Research Article | Open Access
AN INTERESTING CASE REPORT OF PORENCEPHALIC CYST PRESENTING WITH SPASTIC HEMIPLEGIA
Dr.Popuri Naga Jyothi, Dr.Revathi.V., Dr.Shanthi Ramesh , Dr.Sundari. S
Pages: 3245-3247
Abstract
Porencephaly is an extremely rare neurological disease characterized by the presence of solitary or multiple
degenerative cerebrospinal fluid (CSF) cavities within the brain parenchyma2
. This condition is probably caused
by vascular occlusion resulting from an insult during foetal development or an injury occurring later in life. Its
diagnosis depends on demonstrating a well-defined CSF-filled space-occupying lesion communicating with
ventricles on CT scan or MRI of brain1
.They are often associated with other malformations of the brain,
including microcephaly, abnormal patterns of adjacent gyri and encephalocele. Affected infants tend to have
many problems including intellectual disability, spastic hemiparesis or quadriparesis, optic atrophy and seizures.
Mutations in the COL4A1 and COL4A2 genes have been described in cases of familial porencephaly.1 The
rarity of occurrence and varied presentation of such a lesion present a challenge to clinicians. Little is known
about the pathogenesis and appropriate management of porencephalic cysts. The prognosis of porencephaly
depends on the location and extent of the cyst. The best way to contain the increase in incidence of congenital
porencephaly is genetic counselling and prenatal testing in affected individuals and those at risk
Keywords
Spasticity, porencephalic cyst, developmental delay