Teaching NeuroImages: Hypothalamic hamartoma and polydactyly

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Teaching video neuroimages: hypothalamic hamartoma: an overlooked entity.

Neeraj N. Baheti, DM Ajith Cherian, DM Ramshekhar Menon, DM Ashalatha Radhakrishnan, DM A 21-year-old man presented with brief spells of inappropriate laughter since 6 years of age. Three gelastic seizures were recorded during video-EEG monitoring (video on the Neurology Web site at www.neurology. org). MRI revealed a small hypothalamic hamartoma (figure 1). Gelastic seizures can also arise fro...

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Teaching neuroImages: short stature, imperforate anus, and polydactyly: When is a hypothalamic mass an incidentaloma?

A 4-year-old girl with history of polydactyly and imperforate anus surgeries presented with short stature. Brain MRI revealed a large hypothalamic hamartoma (figure). Pallister-Hall syndrome (PHS) was diagnosed. Biopsy was not indicated given stable and typical tumoral appearance. Annual evaluations, hormonal replacement, and medical genetics consult were recommended. Compared to sporadic hypot...

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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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Hypothalamic hamartoma.

To cite: Grech R, Looby S, Thornton J, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2012008273 DESCRIPTION A 9-year-old girl presented with an 8-month history of gelastic seizures and precocious puberty. Clinical evaluation including full neurological assessment was otherwise unremarkable. Endocrine tests were normal and there was no family or surgical h...

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Hypothalamic hamartoma in adults.

Epilepsy in adult patients with hypothalamic hamartoma has not been well studied. It is uncommon but merits recognition. In this paper, 14 adult patients with hypothalamic hamartoma and epilepsy, of whom three developed epilepsy only in adult life, are presented. The later onset of epilepsy appears to be associated with a milder epilepsy syndrome, less severe learning difficulties and behaviour...

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ژورنال

عنوان ژورنال: Neurology

سال: 2019

ISSN: 0028-3878,1526-632X

DOI: 10.1212/wnl.0000000000008580