Case Report and Review Study Reveals Cranial Neuropathies in the Acute Phase of Chronic Inflammatory Demyelinating Polyneuropathy

A case report of a 28-year-old woman and a review study newly published in the Journal of Clinical Neuromuscular Disease showed that one-fourth of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and cranial nerve (CN) neuropathy had CN involvement occur in the acute phase. These results suggest the need to consider CIDP among differential diagnoses of patients with CN involvement and polyneuropathies which may help clinicians counsel patients regarding potential redirection of diagnosis and long-term treatment.¹

In the case report portion of the study, investigators identified a 28-year-old woman at the Penn State Hershey Medical Center who presented with progressive weakness and had repeated brain MRI that was suggestive of multiple CN enhancement. Based on the clinical history of symptoms’ fluctuating progression and evidence of acquired sensorimotor demyelinating polyneuropathy, clinicians made the diagnosis of CIDP. The patient underwent plasmapheresis and was discharged with daily prednisone 30 mg and mycophenolate mofetil 500 mg twice daily, which resulted in significant, durable improvement of her symptoms.

“Our case report and review of the literature suggested that among patients with CIDP and CN involvement, 25% present their CN symptoms within the acute phase. Historically patients with neuropathy and CN palsy involvement in the early phase were primarily diagnosed as [Guillain–Barré Syndrome],” lead author Mansoureh Mamarabadi, MD, assistant professor in the department of neurology at Penn State University, and colleagues wrote.¹ “As GBS and CIDP share many clinical characteristics, but vary in their response to different therapies, considering A-CIDP among differential diagnoses for patients with acute CN palsy alongside polyneuropathy is crucial and guides ongoing surveillance and treatment.”

In addition to the case presentation, investigators conducted a literature review of 59 patients with CIDP who showed symptoms of CN impairment with available patient-level data.²Authors presented findings as a proportion of patients with positive results—N (%) and had continuous variables with nonparametric distributions presented with a median and interquartile [Q1–Q3]. Furthermore, researchers performed a subgroup analysis on the timing of CN involvement. Investigators noted that instances where CN palsy manifested in the onset of the disease were defined as acute-phase presentations. Inversely, cases of CN involvement reported in subsequent relapses were identified as delayed presentations.

Top Clinical Takeaways

One-fourth of patients who had CIDP with cranial nerve involvement show symptoms in the acute phase, which challenges conventional diagnosis patterns.
Acute-phase cranial nerve involvement in patients with CIDP often leads to plasmapheresis treatment, with a higher frequency compared to those with delayed presentations.
There is a lack of universal diagnostic criteria for CIDP when cranial nerves are involved, creating the potential for misdiagnosis with other neuropathies like Guillain–Barré Syndrome.

“In this review, we attempted to present the associated biomarker panel reported in patients with CIDP and CN involvement. Our findings demonstrate marked heterogeneity among the reports in what testing they performed. This may reflect shifting practice over time, the absence of universal guidelines, and the unavailability of much of the more recent immunologic testing given that only half of the studies reported in our review were published after 2000,” Mamarabadi et al noted.¹

In the literature review, authors reported that in 10 out of 43 patients (23.3%), CN impairment was present in the acute phase of polyneuropathy and it took a median of 7.7 years [Q1-Q3; 3-13] for other patients to present with CN palsy. Researchers noted that in sensitivity analysis, there was no difference in patients with acute-phase presentation of CN symptoms (N = 11) compared with those with delayed CN palsy (N = 33) in terms of demographics, patterns of CN involvement, associated diminished sensorimotor results, or relapse. Despite this finding, investigators observed that patients with acute presentation of CN palsy experienced plasmapheresis approximately 4 times more compared with those who had delayed CN presentations (45.5% vs. 12.1%; P = .02, respectively).

“As discussed in the body of the literature, despite multiple reports of CN palsy in patients with CIDP, diagnostic criteria of CIDP lack any insight into CN involvement,” Mamarabadi et al wrote.¹ “The introduction of CN involvement in CIDP can prevent misdiagnosis of this pathology with other neuropathies such as GBS and diabetic neuropathies. In addition, there is no consensus on biomarkers to support the clinical findings or management of choice.”