Hamartoma of CNS Associated With Precocious Puberty

Of, Zúñiga; Sm, Tanner; Wo, Wild; Hd, Mosier

doi:10.1001/archpedi.1983.02140280025007

Cited by 30 publications

(4 citation statements)

References 25 publications

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“…Patients with central precocious puberty (CPP) and HH, without seizures, are effectively treated by gonadotropin‐releasing hormone administration, 19 and surgery is considered only in particular conditions. 20 Ancient series report about the efficacy of microsurgery to treat both CPP and seizures in patients with HH, 21 , 22 , 23 with only one recent consistent study confirming these data, selectively in type I HH. 24 In our series, CPP improves after surgery in 58% of cases (7/12) (with stability of symptoms in 33%), with all improved patients being also in Engel class I.…”

Section: Discussionmentioning

confidence: 99%

Surgical and radiosurgical treatment of hypothalamic hamartoma: The Italian experience between 2011 and 2021

Rizzi,

Consales,

Tramacere

et al. 2024

Epilepsia Open

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ObjectiveTo investigate the Italian experience on the surgical and radiosurgical treatment of drug‐resistant epilepsy due to hypothalamic hamartoma (HH) in the period 2011–2021 in six Italian epilepsy surgery centers, and to compare safety and efficacy profiles of the different techniques.MethodsWe collected pseudo‐anonymized patient's data with at least 12 months of follow‐up. Surgical outcome was defined according to Engel classification of seizure outcome. Univariate analysis was performed to assess the risk of post‐operative seizures, categorized in dichotomous variable as favorable and unfavorable; explanatory variables were considered. Mann–Whitney or Chi‐squared test were used to assess the presence of an association between variables (p < 0.05).ResultsFull presurgical and postoperative data about 42 patients from 6 epilepsy surgery centers were gathered. Engel class I was reached in the 65.8% and 66.6% of patients with gelastic and non‐gelastic seizures, respectively. Other than daily non‐gelastic seizures were associated with seizure freedom (p = 0.01), and the radiological type presented a trend toward significance (p = 0.12).SignificanceEndoscopic disconnection and laser interstitial thermal therapy are effective in the treatment of HH‐related epilepsy, with a tolerable safety profile. Both gelastic and non‐gelastic seizures can be treated, also in patients with a long history of seizures.Plain Language SummaryThis study collected data about 42 patients with HH‐related epilepsies. Endoscopic disconnection and laser therapy are both effective and safe in the treatment of hypothalamic hamartoma‐related epilepsies.

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Section: Discussionmentioning

confidence: 99%

Surgical and radiosurgical treatment of hypothalamic hamartoma: The Italian experience between 2011 and 2021

Rizzi,

Consales,

Tramacere

et al. 2024

Epilepsia Open

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“…Rivarola et al [ 26 ] reported that among the 30 patients with CPP and organic central lesions, 3 were younger than one year and had hypothalamic hamartoma, germinoma or suprasellar arachnoid cyst. Zuniga et al [ 27 ] reported that CPP due to hypothalamic hamartoma begins before 3 years of age in 84% of cases. Cacciari et al [ 28 ] showed that all girls with pubertal signs before 2 years of age and 80% with early menarche due to CPP had a suspected hamartoma.…”

Section: Discussionmentioning

confidence: 99%

Premature Pubarche before One Year of Age: Distinguishing between Mini-Puberty Variants and Precocious Puberty

et al. 2015

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BackgroundThe aim of this study was to facilitate the distinction between the benign “mini-puberty of early infancy” and precocious puberty (PP).Material/MethodsWe compared 59 patients (21 boys and 38 girls) seen for pubic hair development before one year of age diagnosed as mini-puberty to 13 patients (2 boys) in whom pubertal development before one year revealed a PP.ResultsThe boys with mini-puberty presented with pubic hair development and prepubertal testicular volume, with low plasma testosterone concentrations. Their gonadotropin responses to gonadotropin releasing hormone (GnRH) test showed predominant luteinising hormone increase in 9/13. The girls presented with pubic hair development that was accompanied by breast development in 47% of cases, with low plasma estradiol concentrations. Their gonadotropin responses showed predominant follicle-stimulating hormone increase in the 17 evaluated.The patients with PP had organic central PP (5 hypothalamic hamartoma) or idiopathic central PP (n=6), or peripheral PP (one ovarian tumor and one congenital adrenal hyperplasia). The diagnosis was challenging only in 3 girls with idiopathic central PP presenting with prepubertal plasma estradiol concentrations and responses to GnRH test.ConclusionsThe diagnosis of PP was easily determined based on the clinical presentation and the pubertal concentrations of testosterone in boys or of estradiol in girls, as was the diagnosis of central or peripheral origin of PP based on gonadotropin response to the GnRH test. Once PP is excluded, these patients need careful follow–up and physician consultation is needed if clinical pubertal signs progress.

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“…Seizures in HH have a profound effect on cognitive and behavioral development. Forty-eight percent of patients with HH and gelastic generalized seizures have behavioral disturbance [20]. In older children, these symptoms can include hyperkinesia, aggressiveness, psychosis, frontal lobe syndrome, and anxiety disorders [1].…”

Section: Presentation and Diagnosismentioning

confidence: 99%

“…There is a well-documented link between HH and central precocious puberty [7]. Between 21-74% of patients with HH and gelastic seizures also have central precocious puberty [1,17,20]. Patients with precocious puberty have also been found to have significantly larger HH volumes [17].…”

Section: Presentation and Diagnosismentioning

confidence: 99%

Advances in the neurosurgical management of epilepsy caused by hypothalamic hamartoma

et al. 2015

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Hypothalamic hamartoma (HH) is an uncommon intrinsically epileptogenic lesion of the hypothalamus that is associated with medically intractable epilepsy. Intractable seizures caused by HH may lead to progressive cognitive delay and are also associated with generation of secondary epileptic foci. Therefore, early referral and surgery for children with HH is critical. Recent advances in minimally invasive neurosurgical techniques, including endoscopy, radiosurgery, magnetic resonance-guided laser interstitial ablation, and deep brain stimulation, have increased both the variety and feasibility of treatment options available for treating the intractable seizures caused by HH. In this manuscript, we review emerging techniques available for treatment of intractable seizures associated with HH.

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Hamartoma of CNS Associated With Precocious Puberty

Cited by 30 publications

References 25 publications

Surgical and radiosurgical treatment of hypothalamic hamartoma: The Italian experience between 2011 and 2021

Surgical and radiosurgical treatment of hypothalamic hamartoma: The Italian experience between 2011 and 2021

Premature Pubarche before One Year of Age: Distinguishing between Mini-Puberty Variants and Precocious Puberty

Advances in the neurosurgical management of epilepsy caused by hypothalamic hamartoma

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