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Congenital varicella syndrome

To Dr Richard M. Ransohoff, M.D.
Specialty interests: Neurovirology

To Dr Bruce H. Cohen, M.D.
Specialty interests: Pediatric neurology

Dear Dr Ransohoff and Dr Cohen,

The reports on my 3 years old son MRI scan Diagnostic Radiology state as follows:

First MRI scan:
There is a large CSF space in the middle cranial fossa on the left. The adjacent temporal lobe is compressed postero-medially. The left frontal lobe is compressed supero-medially. There is a similar much smaller cystic CSF space in the right middle cranial fossa. This is displacing the right temporal lobe minimally but is not causing any significant mass effect.

The brain is overall small and there is some relative atrophy of the left frontal lobe. There is a focal area of grey matter heterotopia in the left occipital lobe. There is associated T2 high signal extending from this region to the trigone of the left lateral ventricle. A further similar aea is seen on the medial border of the right occipital lobe with further white matter changes.

There does not appear to be any abnormality of the intra petrous structures.

Conclusion: There is a large left middle cranial fossa arachnoid cyst in the smaller right middle fossa arachnoid cyst. There are in addition changes within the cerebral hemispheres. These are most marked in the occipital lobes where there is apparent cortical dysplasia and associated white matter change. The combination of these findings suggest an intra uterine gestational insult. It is possible that the arachnoid cysts are congenital and the changes seen in the occipital lobes relate to hypoxic ischaemic injury in the perinatal period but there are no associated features in the basal ganglia to support this.

Second MRI (repeat) scan:
The bilateral middle cranial fossa arachnoid cysts have not altered in their size or extent. Similarly the parenchymal changes in the occipital lobes are unaltered. The appearances are in keeping with a gestational insult as previously suggested. There is no evidence of progression.

My son's brain and sight abnormalities as well as two chicken pox scars (pock marks) on his body and VZV persistent IgG detected six months ago, despite the fact that he never had any chicken pox rash since birth make me strongly believe that this is the result of congenital varicella syndrome.
During the pregnancy with my son,  I was exposed to the virus during my 13th week gestation and VZV visible signs (chicken pox rash) appeared during my 15th week gestation. I was also feeling terribly itchy and dizzy.

Could you please let me know if my son's brain abnormality is consistent with congenital varicella syndrome?

I thank you in advance,

Looking forward to hearing from you soon,

Yours sincerely,

1 Responses
Avatar universal
Dear Safia:

Both Dr Cohen and Dr. Ransohoff are not part of the medhelp forum.  In order to contact them you must make an appointment with the Cleveland Clinc Foundation.  


CCF Neuro MD
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