Title : Imatinib as a treatment for prion disease: A historical case of Sporadic Creutzfeldt Jacob disease complicating chronic myeloid leukemia on Imatinib.
Abstract:
Background:
Creutzfeldt Jakob disease (CJD) is a fatal and rare neurodegenerative disease, It is classified into 3 subgroups: sporadic, genetic and acquired. Sporadic CJD is the most common form, which may be manifested with misleading neuropsychiatric symptoms and resulting in death within a year. Several treatments have failed in sporadic CJD, while 'imatinib mesylate', the first drug to target the Bcr-Abl pathway in chronic myeloid leukemia (CML), has been demonstrated to be effective against prion protein aggregation in experimental model.
Objective:
To describe the first case of sporadic Creutzfeldt Jakob disease (CJD) in a patient with Bcr-ABL-positive chronic myeloid leukemia who survived more than two years on "imatinib mesylate" treatment.
Method:
A 60-year-old female followed for Bcr-Abl positive chronic myeloid leukemia in imatinib. Who presented psychiatric disorders for which was put on antipsychotic medication for 12 months, with a progressive onset of dementia and extrapyramidal syndrome of the upper limb. A brain CT scan, metabolic assessment and vitamin dosages were normal.
The patient was put on L-dopa in addition to psychiatric drugs for more than 6 months, with initial improvement and then progressive worsening of the extrapyramidal syndrome, associated with myoclonus, visual disturbance. MRI Brain was performed and demonstrated bilateral symmetrical hypersignals of the striatum. The electroencephalogram showed a theta rhythm and triphasic periodic abnormalities. A CSF test for 14.3.3 was positive. A diagnosis of probable sporadic CJD was made.
Imatinib mesylate was majored to 800mg/day in addition to symptomatic treatment. Patient died 8 months later.
Results:
Over two years of survival-term in a patient with sporadic CJD and Bcr-Abl positive chronic myeloid leukemia on imatinib mesylate treatment.
Conclusions:
The Bcr-Abl pathway inhibitors represent a promising treatment in sporadic CJD. But more clinical studies are needed to establish their role in long-survival sporadic CJD patients.
Keywords:
Sporadic CJD – Chronic myeloid leukemia – BcrAbl pathway – Imatinib mesylate
What will audience learn from your presentation?
- This historical case of sporadic CJD complicating a Bcr-Abl positive CML, demonstrates a rare long-survival sporadic Creutzfeldt-Jakob disease that is contradictory to traditional survival duration, and supports the suggested role of Bcr-Abl inhibitors to induce a clearance of the pathological prion protein in an experimental model.
- Imatinib mesylate may be an effective treatment in the early stages of the disease and encourages further studies on the use of the Bcr-Abl inhibitor in prion diseases.
- Psychiatric symptoms or parkinsonism can be an early clinical feature in sporadic CJD and should be carefully considered.
