Sturge Weber Syndrome

Case report study.

AUTORS :

Govori V.,Vranica S.,Morina N., Aj vazi H.

Unive rsity Clini cal Center of Kosovo

Departament of Neurology - PRISHTINA

Date:
09.OCT.2008.
E-mail:valbonag@hotmail.com
 Background
• The Sturge- Weber syndrome is a rare
disease, which is characterized by
presence of a birth mark called port wine
stain, usually on one side of the face,
associated with abnormality of the brain
caused by abnormal blood vessels
( angiomas). That occurs in the cerebral
cortex, usually on one side of the brain.
Both sexes are equally affected by the
disease, and there are no racial difference
reported. There is no known hereditary
Valbona GOVORI factor, and it occurs sporadically.
Sylen VRANICA
Halil AJVAZI
9.oct.2008
Prishtina University Clinical Center of Kosovo , Departament of Neurology
 Case report:
• this is a case report study of a 18 years old mentally disabled boy, who
developed seizures at the age of 4 months. At that time he was
hospitalized and treated in the Department of Neurology in Skopje –
Macedonia. The diagnosis was done according to the clinical signs: a
port- wine stain on the left side of the face along the distribution of the
trigeminal nerve, generalized seizures and hemi paresis of the right side
of the body. He was treated with Phenobarbital 30 mg per day until 2005.
Despite that treatment he had had serial seizures from time to time until
2005, when he came for the first time in the Clinic of Neurology in
Prishtina.

He was brought in the Clinic of Neurology with severe complex partial

seizures, presenting history of not well controlled epilepsy, on long term
period of time with Phenobarbital. Physical examination revealed with port
Valbona GOVORI wine stain, localized in the complete left half of the face, including left half
Sylen of the neck, with right sided hemi paresis. Phenobarbital was excluded,
VRANICA and we started treatment with Carbamazepine 600 mg per day. He has
Halil AJVAZI
9.oct.2008
been seizure free for almost three years.
Prishtina University Clinical Center of Kosovo , Departament of Neurology
 Imaging findings:
X ray of the skull showed confluent “tram- line” calcifications,
from the left frontal sinus to the posterior part of parietal
region.

Valbona GOVORI
Sylen
VRANICA
Halil AJVAZI University Clinical Center of Kosovo , Departament of Neurology
9.oct.2008
Prishtina
 • Cranial Computed Tomography showed cortical and sub cortical
gyriform calcifications of the left side and cortical atrophy.

Valbona
Govori
Sylen
Vrannica
Halil AJVAZI University Clinical Center of Kosovo , Departament of Neurology
9.oct.2008
University Clinical Center of Kosovo , Departament of Neurology
University Clinical Center of Kosovo , Departament of Neurology
• MRI of the brain revealed with severe left cerebral
hemi atrophy, bilateral cerebellar cortical atrophy,
choroids plexus of the left ventricle
wider then the right one.

University Clinical Center of Kosovo , Departament of Neurology

 Interictal encephalogram showed bilateral slow wave activity, grater
over the left side with epileptogenic activity in the left temporo
parietal region
The patient was referred for neuropsychological and
neuroophthalmologic examination
Psychological examination revealed with IQ 52. He also showed
latent aggressive tendencies and emotional lability.

Ophthalmologic examination of the left eye showed congestion of

blood vessels, initial compensated glaucoma with increased
ocular tension of 24 mmHg, papilla nerve optic had Excavation
of 0.3-0.4, Deflection D=I. Irido-corneal open angle and presents
neovascularization, Schlem channel was filled with blood. The
right eye was unremarkable.
1. 2.

Valbona GOVORI
Sylen
VRANICA
Halil AJVAZI (Foto:1,2. Gonyoscopi examination. dr.A.H ).
9.oct.2008
Prishtina University Clinical Center of Kosovo , Departament of Neurology
 Discussion:
• This is a complete Sturge- Weber syndrome that consists of
cerebral calcifications, birth mark, seizures, glaucoma, hemi
paresis, mental retardation and cerebral atrophy.
• Most cases with Sturge- Weber syndrome are not life
threatening. With a vigorous control and treatment of
symptoms, such as seizures, visual problems, paralysis, mental
disorders, quality of life can be preserved. Treatment involves
early control of seizures and prevention of complications.

Conclusion:
• Sturge- Weber syndrome is a rare disease in the group of
phakomatoses that cause physical, psychical and social
disorders. Professional counseling and professional
organizations and groups can provide help to patients and their
family to work out their problems.

Valbona
GOVORI
Sylen VRANICA
Halil AJVAZI
9.oct.2008.
University Clinical Center of Kosovo , Departament of Neurology
Prishtina
 THANK YOU

JU FALEMDEROJM

Sturge Weber Syndrome. Case report study.

Valbona GOVORI
Sylen
VRANICA
Halil AJVAZI
9.oct.2008 Autors:
Prishtina