Young Male with Acute-Onset Chronic Inflammatory Demyelinating Polyneuropathy (A-CIDP)
Year: 2024
Type: article
Abstract: Dear Editor, Chronic inflammatory demyelinating polyneuropathy (CIDP) has a progression over >8 weeks with a relapsing course. CIDP presents like acute inflammatory demyelinating polyneuropathy (AIDP) and is referred to as A-CIDP. Here we discuss a case 24-year-old male presenting a second episode with A-CIDP. Now he presented with tingling and numbness in the bilateral lower limb and upper limb more distally than proximal, slippage of chappal without knowledge, and imbalance while walking for 8 days. No palpable thickened nerve and no cranial nerve involvement were seen. Power at hip extension was 4/5 and on the rest of the joints 5/5. All superficial and deep tendon reflexes were absent. Pinprick, vibration sense was severely reduced till the thigh and above the elbow joint from distally. Joint position sense was diminished till the ankle joint, and the Romberg test was positive. There was a history of acute onset quadriparesis 2 years back that developed over 10 days. The nerve conduction study (NCS) was suggestive of severe generalized symmetric predominantly motor polyneuropathy affecting the upper and lower limbs. Cerebrospinal fluid (CSF): Protein-357.2 mg/dl, glucose 62 mg/dl (corresponding blood sugar level 110 mg/dl), two leucocytes. The patient received intravenous immunoglobulin and recovered completely within 1 month. During the second episode, CSF:– Protein- 374 mg/dl, glucose-57 mg/dl (corresponding blood sugar level 101 mg/dl), four leucocytes. NCS was suggestive of severe axonal sensory-motor polyneuropathy involving all four limbs. ANA/ANCA-profile/Sr SPEP was negative. Positron emission tomography scan was normal. Nerve biopsy (Right sural nerve) Expanded nerve funicles secondary to the prominent formation of bands of Bungner (Schwann cell hyperplasia) and early onion bulbs. Kulchitsky stain showed severe non-uniform loss of myelinated fibers (large>small) with many thinly myelinated fibers suggestive of chronic demyelinating neuropathy [Figure 1]. Patient received steroid and plasmapheresis. He had a significant improvement in symptoms. Now he is on azathioprine and is under regular follow-up with no further relapse seen since the last second episode 12 months back.Figure 1: Right sural nerve biopsyAIDP presentation is seen in up to 16% of patients with CIDP as shown in some studies.[1] A-CIDP was more common in men as seen in our case also. The mean age group for the presentation is the 4th and 5th decades.[2] Age of our patient’ was 24 years. Cranial nerve involvement is more in the case of GBS as compared to A-CIDP.[2] CSF examination showed albumin cytological dissociation which was similar to prior study.[3] When GBS deteriorates after 8–9 weeks one should suspect A-CIDP.[2] In this case, the patient had a second episode of A-CIDP after 2 years with biopsy-proven CIDP. A-CIDP long-term immunosuppression.[4] Therefore, diagnosis A-CIDP 2 episodes 2 years apart, with NCS favoring CIDP as per criteria of European Academy of Neurology, Peripheral Nerve Society.[5] Nerve biopsy favors the diagnosis of CIDP. A-CIDP needs to be treated like CIDP with long-term immunosuppression. NCS and nerve biopsy can play a significant role. The prognosis of AIDP is much better than A-CIDP as A-CIDP has a relapsing course and requires close follow-up and long-term immunosuppression. Data availability statement Nil. Author contribution Dr. Pranit Khandait: Responsible for concept and design, drafting article; Dr. Shalesh Rohatgi and Dr. Satish Nirhale: done criticle revision and approval of final version. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Language: English
Subfield: Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4407834861