The benign spectrum of hypothalamic hamartomas: Infrequent epilepsy and normal cognition in patients presenting with central precocious puberty

Show simple item record

dc.contributor Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.author CUKIER, Priscilla FMUSP-HC
CASTRO, Luiz Henrique Martins FMUSP-HC
BANASKIWITZ, Natalie FMUSP-HC
TELES, Leandro Roberto
FERREIRA, Luiz Roberto Kobuti FMUSP-HC
ADDA, Carla Cristina FMUSP-HC
LEITE, Claudia da Costa FMUSP-HC
ARNHOLD, Ivo J. P. FMUSP-HC
MENDONCA, Berenice Bilharinho FMUSP-HC
LATRONICO, Ana Claudia FMUSP-HC
BRITO, Vinicius Nahime FMUSP-HC
dc.date.issued 2013
dc.identifier.citation SEIZURE-EUROPEAN JOURNAL OF EPILEPSY, v.22, n.1, p.28-32, 2013
dc.identifier.issn 1059-1311
dc.identifier.uri http://observatorio.fm.usp.br/handle/OPI/1899
dc.description.abstract Purpose: Hypothalamic hamartoma (HH) is the main structural cause of central precocious puberty (CPP). HH is frequently associated with cognitive impairment and epileptic encephalopathies. Disease severity in case series from neurology services may be biased towards more neurologically impaired patients. Aim: To perform a prospective cognitive evaluation in patients with HH presenting with CPP in an endocrinology outpatient clinic setting. Methods: We evaluated fifteen consecutive patients with CPP due to HH presenting to an endocrinology outpatient clinic. CPP was diagnosed at a median age of 0.7 yr (0.4-7 yr). Mean age at neurologic evaluation was 13.9 yrs. Eight patients (53.3%) were male. Epileptic seizures occurred in 5/15 (33%) patients. Two patients presented a single unprovoked seizure (SUS). Three patients were diagnosed with epilepsy. Cognitive evaluation, using age-appropriate Wechsler Intelligence Scale, was performed in 11 patients. Results: All patients without epilepsy, including two patients with a history of a SUS, had normal neurologic and cognitive evaluation. Epilepsy and SUS were only seen in patients with sessile HH. Three patients with epilepsy presented cognitive or behavioral findings. Reduced intelligence quotients (IQ), in the borderline range, were noted in both patients with epilepsy who underwent full cognitive evaluation. We found no significant correlation between HH diameter or shape and mean full-scale IQ. Conclusions: Patients who presented with isolated CPP without epilepsy displayed normal cognition when evaluated after a mean period of 13 years. Occurrence of epilepsy, seen in a minority of patients, but not of a single seizure, was associated with mild cognitive deficit and behavioral disturbances in this case series.
dc.description.sponsorship · FAPESP [06/56531-0]
· CNPq [300828/2005-5, 300469/2005]
dc.language.iso eng
dc.publisher W B SAUNDERS CO LTD
dc.relation.ispartof Seizure-European Journal of Epilepsy
dc.rights restrictedAccess
dc.subject Central precocious puberty; Hypothalamic hamartomas; Cognition; Epilepsy
dc.subject.other gelastic epilepsy; clinical spectrum; natural-history; seizures; children; surgery; pattern
dc.title The benign spectrum of hypothalamic hamartomas: Infrequent epilepsy and normal cognition in patients presenting with central precocious puberty
dc.type article
dc.rights.holder Copyright W B SAUNDERS CO LTD
dc.description.group LIM/42
dc.description.group LIM/44
dc.description.group LIM/21
dc.description.group LIM/45
dc.identifier.doi 10.1016/j.seizure.2012.09.013
dc.identifier.pmid 23092686
dc.type.category original article
dc.type.version publishedVersion
hcfmusp.author CUKIER, Priscilla:HC:ICESP
hcfmusp.author CASTRO, Luiz Henrique Martins:HC:ICHC
hcfmusp.author BANASKIWITZ, Natalie:FM:
hcfmusp.author FERREIRA, Luiz Roberto Kobuti:FM:
hcfmusp.author ADDA, Carla Cristina:HC:ICHC
hcfmusp.author LEITE, Claudia da Costa:FM:MDR
hcfmusp.author ARNHOLD, Ivo J. P.:HC:ICHC
hcfmusp.author MENDONCA, Berenice Bilharinho:FM:MCM
hcfmusp.author LATRONICO, Ana Claudia:FM:MCM
hcfmusp.author BRITO, Vinicius Nahime:HC:LIM/42
hcfmusp.author.external · TELES, Leandro Roberto:Univ Sao Paulo, Div Neurol, Hosp Clih, Fac Med, BR-05403900 Sao Paulo, Brazil
hcfmusp.origem.id 2-s2.0-84872163764
hcfmusp.origem.id WOS:000314560200007
hcfmusp.publisher.city LONDON
hcfmusp.publisher.country ENGLAND
hcfmusp.relation.reference · Arita K, 1999, J NEUROSURG, V91, P212, DOI 10.3171/jns.1999.91.2.0212
· Arita K, 2005, NEUROL MED-CHIR, V45, P221, DOI 10.2176/nmc.45.221
· Berkovic SF, 2003, EPILEPSIA, V44, P969, DOI 10.1046/j.1528-1157.2003.59102.x
· BOYKO OB, 1991, AM J NEURORADIOL, V12, P309
· Brito VN, 1999, J CLIN ENDOCR METAB, V84, P3539, DOI 10.1210/jc.84.10.3539
· Castro LH, 2007, SEIZURE-EUR J EPILEP, V16, P50, DOI 10.1016/j.seizure.2006.10.008
· Debeneix C, 2001, HORM RES, V56, P12, DOI 10.1159/000048084
· de Brito VN, 1999, ARCH DIS CHILD, V80, P231
· Frattali CM, 2001, NEUROLOGY, V57, P43
· Freeman JL, 2003, NEUROLOGY, V60, P762
· Garcia-Morales I, 2007, NEUROLOGIA, V22, P11
· Greulich W., 1959, RADIOGRAPHIC ATLAS S
· Jung H, 1999, J CLIN ENDOCR METAB, V84, P4695, DOI 10.1210/jc.84.12.4695
· MAHACHOKLERTWATTANA P, 1993, J CLIN ENDOCR METAB, V77, P118, DOI 10.1210/jc.77.1.118
· MARSHALL WA, 1969, ARCH DIS CHILD, V44, P291
· MARSHALL WA, 1970, ARCH DIS CHILD, V45, P13
· Mullatti N, 2003, EPILEPSIA, V44, P1310, DOI 10.1046/j.1528-1157.2003.04103.x
· Nguyen D, 2003, EPILEPSY BEHAV, V4, P246, DOI 10.1016/S1525-5050(03)00086-6
· Palmini A, 2003, EPILEPTIC DISORD, V5, P249
· Prigatano George P, 2007, Semin Pediatr Neurol, V14, P65, DOI 10.1016/j.spen.2007.03.004
· Regis J, 2000, NEUROSURGERY, V47, P1343
· Striano S, 2005, SEIZURE-EUR J EPILEP, V14, P232, DOI 10.1016/j.seizure.2005.01.004
· VALDUEZA JM, 1994, NEUROSURGERY, V34, P949
· Wechsler D., 1997, WECHSLER ADULT INTEL
· Wechsler D, 1989, WECHSLER PRESCHOOL P
· Wechsler D., 1991, WECHSLER INTELLIGENC
dc.description.index MEDLINE
hcfmusp.citation.scopus 8
hcfmusp.citation.wos 6
hcfmusp.affiliation.country Brasil


Files in this item

This item appears in the following Collection(s)

Show simple item record

Search DSpace



Browse

My Account

Statistics