Acute‐onset chronic inflammatory demyelinating polyneuropathy with cranial nerves and respiratory tract involvement: A case report

Abstract Sixteen percent of chronic inflammatory demyelinating polyneuropathy (CIDP) patients may present acutely like acute idiopathic demyelinating polyneuropathy (AIDP) the demyelinating form of GBS, developing in <8 weeks 2. This entity is classified as acute‐onset CIDP (A‐CIDP) which presents overlapping clinical and electrophysiological findings with GBS during early stages of disease, but followed with a chronic course beyond 2 months. Also, those who have three or more treatment‐related fluctuations (TRF) are included under this term. Distinguishing between acute‐onset chronic inflammatory demyelinating polyneuropathy (A‐CIDP) and acute idiopathic demyelinating polyneuropathy (AIDP) may be difficult during early stages but is crucial in order to guide treatment strategies without delay. These two forms share some overlapping clinical and electrophysiological findings, including some severe clinical features such as cranial nerve and respiratory tract involvement making the diagnosis of A‐CIDP more difficult.


Abstract
Sixteen percent of chronic inflammatory demyelinating polyneuropathy (CIDP) patients may present acutely like acute idiopathic demyelinating polyneuropathy (AIDP) the demyelinating form of GBS, developing in <8 weeks 2. This entity is classified as acute-onset CIDP (A-CIDP) which presents overlapping clinical and electrophysiological findings with GBS during early stages of disease, but followed with a chronic course beyond 2 months. Also, those who have three or more treatment-related fluctuations (TRF) are included under this term.
Distinguishing between acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) and acute idiopathic demyelinating polyneuropathy (AIDP) may be difficult during early stages but is crucial in order to guide treatment strategies without delay. These two forms share some overlapping clinical and electrophysiological findings, including some severe clinical features such as cranial nerve and respiratory tract involvement making the diagnosis of A-CIDP more difficult.

K E Y W O R D S
acute-onset chronic inflammatory demyelinating polyneuropathy, Bulbar and respiratory tract involvement, chronic inflammatory demyelinating polyneuropathy, Guillain-Barré syndrome 2200 |

| CASE REPORT
A 59-year-old man, without any particular medical history, presented at our department of neurology at the military hospital with a progressive clinical picture, 10 days following a flu, made of initial ascending paresthesia attending rapidly the upper limbs in <24 hours followed after 2 days by weakness in the lower then the upper limbs, and a discrete dysphagia with frequent choking to liquids. He was admitted to our hospital 8 days after the beginning of the symptoms on 11 April 2016. During hospitalization, he developed 3 days after his admission, respiratory weakness with worsening of the dysphagia, and facial diplegia on the 14th day of evolution. No autonomic dysfunction was noted. Physical examination showed flaccid tetraplegia graded 3 in upper limbs and 2 in lower limbs according to MRC scale, with absence of all reflexes, hypoesthesia in glove and stocking distribution, a positive Romberg sign, and facial diplegia. The EMG (Figures 1, 2 and 3) showed sensory and motor demyelinating polyradiculoneuropathy with decreased motor conduction velocities, prolonged distal latencies of ulnaris, medianus, tibilais, and peroneus, prolonged F-wave latencies in the four limbs, with decreased sensory conduction velocities of the right musculocutaneous and left ulnaris. CSF analysis highlighted albumino-cytological dissociation with 0 white cell and CSF protein level at 0.7 mg/dL. The patient was initially diagnosed as GBS and received an immunoglobulin therapy. His symptoms improved then progressively, and he was discharged on 25 April 2016, after the total regression of the respiratory signs and the improvement of the dysphagia, facial diplegia, and muscle weakness which was graded 4+ at his discharge. Two weeks later, he presented with worsening of the dysphagia and a sensory ataxia. He was readmitted to our department on 10 May 2016. Physical examination showed sensory ataxia, positive Romberg sign, loss of deep sensation at right, absence of all reflexes, with the same muscle strength grade 4 at his discharge, a weak nausea reflex, and a discrete facial diplegia. This was considered as a treatment-related fluctuations (TRF), and the patient received a second immunoglobulin therapy with a complete regression of the dysphagia and the sensory ataxia. After his discharge, he continued to improve with a clear regression of symptoms and recovery of all reflexes, only a discrete proximal muscle weakness persisted with few paresthesia of extremities which responded well to F I G U R E 2 Latency and amplitude study pregabalin. One year later, on April 2017, the patient manifested recurrent symptoms with flaccid tetraplegia graded 4, absence of all reflexes, and a positive Romberg sign noted at the physical examination. He was then differentially diagnosed with A-CIDP according to Ruts et al criteria. 2 He started taking methylprednisolone daily with a total recovery. The evolution was marked by steroid dependence, leading to the association of an immunosuppressive agent (azathioprine) with stability of the disease. He only kept a discrete proximal muscle weakness in lower limbs.

| DISCUSSION
Although CIDP is defined as a chronic progressive or relapsing condition that develops over at least 2 months, studies have shown that up to 16% of CIDP patients may present acutely like AIDP the demyelinating form of GBS, developing in <8 weeks. 2 This entity is classified as acute-onset CIDP (A-CIDP) which presents overlapping clinical and electrophysiological findings with GBS during early stages of disease, but followed with a chronic course beyond 2 months. Also, those who have three or more treatment-related fluctuations (TRF), during which secondary deterioration lasting <8 weeks is observed after treatment, 4 were included under this term according to Ruts et al. 2 Many studies 1,2,3,4 were conducted to find these distinctive clinical features between patients with AIDP and A-CIDP to help predict clinical behavior of patients with A-CIDP and guide treatment strategy and prognosis. All these studies demonstrated that A-CIDP was more frequent in men. The mean age at onset of symptoms was 43 years in Dionne et al study, 1 47 years in Ruts et al, 2 and 55.5 years in Alessandro et al study 4 closer to our patient who was 59 years old at the onset of symptoms. The infectious prodrome presented by our patient F I G U R E 3 F-wave study