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A middle-aged woman with numbness and weakness of the extremities

Published:September 23, 2020DOI:https://doi.org/10.1016/j.ejim.2020.09.015

      1. Case description

      A 45-year-old woman presented to the neurology department with numbness and weakness of extremities, which had progressed during 3 months. The patient had tingling sensation in the left neck. Although muscle atrophy was not observed, manual muscle test revealed grade 4 weakness in bilateral deltoid muscles, right upper and left lower limbs. The patient had normal deep tendon reflex, and Babinski reflex was negative bilaterally. Laboratory blood tests showed no abnormalities. Spinal fluid examination revealed a slightly increased protein level of 56 mg/dL (normal range: 10–45 mg/dL) and no white blood cells. Nerve conduction study revealed prolonged distal latencies, reduction in conduction velocities, and delayed F wave latencies of the median, ulnar and tibial nerves, which was suggestive of predominantly distal sensorimotor demyelinating polyneuropathy. The patient had no family history of Charcot-Marie-Tooth (CMT) disease, and the CMT type 1A-related PMP22 gene duplication was absent in the gene analysis. Cervical magnetic resonance neurography (MRN) was performed for the evaluation of peripheral neuropathies (Fig.1). What is your diagnosis for this patient?
      Fig 1
      Fig. 1MRN using coronal fat-saturated T2-weighted imaging revealing symmetrical enlargement and increased signal intensity of cervical nerve roots.

      2. Discussion section

      The patient was diagnosed with idiopathic chronic inflammatory demyelinating polyneuropathy (CIDP) according to the American Academy of Neurology criteria after excluding the causes of secondary CIDP, such as diabetes mellitus, POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes), monoclonal gammopathy, and malignancy. The patient's symptoms remitted after corticosteroid therapy, but relapsed 3 months later. Corticosteroids and intravenous immunoglobulin were repeatedly administered, and the patient has experienced remissions and exacerbations of the neurological symptoms.
      CIDP is considered an autoimmune disorder targeting the myelin of peripheral nerve that progresses over more than 8 weeks. This rare disease is often underrecognized due to its varied presentation and the limitations of clinical, serologic, and electrophysiologic diagnostic criteria. Despite these limitations, early diagnosis and treatment are highly important in preventing axonal degeneration and recovering neurological function. Corticosteroids, intravenous immunoglobulin, and plasma exchange are the most common first-line therapies for CIDP patients [
      • Dimachkie MM
      • Barohn RJ
      Chronic inflammatory demyelinating polyneuropathy.
      ]. Greater than 90% of CIDP patients initially improved with immunosuppressive treatment, but the relapse rate was approximately 50% [
      • Barohn RJ
      • Kissel JT
      • Warmolts JR
      • Mendell JR
      Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria.
      ]. MRI evaluation of the spinal nerve roots is useful for the diagnosis of CIDP. CIDP is one of the hypertrophic neuropathies; the repetitive demyelination and remyelination results in enlargement of peripheral nerves and roots [
      • Freitas MR
      • Nascimento OJ
      • Soares CN
      • Brito AR
      • Domingues RC
      Chronic inflammatory demyelinating polyradiculoneuropathy: two cases with cervical spinal cord compression.
      ]. Moreover, increased T2 signal intensity of nerve roots is considered to reflect the active inflammation with edema in the endoneurium and perineurium.

      Declaration of Competing Interest

      None declared. I disclose no actual or potential conflict of interest including any financial, personal or other relationships with other people or organizations within last three years.

      References

        • Dimachkie MM
        • Barohn RJ
        Chronic inflammatory demyelinating polyneuropathy.
        Curr Treat Options Neurol. 2013; 15: 350-366
        • Barohn RJ
        • Kissel JT
        • Warmolts JR
        • Mendell JR
        Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria.
        Arch Neurol. 1989; 46: 878-884
        • Freitas MR
        • Nascimento OJ
        • Soares CN
        • Brito AR
        • Domingues RC
        Chronic inflammatory demyelinating polyradiculoneuropathy: two cases with cervical spinal cord compression.
        Arq Neuropsiquiatr. 2005; 63: 666-669