PMC5433337_01
EDSS and EDSS equivalent from diagnosis to last clinical contact June 2015. At the time of diagnosis, the patient had reached an EDSS of 2.5. He experienced several clinical relapses, which were supported by MRI findings and his EDSS was 3.0 when natalizumab (NTZ) was introduced four years after diagnosis. Despite treatment with NTZ, he continued to experience clinical relapses and exhibited improvement when treated with steroids. MRI showed only a few medullary lesions on T2 at the time of NTZ discontinuation three years later, and no new lesions on MRI brain. His EDSS was at this time 5.5 due to a spastic paraparesis and this was unchanged as he started BEAM six months later. For the few months post-treatment his EDSS climbed to 7.0, most likely due to side effects of BEAM and repeated infections, though engraftment syndrome cannot be excluded. After some improvement, he once again experienced gait difficulties in September and in January 2014 he was diagnosed with probable CIDP based on neurographic findings and a flaccid paraparesis. EDSS was equivalent to 7.5 at the time of diagnosis. EDSS: Expanded Disability Status Scale; MRI: magnetic resonance imaging; BEAM: carmustine, etoposide, cytarabine and melphalan; CIDP: chronic inflammatory demyelinating polyradiculoneuropathy.
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Disability Status Scale (EDSS) score at the time was 3.0 (Figure 1). Despite NTZ, he continued to experience yearly relapses and was treated with steroids with clinical improvement.
PMC5433337_02
Summary of neurographic findings at time of diagnosis. The patient had more than two years' progression of symmetrical polyradiculopathy with areflexia and no wasting. According to the European Federation of Neurological Societies/Peripheral Nerve Society guideline on Management of Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP), the patient had probable, if not definite, CIDP. The image on the bottom left shows prolongation of F-wave latency in the left tibial nerve and the image on the bottom right shows amplitude reduction and a partial motor conduction block in the right peroneal nerve.
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Nerve conduction studies showed sensory-motor polyneuropathy of probable demyelinating type in the lower extremities with pronounced F-response abnormality (Figure 2).
PMC5433337_03