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Ohtahara syndrome associated with hemimegalencephaly and intracranial lipoma.

Gowda VK, Bhat A, Bhat M, Ramaswamy P - J Pediatr Neurosci (2015 Apr-Jun)

Bottom Line: Hemimegalencephaly is a disorder of cortical malformation and is associated with various disorders including various neurocutaneous syndromes and many seizure types.We present a case of hemimegalencephaly associated with Ohtahara syndrome and intracranial and facial lipoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Neurology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India.

ABSTRACT
Hemimegalencephaly is a disorder of cortical malformation and is associated with various disorders including various neurocutaneous syndromes and many seizure types. We present a case of hemimegalencephaly associated with Ohtahara syndrome and intracranial and facial lipoma.

No MeSH data available.


Related in: MedlinePlus

Electroencephalogram showing multifocal bursts of spikes, sharp waves, and poly-spikes followed by suppression lasting for 3–4 s suggestive of burst suppression pattern
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Figure 2: Electroencephalogram showing multifocal bursts of spikes, sharp waves, and poly-spikes followed by suppression lasting for 3–4 s suggestive of burst suppression pattern

Mentions: On investigation, complete blood count, serum electrolytes, and serum calcium were within normal limits. EEG findings were suggestive of burst suppression pattern [Figure 2]. Magnetic resonance imaging (MRI) showed a hyper intense lesion in the subcutaneous plane on the left side of face suggestive of a lipoma on axial T1-weighted imaging (T1-WI) [Figure 3a]. Axial T1-WI also demonstrated a hyper intense lesion in the ambient cistern on the right side suggestive of lipoma [Figure 3b]. Axial T2-WI demonstrated left-sided hemimegalencephaly with an enlarged hemisphere and midline shift of the left occipital lobe [Figure 3c]. Axial fluid-attenuated inversion recovery image showed asymmetric cerebellum with abnormal folia [Figure 4a]. Computed tomography brain showed left hemimegalencapahly and intracranial calcification in the right ambient cistern demonstrating a calcified lipoma [Figure 4b].


Ohtahara syndrome associated with hemimegalencephaly and intracranial lipoma.

Gowda VK, Bhat A, Bhat M, Ramaswamy P - J Pediatr Neurosci (2015 Apr-Jun)

Electroencephalogram showing multifocal bursts of spikes, sharp waves, and poly-spikes followed by suppression lasting for 3–4 s suggestive of burst suppression pattern
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4489072&req=5

Figure 2: Electroencephalogram showing multifocal bursts of spikes, sharp waves, and poly-spikes followed by suppression lasting for 3–4 s suggestive of burst suppression pattern
Mentions: On investigation, complete blood count, serum electrolytes, and serum calcium were within normal limits. EEG findings were suggestive of burst suppression pattern [Figure 2]. Magnetic resonance imaging (MRI) showed a hyper intense lesion in the subcutaneous plane on the left side of face suggestive of a lipoma on axial T1-weighted imaging (T1-WI) [Figure 3a]. Axial T1-WI also demonstrated a hyper intense lesion in the ambient cistern on the right side suggestive of lipoma [Figure 3b]. Axial T2-WI demonstrated left-sided hemimegalencephaly with an enlarged hemisphere and midline shift of the left occipital lobe [Figure 3c]. Axial fluid-attenuated inversion recovery image showed asymmetric cerebellum with abnormal folia [Figure 4a]. Computed tomography brain showed left hemimegalencapahly and intracranial calcification in the right ambient cistern demonstrating a calcified lipoma [Figure 4b].

Bottom Line: Hemimegalencephaly is a disorder of cortical malformation and is associated with various disorders including various neurocutaneous syndromes and many seizure types.We present a case of hemimegalencephaly associated with Ohtahara syndrome and intracranial and facial lipoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Neurology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India.

ABSTRACT
Hemimegalencephaly is a disorder of cortical malformation and is associated with various disorders including various neurocutaneous syndromes and many seizure types. We present a case of hemimegalencephaly associated with Ohtahara syndrome and intracranial and facial lipoma.

No MeSH data available.


Related in: MedlinePlus