Hypothalamic Hamartomas and Seizures: Distinct Natural History of Isolated and Pallister-Hall Syndrome Cases

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Teaching NeuroImages: isolated hypothalamic hamartoma vs Pallister-Hall syndrome: imaging and clinical correlation.

Antonio J. da Rocha, PhD Marcos Rosa Junior, MD Fernando Norio Arita, PhD Isolated hypothalamic hamartomas (HH) have a distinct clinical phenotype from that of PallisterHall syndrome (PHS), as HH consist of more severe seizures and cognitive, behavioral, and endocrine disorders.1 The imaging features help one to distinguish these conditions (figures 1 and 2). Isolated HH are hyperintense on flu...

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Familial Pallister-Hall syndrome.

In their report of sibs with the Pallister-Hall syndrome, Thomas et al' make the points that choanal atresia may be a feature of this condition and that it may be caused by a dominant gene. We have recently encountered an infant in whom the findings lend support to both of these suggestions. Our patient, a female, was the first child born to a healthy 31 year old female and her healthy and unre...

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Hypothalamic Hamartomas: Two Cases

This is a MRI study of two patients presented with history of gelastic seizures since many years ago. Plain and post intravenous gadolinium multiplanar MRI imaging of the brain is performed. It showed well-defined non-enhancing mass lesions in the region of hypothalamus and tuber cinereum. It showed signal intensity similar to the gray matter and imaging diagnosis of hypothalamic hamartoma is m...

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Familial Pallister-Hall syndrome.

In their report of sibs with the Pallister-Hall syndrome, Thomas et al' make the points that choanal atresia may be a feature of this condition and that it may be caused by a dominant gene. We have recently encountered an infant in whom the findings lend support to both of these suggestions. Our patient, a female, was the first child born to a healthy 31 year old female and her healthy and unre...

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Pallister-Hall syndrome: clinical and MR features.

A 4-month-old boy with polydactyly and bifid epiglottis was found to have a large sellar and suprasellar mass. When the diagnosis of Pallister-Hall syndrome was made, conservative management was elected. When the patient was 2 years old, the tumor had grown proportionally with the patient, and he was developing appropriately. Although rare, this entity is important to recognize not only for cli...

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ژورنال

عنوان ژورنال: Epilepsia

سال: 2005

ISSN: 0013-9580,1528-1167

DOI: 10.1111/j.0013-9580.2005.68303.x