Combination Therapy of Corticosteroids and Immunosuppressants Shows Efficacy in Rare Case of Overlapping CIDP and NMOSD

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Results show that chronic inflammatory demyelinating polyradiculoneuropathy was associated with neuromyelitis optica spectrum disorder and may be treated through a combination approach, although the mechanisms of it are still not understood.

Baarid Luqman Hamidi, MD  (Credit: Dr. Moewardi Surakarta)

Baarid Luqman Hamidi, MD

(Credit: Dr. Moewardi Surakarta)

A rare case study of a 49-year-old woman who presented with an overlap of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and neuromyelitis optica spectrum disorder (NMOSD) showed that a combination of corticosteroids and immunosuppressants treatment may improve clinical outcomes.1

Following the diagnosis of both CIDP and NMOSD, the woman was administered pulse therapy with high-dose corticosteroids (1000 mg, q.1.d) for 3 days. After the completion of those doses, the authors reported a marked improvement in the extremities. To serve as a disease-modifying therapy for NMOSD, the authors had the patient on a regimen of methotrexate (25 mg each week), azathioprine (50 mg TID), and prednisone (5 mg TID). Researchers noted that the patient’s vertigo, nausea, and vomiting subsided, and then 7 days following hospitalization, the woman became discharged from the facility.

Conducted by lead author Baarid Luqman Hamidi, MD, a neurologist at Rumah Sakit Umum Daerah Dr. Moewardi Surakarta in Indonesia, and colleagues, the patient was brought to the emergency department with severe acute-onset vertigo 4 days before admission. Her condition developed suddenly while watching television and was felt throughout the day. Thereafter, the woman reported experiencing nausea, recurrent vomiting, hiccups, and blurry vision. The patient also had progressive weakness in the lower and upper extremities 10 weeks prior to admission, requiring a wheelchair for mobilization as weakness worsened. The woman also experienced a tingling sensation that ascended from the lower to the upper extremities and had weakness that persisted until arrival at the emergency room.

Top Clinical Takeaways

  • The case underscores the challenge of diagnosing overlapping neurological conditions and the importance of a comprehensive diagnostic approach.
  • Treatment with a combination of corticosteroids and immunosuppressants proved effective in alleviating symptoms and improving the patient's clinical condition.
  • This study highlights the need for further research and understanding of the complex interplay between CIDP and NMOSD to optimize patient management strategies.

READ MORE: 2021 European Guidelines Improve Diagnosis and Treatment of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Variants

The patient had recurrent episodes of vertigo 1 year before admission and weakness in the right extremities as well as blurry vision 8 months before admission, which resolved after 2 weeks. Investigators observed that ischemic and hemorrhagic stroke were excluded in the head CT scan and further MRI revealed a multifocal paraventricular lesion. The authors noted that as the patient experienced symptoms of weakness in the extremities and tingling 6 weeks before admission, she was treated with methylprednisolone after being suspected of having an autoimmune disease. Following treatment, the patient had significant improvement in weakness in extremities despite the tingling sensation remaining.

The authors recorded visual acuities down to 1/60 for both eyes, but neurological examination showed normal ocular movements and no nystagmus. Furthermore, investigators observed lower motor neuron weakness in the patient, showing a Medical Research Council score of 3 throughout the left and right extremities, and paresthesia in the glove and stocking distribution. The researchers also noted that the woman had a coordination disorder.

In the screening process, the authors performed a CT scan and lumbosacral radiography, reporting no abnormalities for the patient. Continuing the investigation of the suspicion of peripheral lesion and blurry vision, the authors reported a bilateral pre-chiasmal lesion and somatosensory evoked potential, which they noted indicated a lesion between the medulla and cerebral cortex. Additional MRI examination revealed new hyperintense lesions in both the paraventricular and brainstem compared with the previous MRI scans. Investigators then reported a diagnosis of NMOSD in the patient because of the recurrence of blurry vision and positive aquaporin-4 even though the MRI findings were atypical of the autoimmune disorder.

In the spinal cord of the patients, the whole-spine MRI scan displayed no active lesions. Researchers then questioned the clinical manifestation of ascending weakness and hypothesized that the occurrence of a pathological process was in the peripheral nerves of the patient. Investigators reported that the patient’s electrolytes, serum creatinine, and complete blood count were all in normal ranges. Notably, findings from the cerebrospinal fluid showed albumino-cytological dissociation of an increasing protein (84 mg/dL; normal 15–40 mg/dL) without increasing the number of cells (1/µL; normal 0–5/µL). Thus, the authors noted that this finding supports the diagnosis of CIDP for the woman, as previously reported.2

Furthermore, authors performed a nerve conduction study which revealed reduced compound muscle action potentials of bilateral fibular nerve (1.2 mV and 1.4 mV in the left and right nerves, respectively; reference > 2.5 mV), right median nerve (4.1 mV; reference > 5 mV), and sensory demyelinating neuropathy of left sural nerve (5.5 ms; reference < 4.0 ms). Investigators noted that the right fibular motor velocity (23 m/s; reference > 40 m/s) and the left median motor velocity (23 m/s; reference > 50 m/s) was also significantly reduced in the patient. In addition, authors reported prolonged F-wave latencies in the left and right fibular nerves (58 ms and 64 ms, respectively; reference < 46 ms) and left and right median nerve (31 ms and 35 ms, respectively; reference < 28 ms). Overall, the findings gathered in the nerve conduction study suggested a diagnosis of CIDP, confirming the previous report.3

REFERENCES
1. Hamidi BL, Mirawati DK, Rahayu RF, Prabaningtyas HR, Hafizhan M, Putra SE. Chronic inflammatory demyelinating polyradiculoneuropathy associated with neuromyelitis optica spectrum disorder: A rare case report. Prev Med Rep. 2024;42:102702. Published 2024 Apr 27. doi:10.1016/j.pmedr.2024.102702
2. Koike H, Katsuno M. Pathophysiology of Chronic Inflammatory Demyelinating Polyneuropathy: Insights into Classification and Therapeutic Strategy. Neurol Ther. 2020;9(2):213-227. doi:10.1007/s40120-020-00190-8
3. Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision [published correction appears in Eur J Neurol. 2022 Apr;29(4):1288]. Eur J Neurol. 2021;28(11):3556-3583. doi:10.1111/ene.14959
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