qkuk.departament of neurology presentation doc.dr.halil ajvazi-doc.dr.valbona neurolog
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Sturge Weber sindromi; Halil Ajvazi; Ophtalmology; Kosovo2008 - Prishtina Salzburg by Austrian and American expertsKosova Open Medical ClubeTRANSCRIPT
Sturge Weber SyndromeCase report study.
U n i v e r s i t y C l i n i c a l C e n t e r o f K o s o v o , Departament of Neurology - PRISHTINA
U n i v e r s i t y C l i n i c a l C e n t e r o f K o s o v o , Departament of Neurology - PRISHTINA
G o v o r i V . , V r a n i c a S . , M o r i n a N . , A j v a z i H .
AUTORS :
Date:
09.OCT.2008.E-mail:[email protected]
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 factor, and it occurs sporadically.
University Clinical Center of Kosovo , Departament of Neurology
Valbona GOVORISylen VRANICA
Halil AJVAZI9.oct.2008
Prishtina
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 wine stain, localized in the complete left half of the face, including left half of the neck, with right sided hemi paresis. Phenobarbital was excluded, and we started treatment with Carbamazepine 600 mg per day. He has been seizure free for almost three years.University Clinical Center of Kosovo , Departament of Neurology
Valbona GOVORISylen
VRANICAHalil AJVAZI9.oct.2008
Prishtina
Imaging findings:X ray of the skull showed confluent “tram- line” calcifications,
from the left frontal sinus to the posterior part of parietal region.
University Clinical Center of Kosovo , Departament of Neurology
Valbona GOVORISylen
VRANICAHalil AJVAZI9.oct.2008
Prishtina
• Cranial Computed Tomography showed cortical and sub cortical gyriform calcifications of the left side and cortical atrophy.
University Clinical Center of Kosovo , Departament of Neurology
Valbona GovoriSylen
VrannicaHalil AJVAZI9.oct.2008
Prishtina
University Clinical Center of Kosovo , Departament of Neurology
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.
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.
University Clinical Center of Kosovo , Departament of Neurology
Valbona GOVORISylen
VRANICAHalil AJVAZI9.oct.2008
Prishtina
(Foto:1,2. Gonyoscopi examination. dr.A.H ).
1. 2.
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.
University Clinical Center of Kosovo , Departament of Neurology
Valbona GOVORI
Sylen VRANICA Halil AJVAZI9.oct.2008.
Prishtina
THANK YOU
JU FALEMDEROJM
Sturge Weber Syndrome. Case report study.
Autors:
1. Govori Valbona 2. Vranica Sylen 3. Morina Nada 4. Ajvazi Halil
Sincerely, yours .., University Clinical Center of Kosovo, Departament of Neurology - PRISHTINA.
October-2008. E-mail:[email protected]
QKUK-Prishtina
Valbona GOVORISylen
VRANICAHalil AJVAZI9.oct.2008
Prishtina