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Dr. Andrew Rynne

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3 years old baby suffering from Agyria pachygyria

About my son age 3yrs. healthy,he is suffering from AGYRIA- PACHYGYRIA COMPLEX (according to MRI ).He is getting 3 to 4 attacks daily,taking medicine SOVAL 2.5ML,TEGRETAL 5MLtwice a day.
Sun, 5 Jun 2011
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Dermatologist 's  Response
Agyria-pachygyria is a disease of neuronal migration characterized by total or partial absence of sulci and thickened cortical mantle.
Pachygyria was considered as a less severe form of agyria-pachygyria complex and had a broader spectrum of clinical features than agyria
. In many cases, agyria and pachygyria coexist in the same patient.
Although some of these disorders have a genetic basis, the cause of the migrational arrest is still unknown.
Seizure and development delay are the most common presentations.
These patients are always retarded in motor or intellectual function.

The advent of CT and MRI have made the diagnosis of agyria-pachygyria during life possible.
In addition to neuroimaging studies, EEG abnormalities are also helpful in the diagnosis of agyria-pachygyria
These abnormalities in agyria-pachygyria described previously including generalized high amplitude fast activity, high amplitude sharp- and slow-wave complexes and an alternating pattern consisting of bursts of sharp waves alternating with periods of electrocerebral depression.

Agyria is often associated with dysgenetic syndromes such as Miller-Dieker (A developmental defect of the brain caused by incomplete neuronal migration and characterized by smoothness of the surface of the brain (lissencephaly) occurring in association with absence of the sulci and gyri (agyria) and thickening of the cerebral cortex with four rather than six layers (pachygyria), microcephaly, characteristic facial appearance, retarded growth and mental development, neurological complications, and multiple abnormalities of the brain, kidneys, heart, gastrointestinal tract, and other organs.) and Walker-Warburg (cobblestone lissencephaly II).
do continue with this medicine
attack will reduce
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Hi Doctor,

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Global delayed deveploment .Currently child is 2yrs and 2month. Still unable to crawl,walk, unable to suck milk and unable talk.Had brain injury during delivery was admitted at NICU for 14 days,was turn into bluish and hypoglycemia while admitted at NICU.child having fits (jerketing movement) on and of since born.child development is getting better but not normal for child age.

Diet, Motion, Urine:
Maintly taking soft diet only (porridge)Able to pass motion everyday if take medication(Duphalac)no problem in passing urine.
Treatment Done:
Under pediatrician follow up and taking medication for fits. No any surgery done.(syr clonazepam 0.2mg/2ml)
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CT brain:microcephaly.
Other problem:
Left side upper limb & lower limb weakness.reduce in left hand function.child tries to getting up since having weakness onleft side tend to fall.
Do you have any suggestion and treatment for my son.


Regards & Thanks
Nirmala

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