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Sturge-weber syndrome: a case report with persistent headache.

Balkuv E, Isik N, Canturk IA, Isik N, Basaran R - Pan Afr Med J (2014)

Bottom Line: Sturge-Weber syndrome (SWS) is a rare congenital disorder characterized by a facial vascular nevus associated with an ipsilateral leptomeningeal angioma.SWS are a very rare and challenging disease for both the patients and their families.Usually migraine type headache is seen in SWS but it should not be forgotten that more generalized headaches like tension type may also be seen.

View Article: PubMed Central - PubMed

Affiliation: Istanbul Medeniyet University Goztepe Education and Research Hospital, Neurology Department, Istanbul, Turkey.

ABSTRACT
Sturge-Weber syndrome (SWS) is a rare congenital disorder characterized by a facial vascular nevus associated with an ipsilateral leptomeningeal angioma. Headache is a rare component of SWS and when it occurs it usually occurs as a migraine-like headache. We aimed to present a SWS patient with episodic tension type headache and to draw attention in different types of headaches that can be seen in SWS. A 21 year old female patient with the diagnosis of SWS was suffering from severe headaches. At her physical examination a facial nevus -occurred due to choroid angioma- was observed. On her neurological examination a mild asymmetry of upper extremities was visible. She had a 2 year history of frequent non-pulsating headaches. There was no nausea or aura like symptoms accompanying the headache. Headaches were lasting for hours. The pain was bilateral and pressing in quality. SWS are a very rare and challenging disease for both the patients and their families. Usually migraine type headache is seen in SWS but it should not be forgotten that more generalized headaches like tension type may also be seen.

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Related in: MedlinePlus

Axial T1 weighted cranial MRI shows right calvarial thickness compared to the left and right hemisphere is asymmetrically smaller then the left
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Figure 0003: Axial T1 weighted cranial MRI shows right calvarial thickness compared to the left and right hemisphere is asymmetrically smaller then the left

Mentions: Cranial CT scans showed diffuse atrophy in the right hemisphere and irregular double-contoured gyriform cortical calcifications in the right occipital area (Figure 2). Gadolinium enhanced brain MRI revealed multiple dilated pial venous vascular structures on right hemisphere also with the diffuse atrophy on the same side. Axial T1 weighted cranial MRI shows right calvarial thickness compared to the left and right hemisphere is asymmetrically smaller than the left (Figure 3). In addition to that, T2 weighted MRI shows extensive venous formations around corpus of right lateral ventricle and at Gallen vein localization and widespread vascular formations are seen at perivascular space, anterior to third ventricle at Willis polygon localization and at right temporooccipital area at quadrigeminal cistern localization (Figure 4). She was performed a proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded. Proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded.


Sturge-weber syndrome: a case report with persistent headache.

Balkuv E, Isik N, Canturk IA, Isik N, Basaran R - Pan Afr Med J (2014)

Axial T1 weighted cranial MRI shows right calvarial thickness compared to the left and right hemisphere is asymmetrically smaller then the left
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4231313&req=5

Figure 0003: Axial T1 weighted cranial MRI shows right calvarial thickness compared to the left and right hemisphere is asymmetrically smaller then the left
Mentions: Cranial CT scans showed diffuse atrophy in the right hemisphere and irregular double-contoured gyriform cortical calcifications in the right occipital area (Figure 2). Gadolinium enhanced brain MRI revealed multiple dilated pial venous vascular structures on right hemisphere also with the diffuse atrophy on the same side. Axial T1 weighted cranial MRI shows right calvarial thickness compared to the left and right hemisphere is asymmetrically smaller than the left (Figure 3). In addition to that, T2 weighted MRI shows extensive venous formations around corpus of right lateral ventricle and at Gallen vein localization and widespread vascular formations are seen at perivascular space, anterior to third ventricle at Willis polygon localization and at right temporooccipital area at quadrigeminal cistern localization (Figure 4). She was performed a proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded. Proteus intelligence test in which she had 75 points and accepted as mildly mentally retarded.

Bottom Line: Sturge-Weber syndrome (SWS) is a rare congenital disorder characterized by a facial vascular nevus associated with an ipsilateral leptomeningeal angioma.SWS are a very rare and challenging disease for both the patients and their families.Usually migraine type headache is seen in SWS but it should not be forgotten that more generalized headaches like tension type may also be seen.

View Article: PubMed Central - PubMed

Affiliation: Istanbul Medeniyet University Goztepe Education and Research Hospital, Neurology Department, Istanbul, Turkey.

ABSTRACT
Sturge-Weber syndrome (SWS) is a rare congenital disorder characterized by a facial vascular nevus associated with an ipsilateral leptomeningeal angioma. Headache is a rare component of SWS and when it occurs it usually occurs as a migraine-like headache. We aimed to present a SWS patient with episodic tension type headache and to draw attention in different types of headaches that can be seen in SWS. A 21 year old female patient with the diagnosis of SWS was suffering from severe headaches. At her physical examination a facial nevus -occurred due to choroid angioma- was observed. On her neurological examination a mild asymmetry of upper extremities was visible. She had a 2 year history of frequent non-pulsating headaches. There was no nausea or aura like symptoms accompanying the headache. Headaches were lasting for hours. The pain was bilateral and pressing in quality. SWS are a very rare and challenging disease for both the patients and their families. Usually migraine type headache is seen in SWS but it should not be forgotten that more generalized headaches like tension type may also be seen.

Show MeSH
Related in: MedlinePlus