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Cerebellar Atrophy

Dear Sir/ Madam
My son aged 13 years has been diagonosed Wilson Disease. The MRI of Brain reads "Hyperintense signal is seen in bilateral caudate and putamen on FLAIR and T2W images. It appears hypointense on T1W images. No abnormal signal is seen on gradient echo images. There is mild atrophy of caudate and putamen with mild exvacuo dilatation of the frontal horn of lateral ventricles."
He is having slurring in speech, tremor in hand resulting in poor handwriting. This is seen during last 3-4 months. He is taking Tiientine Dihydrochloride 300 mg cap thrice a day and one Zinc Acetatae capsule a day.
Please tell me whether the effect of copper deposition on brain will go away or not ?
How much time it may take to recover the normal speech and normal handwriting ?
Whether any other medicine is to be given ?

Thanks and regards

Ravi Kant
2 Responses
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Avatar universal
Although your son has been diagnosed with Wilson's, it sounds as if he's not getting any better. Has he chelated or is he chelating at the moment? Are they regularly monitoring his urine to see how well his body is ridding itself of not only the excess copper, but other heavy metals as well (usually, the body has stores of other heavy metals as well as copper)? Is he making an effort to stay away from the copper-rich foods, the ones those who have Wilson's should avoid? Also, if he is chelating, it is very important that he is drinking enough fluids, especially water. You don't state if you see a conventional doctor or an alternative doctor; I see an Integration (alternative) doctor who has an M.D. She not tests me for heavy metals, but also for nutrient elements. Perhaps your son is either deficient or has an excess in one of those; taking supplements to build up the body and make it strong again will help tremendously.
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Avatar universal
Hi Ravikant,
Wilson’s disease is an inherited disorder of copper metabolism, resulting in the toxic accumulation of copper in the liver, brain, and other organs.
Individuals with Wilson’s disease have mutations in the ATP7B gene.
Dystonia, in-coordination, or tremor may be present, and dysarthria and dysphagia are common. Autonomic disturbances may also be present.
Microscopic hematuria is common.
How was he diagnosed with Wilson's disease? What investigations have been done? Have you seen a neurologist and ophthalmologist so far?
The “gold standard” for diagnosis is an elevated copper level on liver biopsy.
Treatment for Hepatitis or cirrhosis without de-compensation should be treated with zinc.
For pts with hepatic de-compensation, trientene plus zinc is recommended, though liver transplantation should be considered for severe hepatic de-compensation.
How long your son's medications have been planned?
If detected and started on treatment the effects of copper are reversible with rapid improvement in symptoms.
Keep me informed if you have any queries.
Bye.
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