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Calcineurin-inhibitor-Induced Pain Syndrome clinically mimicking Guillain-Barre’ Syndrome in a pediatric transplant patient (P3.141)

2015; Lippincott Williams & Wilkins; Volume: 84; Issue: 14_supplement Linguagem: Inglês

10.1212/wnl.84.14_supplement.p3.141

1526-632X

Aldo Doerner Rinaldi, Jennifer Bain, Staci D. Arnold, Monica Bhatia, Rama S. Ayyala, Jahannaz Dastgir,

Neurogenetic and Muscular Disorders Research

OBJECTIVE: We present a rare case of Calcineurin-inhibitor-Induced Pain Syndrome (CIPS) in a pediatric transplant patient with lower extremity (LE) pain, absent LE reflexes, and difficulty ambulating. BACKGROUND: CIPS presents with bilateral, symmetric LE pain sparing hips and spine, which is frequently intractable and exacerbated by weight bearing. Symptoms are related to calcineurin-inhibitor (CI) toxicity by neuronal hyperexcitability and enhanced vascular tone which lead to hyperalgesia and increased bone metabolism and vascularity with marrow and periosteal edema. Onset has been associated with elevated CI levels. Diagnosis is made by MRI or bone scan findings of skeletal hypermetabolism and edema. Resolution of symptoms upon reduction or discontinuation of CIs supports the diagnosis. DESIGN/METHODS: A 2 year old girl with B-thalassemia Major, following an allogenic bone marrow transplant on tacrolimus, presented to the pediatric neurology consult service with several weeks of progressive LE pain and difficulty ambulating. Tacrolimus level was supra-therapeutic three weeks prior to symptom onset. On examination, she demonstrated bilateral LE tenderness to touch and discomfort with weight-bearing. Her ambulation was further limited by bilateral foot drop, and LE reflexes could not be elicited. RESULTS: Studies showed normal ESR, CRP, and CK levels, CSF values, MRI umbar spine, and nerve conduction studies. X-rays showed focal periosteal reactions of bilateral tibia and left fibula without fracture. MRI of bilateral LE demonstrated focal periosteal bone formation of the proximal tibia and mid fibular shaft bilaterally with surrounding muscular edema as well as focally increased T2 marrow signal with corresponding contrast enhancement. Tacrolimus was weaned and prednisolone initiated with partial resolution of symptoms. CONCLUSIONS: CIPS is a rare and often difficult to diagnose neurological complication of CIs in the post-transplant patient that, if recognized, may be reversed and effectively treated.