WBR0197

Author	PageAuthor::Vendhan Ramanujam
Exam Type	ExamType::USMLE Step 2 CK
Main Category	MainCategory::Pediatrics
Sub Category	SubCategory::Endocrine, SubCategory::Neurology, SubCategory::Oncology
Prompt	[[Prompt::A 9 year old girl is brought to the physicians office by her mother who complaints of few recent changes in her daughter. Her daughter has been having episodes of uncontrollable laughter. Her performance in her school has been poor and she often tends to turn aggressive on her classmates and even on her mother. She has also started menstruating recently and her breast development started at the age of 7 years, which is unusual. Which of the following mass lesions might explain her symptoms?]]
Answer A	AnswerA::Craniopharyngioma
Answer A Explanation	[[AnswerAExp::Incorrect-Craniopharyngiomas are epithelial neoplasms arising in the sellar and third ventricular regions. They usually cause hypopituitarism and visual field disturbances.]]
Answer B	AnswerB::Metastatic carcinoma
Answer B Explanation	AnswerBExp::'''Incorrect'''-Metastatic carcinoma generally occurs in older patients and would not be expected to cause these symptoms.
Answer C	AnswerC::Hypothalamic hamartoma
Answer C Explanation	[[AnswerCExp::Correct-Hypothalamic hamartomas may cause symptoms referable to the hypothalamus and its neuroendocrine functions. Most often they lead to either precocious puberty or acromegaly due to overproduction of growth hormone releasing hormone. Patients may also experience paroxysms of laughter along with facial contractions, known as gelastic seizures. If the vocalization has a crying quality, it is called as dacrystic seizures. Cognitive impairments like memory and learning disabilities along with hypothalamic rages might occur. Hypothalamic rages are different form temper tantrums in the sense they occur without any provocation. Pallister Hall syndrome is a rare genetic disorder where large hypothalamic hamartomas can occur along with polydactyly and cutaneous syndactyly. Hypothalamic hamartomas may be cured surgically.]]
Answer D	AnswerD::Choroid plexus papilloma
Answer D Explanation	[[AnswerDExp::Incorrect-Choroid plexus papillomas usually develop intraventricularly and do not extend down into the sella turcica. These tumors affect both children and adults, but they are rare. They are benign if they are surgically accessible and are extirpated early in their evolution.]]
Answer E	AnswerE::Giant aneurysm
Answer E Explanation	[[AnswerEExp::Incorrect-Giant aneurysms occur in many locations, but typically do not cause gelastic seizures or precocious puberty.]]
Right Answer	RightAnswer::C
Explanation	[[Explanation::Hypothalamic hamartomas are nonneoplastic malformations involving neurons and glia in the region of the hypothalamus. They may be discovered incidentally, either on imaging or at autopsy. They may cause symptoms referable to the hypothalamus and its neuroendocrine functions. Most often they lead to either precocious puberty or acromegaly due to overproduction of growth hormone releasing hormone. Patients may also experience paroxysms of laughter along with facial contractions, known as gelastic seizures. If the vocalization has a crying quality, it is called as dacrystic seizures. Cognitive impairments like memory and learning disabilities along with hypothalamic rages might occur. Hypothalamic rages are different form temper tantrums in the sense they occur without any provocation. Pallister-Hall syndrome is a rare genetic disorder where large hypothalamic hamartomas can occur along with polydactyly and cutaneous syndactyly. Hypothalamic hamartomas may be cured surgically. Educational objective: Hypothalamic hamartoma is a rare benign brain tumor of the hypothalamus. They usually cause a combination of several symptoms like gelastic or dacrystic seizures, cognitive impairments, hypothalamic rages and precocious puberty. Educational Objective: References: ]]
Approved	Approved::Yes
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