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Nigerian Health Journal

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Charcot-Marie-Tooth Disease in a Child with Acute Lymphoblastic Leukaemia: A Case Report and Review of Literature

GK Eke, NA Akani

Abstract


Background: Charcot-Marie-Tooth disease (CMT) is a common inherited neurologic disorder with various modes of inheritance. Existing peripheral neuropathy is a generally accepted risk factor for increased susceptibility to neurotoxic agents and there is a general acceptance of the concept of medication-induced worsening of CMT. Several authors have reported vincristine neurotoxicity in CMT and vincristine treatment triggering the expression of asymptomatic CMT disease. We report the case of a 10 year old male patient who developed severe neuropathy following treatment with vincristine for his Acute Lymphoblastic Leukaemia.
Methods: The case records of the patient and a review of the relevant literature using available books, journals and online literature search was utilized.
Results: Facial nerve palsy, increasing lower extremities muscle weakness and abnormal gait were noticed 4 weeks into vincristine therapy in a ten year old male on treatment for acute lymphoblastic leukaemia (ALL). On a suspicion of vincristine neurotoxicity, vincristine was excluded from his chemotherapy regimen. Although remission of ALL was achieved within 6 weeks of treatment, the patient's neurological symptoms did not improve even with the withdrawal of vincristine. The patient was diagnosed with Charcot-Marie-Tooth disease resulting from vincristine toxicity.
Conclusion: Patients with Charcot-Marie-Tooth disease may show severe toxicities with vincristine. It is therefore recommended that an extensive neurologic examination should be conducted on any paediatric patient with a diagnosis of malignancy to identify any undiagnosed neurologic deficits and screen for suitability or otherwise of a vincristine containing cytotoxic therapy regimen. This case stresses the need for an urgent health sector response to provide adequate facilities for electrophysiological screening and genetic studies in resource limited centres like ours in order to identify possible cases of undiagnosed CMT and prevent the occurrence of exacerbation by vincristine administration.



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