in particular, are more vulnerable.Conclusion:The most frequent complaints that brought these patients to physician included headache, vomiting and strabismus. Common findings of neurological examination were papilledema, abducent nerve palsy and ataxic gait. While all cases recovered with medical treatment, one did need surgical intervention. Abbreviations; Pseudotumor Cerbri = PTC; Intra Cranial pressure = ICP.Keywords :PTC , papilledema , ICP

Ghofrani MD, M.

Hasanpour avanji MD, SH.

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37Iran J Child Neurology  Oct. 200632 CASES OF PSEUDOTUMOR CEREBRI IN TWOPEDIATRIC HOSPITALS OF TEHRAN, IRANSH. Hasanpour avanji MD1,M.Ghofrani MD 2Abstract:Objective:Pseudotumor Cerbri (PTC) is a clinical syndrome characterized byincreased Intra-Cranial Pressure (ICP) without any evidence of a masslesion or any obstructive process. The incidence is 0.9-2 per 100.000people. It is more frequent in adults in the 20-30 years age group,especially obese women, and less common in pediatric age groups;11-16 years old children may however suffer from PTC, with nodifference in the rates of occurrence in either sex.Material & Methods:In this descriptive retrospective study we reviewed the files of 32children with diagnosis of PTC admitted during the past 15 years inthe neurology ward of the Mofid Children's Hospital (25) and AliAsghar Children Hospitals (7) between the years 1988 and 2003.Results:The results of this study revealed that children in the 5-10 years agegroup, girls in particular, are more vulnerable.Conclusion: The most frequent complaints that brought these patients to physicianincluded headache, vomiting and strabismus. Common findings ofneurological examination were papilledema, abducent nerve palsyand ataxic gait. While all cases recovered with medical treatment,one did need surgical intervention.Abbreviations; Pseudotumor Cerbri = PTC; Intra Cranial pressure =ICP.Keywords : PTC , papilledema , ICP.1. Assistant Professor  ,IranUniversity of Medical Sciences, AliAsghar Children's Hospital,2. Professor,Head of PediatricNeurology Department, ShaheedBeheshti University of MedicalSciences, Mofid Chilren's Hospital.Corresponding Author:SH. Hasanpour avanji MDTel: +98 21 22226511Fax: +98 21 22220063E-mail:s_m_hp2000@ yahoo.comIntroductionPseudotumor Cerbri (PTC), a clinical syndrome characterized by increased IntraCranial Pressure (ICP) without any evidence of mass lesion or obstructive process(1), was reported by Quinke in 1893  (2).The incidence is 0.9-2 per 100.000 people, being more frequent in adults of the20-30 years age group(especially obese women), and less common in pediatricage groups. However children 11-16 years of age may suffer from PTC, withsimilar rates of occurrence in either sex (4,5). Considering the lack of literatureon PTC in children in our country, this study was conducted with the aim ofobtaining better understanding of diagnosis, clinical condition and treatment ofPTC in our children .RESEARCH ARTICLE38 Iran J Child Neurology  Oct. 2006Material & MethodsIn this descriptive retrospective study we reviewed thefiles of 32 patients diagnosed with PTC, hospitalized inthe neurology department of the Mofid children's hospital(no=25) and the Ali Asghar Children's Hospital (no=7),between the years 1988 and 2003.ResultsThe results of this study revealed that children of the 5-10 year age group (43.5%), girls in particular (56%) aremore vulnerable (Table I  ).There were more females than males in this study; weight of a third of the patients was under the 5 percentile (33%)(table II).32 CASES OF PSEUDOTUMOR CEREBRI IN TWO PEDIATRIC HOSPITALS OF TEHRAN, IRAN39Iran J Child Neurology  Oct. 2006The most frequent complaints that brought these patients to physician, consisted of headache (85%), strabismus (67%)and vomiting (56%) (Table III).The most common findings at neurological examination were papilledema (100%), abducent nerve palsy(bilateral - 47%, unilateral - 18%) and ataxia 28% (Table IV).32 CASES OF PSEUDOTUMOR CEREBRI IN TWO PEDIATRIC HOSPITALS OF TEHRAN, IRANIran J Child Neurology  Oct. 200640Etiological factors found to be responsible were infectious diseases (66%); upper respiratory infections(URI), otitismedia and urinary tract infections, followed by drugs medications and lipid soluble vitamins. All except one of thePTC cases recovered with medical treatment; this one case required surgical treatment (Table V).One of the patients, who had recurrence one year aftermedication was discontinued, suffered from acutelymphocytic leukemia.DiscussionLiterature reviews show PTC to be a disease with a widespectrum; at one end of which is an infant with a transientincrease of ICP, alleviated by lumbar puncture and at theother more grave conditions with very high ICP and visualcomplications, which may require surgical procedures(6).The ratio of male to female frequency was 1.3:1, beinghigher in males, a finding which differs from that of otherarticles, in which the ratio is almost equal. Whereas theweight of most patients in our study was under the 5percentile, results of other studies show PTC to be morefrequent in adolescents, especially females (4).The etiology of  PTC, reported in different researches,are infectious diseases, drugs, metabolic and endocrinediseases (7, 8, 9). Other causes of PTC are mild headtrauma, corticosteroid withdrawal, Hypo- and hyperthyroidism, parathyroid disease, addison, Systemic LupusErythmatous, carbon dioxide retention secondary tochronic lung diseases (10, 11). In children under the ageof 20 years, thrombosis of one or more of dural sinuses,particularly the lateral, as a complication of otitis mediaor mastoiditis is probably still one of the most commonabnormalites (12, 13).This syndrome may also occur secondary tohypervitaminosis A, anemia and in contraceptive pileusers (14, 15). In our study the most common etiologicfindings were URI, otitis media, and then hypervitaminosisA&D and antibiotic usage, e.g. nalidixic acid,nitrofurantoin.Patients with PTC develop non specific signs of increasedICP, headache-50-75%, nausea and vomiting 35-38%,blurred vision and diplopia 27-35%, cerebral nerve palsy,(especially the 6th )24-47%, and papilledema in themajority of cases (16, 17, 18) .Cerebro-Spinal Fluid results are generally normal, althoughmild pleocytosis is not uncommon (19). The signs andsymptoms of PTC in this study were very similar to thoseof others (Table III, IV). All patients of this study hadnormal cerebrospinal fluid , but 3 of them shownpleocytosis.32 CASES OF PSEUDOTUMOR CEREBRI IN TWO PEDIATRIC HOSPITALS OF TEHRAN, IRAN41Iran J Child Neurology  Oct. 2006Generally imaging studies reveal ventricular system thatis midline and normal or mildly reduced in size (4, 20).In this research brain CT scans of most patients werenormal although 5 of them shown ventriculomegaly andthe ventricular size had reduced in 3.The treatment of PTC varies according to severity ofdiseases and degree of visual impairment. It is directedtowards the monitoring of ICP to within near normallimits to reduce the danger of permanent visual damage(20-21). The first therapeutic step is to treat the underlyingprecipitant factors, like vitamin A or tetracyclinediscontinuation if possible (10-22-23).41% of patients, in this study used drugs such as vitaminA, Nalidixic Acid and corticosteroids; these werediscontinued after diagnosis. All cases of this studyrecovered with medical treatment except for one whorequired surgical intervention. One patient had recurrenceone year after discontinuation of treatment.ConclusionMore attention needs to be paid to clinical training ofphysicians for diagnosis of PTC in patients with headacheand other signs and symptoms of PTC and those withhistories of drug and lipid soluble vitamins usage, anddiseases that are precipitant factors of pseudotumorcerebri.References1. Johan F. Schoolman. Childhood pseudotoumor cerebri: Clinical   and interacranial pressure Response to Acetazolamid, and    Furosemide treatment in a case series. Child Neurology   1994    ; 9(2): 160-164.2. Felman MH,schezinger NS.Benign intracranial  hypertension   ssociated with hypervitaminosis A.Arch Neurol 1970;22:1-7.3. Mccomb  JG. Recent research into the nature of cerebrospinal   fluid formation and absorption . J Neurosurg 1988;59: 364-    386.4. Cobert  J, Thompson   HS. The rational management of     idiopathic interacranial hypertension. Arch Neurol   1989;46:    1049-1051.5.  Paul Babikian   MD, James Corbett   MD, William Bell  MD.    Idiopathic intreracranial hypertension in children: the lowaa    Experience. Journal of child Neurology 1994 Apr ;9(2):144-149.6. Susana P  Campos  M D, Scott olitsky  MD. Idiopathic    intracranial Hypertension after thyroxin therapy for Acquired    primary Hypothyroidism .Clinical pediatrics  1995;334-337.7.    Franklin G, Moser sadek Hilal .MR imaging of pseudotumor      cerebri. AJR   1998 Apr;150:50-51.8.    Johnston  I , Paterson  A. Benign interacranial hypertension,   Diagnosis and prognosis. Brain 1974; 97: 289-300.9.  John  H,  Menkes  MD. Textbook of child Neurology. 6th     ed.2000. P.640-642.10.   Walker  AE , Adamkiewicz  JJ. Pseudotumor crebri associated        with prolonged corticosteroid therapy : Report of four cases.       JAMA  1964;188: 779-784.11.  Toga -O, Jacobson -N, Berexin -S, et al. Recurrent benign      interacranial hypertension due to iron deficiency anemia .      Am-j-Pediatr   1994 Aug;16(3): 246-70.12. Gardner -k  , Coke -t, Digger-KB. Idiopathic interacranial        Hypertension associated with tetracycline use inferatentorial        twins : case report Andre view. Neurology  1998 Jan ;45(1):       6-10.13. Green  M, Sholomorinker , Gambacorta  D. Pseudotumor      cerebri in systemic lupus Erythematous. Seminar in Arthritis     and Rheumatism 1995 Oct;25: (2):103-108.14. Rush  JA. Pseudotumor cerebri. Clinical profile and visual     out come in 63 patients. Mayo clin proc  1980;55: 541-546.15. Gills  JP, Kpp  GP, Odom  GL:Benign intera cranial       hypertension, psedotumor cerebi from obstruction of dural   sinuses. Arch ophthalmologic  1967;78: 592-595.16. Green  M. Benign intera cranial hypertension. Neurology      1964 ;14: 569-573.17.  Guidetti B, Giuffer R , Gambacorta D. Follow-up study of       100 cases of Pseudotumor cerebri. Acta neuro chil  1968;18:      259-267.18. Greer M. Benign intera crania1 hypertension .Neurology      1962;12:472-476.19. Grant liu Jool ,  S  Glaser , Norman  J, Schatz. High dose      Methyl prednisolone and  acethazolamide for visual loss in      Pseudotumor cerebri. Am-J-ophthalmal 1994 Jul ;15: 88-96.20. Rosenberg   ML , Buinic  JR. Cerebrospinal fluid division      procedures in Pseudotumor cerebri. Neurology 1999;43:       1971-1072.21. Grant DN .Benign intera cranial hypertension : A review of      701 cases in infancy and childhood. Arch dis child 1971;46:      651-655.22. Corbett  JJ  , Thompson  HS. Visual loss in pseudotumor      cerebri fallow -up of 57 patients from 5 to 41 years and a       profile of 14 patients with permanent severe visual loss. Arch      neural  1982;39: 461- 474.23. M law, S Yong,H Wang ,et al :Pseudotumot cerebrei. Am-J-      Neuroradio 2002;23;345-346.32 CASES OF PSEUDOTUMOR CEREBRI IN TWO PEDIATRIC HOSPITALS OF TEHRAN, IRAN

32 CASES OF PSEUDOTUMOR CEREBRI IN TWO PEDIATRIC HOSPITALS OF TEHRAN, IRAN

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32 CASES OF PSEUDOTUMOR CEREBRI IN TWO PEDIATRIC HOSPITALS OF TEHRAN, IRAN

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