Chronic Inflammatory Demyelinating Polyneuropathy Variant with Creatine-Kinase Elevation and Vanishing Effect of Immunoglobulins
Challenging differential diagnosis, Unusual or unexpected effect of treatment, Diagnostic / therapeutic accidents, Educational Purpose (only if useful for a systematic review or synthesis), Rare co-existance of disease or pathology
Josef Finsterer, Rahim Aliyev
(Department of Neurology, Krankenanstalt Rudolfstiftung, Vienna, Austria)
Am J Case Rep 2017; 18:834-838
Whether creatine-kinase (CK) is elevated or not in chronic inflammatory demyelinating polyneuropathy (CIDP) and its variants is not comprehensively investigated.
CASE REPORT: We report the case of a 47-year-old male who developed weakness of the left lower leg and the right index finger at age 42 years. At age 44 years, paresthesias and dysesthesias of both lower legs and mild right lower leg weakness additionally developed. CK was recurrently elevated since age 42 years but paraprotein and anti-myelin-associated glycoprotein (MAG)-antibodies were negative. Nerve conduction studies at age 43 years showed an axonal and demyelinating lesion with conduction blocks. Cerebrospinal fluid (CSF) investigations revealed mild pleocytosis and elevated protein, which is why CIDP variant was diagnosed. Immunoglobulins were administered with success. Because of recurrent relapses, immunoglobulins were increased at age 45 years, resulting in stabilization. Currently, the patient is infusing immunoglobulins subcutaneously himself.
CONCLUSIONS: CIDP variants may go along with CK elevation, an axonal lesion, pleocytosis, and asymmetry of the lesion. A vanishing effect of immunoglobulins over time may be characteristic of CIDP variants.
Keywords: creatine kinase, Electromyography, Guillain-Barre Syndrome, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating