Acute‐onset chronic inflammatory demyelinating polyneuropathy with cranial nerve involvement, dysautonomia, respiratory failure, and autoantibodies

Abstract

We examined a 27-year-old woman who developed rapidly progressive quadriplegia and acute respiratory failure that required mechanical ventilation in the intensive care unit. It was unclear whether this was a presentation of Guillain-Barré syndrome (GBS) or acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP). Remarkable features included multiple cranial nerve involvement, respiratory failure, dysautonomia, and skin manifestations. Several autoantibodies were elevated, including antinuclear (ANA), anticardiolipin (aCL), thyroid, and calcium-sensing receptor (CaSR) autoantibodies. The patient was initially diagnosed with GBS and treated with intravenous immunoglobulin (IVIg). After almost complete recovery, relapse with quadriplegia and respiratory failure was observed 12 weeks after motor symptom onset. She then received IVIg and steroid pulse therapy followed by maintenance oral methylprednisolone and plasma exchange. She recovered completely 4 months after the relapse. The further clinical and serological course was consistent with systemic lupus erythematosus (SLE)-associated CIDP. Herein we evaluate the association between A-CIDP and some biological markers of autoimmunity.

Risk of hematoma following needle electromyography of the paraspinal muscles

“…This subtype of CIDP is observed in up to 16% of human CIDP patients who present in their first episode, while they show clinical and electrophysiological abnormalities similar to GBS. [21][22][23][24][25] Longitudinal observations have occasionally identified ACIP-like cases in dogs, which differ from the classical form by evidence of remyelination in nerve biopsies, good response to immunosuppressive therapy, and progressive or recurrent clinical course. 2,6 The suggested diagnosis in these cases was CIDP.…”

Chronic inflammatory demyelinating polyneuropathy: diagnosis and management

“…Although onset is defined as at least over 8 weeks, CIDP may present acutely as Guillain-Barré syndrome (acute-onset CIDP), with an eventual progressive or relapsing chronic course [40,41]. This must be distinguished from treatment-related fluctuations of Guillain-Barré syndrome [42].…”

Neuropsychiatric Lupus in clinical practice

“…Forms of chronic inflammatory demyelinating polyneuropathy can present before or at the onset of another clinical manifestations of SLE 53 . Acute inflammatory demyelinating polyneuropathy, an ascending motor radiculoneuropathy that resembles Guillain-Barré syndrome clinically and electrodiagnostically, is a relatively rare condition, affecting up to 1% of the lupus patients 54 .…”