Chronic inflammatory demyelinating polyneuropathy

Abstract

We report the clinical and EMG details of 67 consecutive patients with strictly defined chronic inflammatory demyelinating polyneuropathy (CIDP) during a 4-year period and compare responses to treatment in patients with idiopathic CIDP (CIDP-I) and CIDP with monoclonal gammopathy of uncertain significance (CIDP-MGUS). Patients were examined an average of 28 months after first symptoms. There were several variant presentations that still conformed to the clinical and electrophysiologic definitions of CIDP, including a pure motor syndrome (10%), sensory ataxic variant (12%), mononeuritis multiplex pattern (9%), paraparetic pattern (4%), and relapsing acute Guillain-Barré syndrome (16%). Pain was more frequent than in previous studies (42%). Conduction block was the commonest EMG abnormality (detected in at least one nerve in 73% of patients), but only 31% had a pure demyelinating neuropathy and the majority had some degree of axonal change. Patients with CIDP-MGUS had less severe weakness, greater imbalance, leg ataxia, vibration loss in the hands, and absent median and ulnar sensory potentials, but were as likely as CIDP-I patients to respond to plasma exchange. Seventeen of 44 patients (39%) with idiopathic CIDP improved for at least 2 months with an initial therapy. Although the response rates among plasma exchange, IVIG, and steroids were similar, functional improvement (Rankin score) was greatest with plasma exchange. Of 26 patients who failed to respond to an initial therapy, 9 (35%) benefited from an alternative treatment, and of the 11 who required a third modality 3 (27%) improved. Overall, 66% responded to one of the three main therapies for CIDP.

Neuro-ophthalmological Complications of Chronic Inflammatory Demyelinating Polyradiculoneuropathy

“…The opening pressure in these papilloedemanegative high-CSF protein cases has not been stated though. 16,17,19,20 The occurrence of papilloedema with hypertrophic nerve enlargement in CIDP has not previously been reported, even though it is a marker of active disease. This may be because most of the cases series of CIDP with papilloedema were in the pre-MRI era.…”

Evaluation of Human Immunoglobulin Effectiveness in Patients With Sensorymotor Polyneuropathy Associated With Monoclonal Gammapathy of Undetermined Significance

“…По вопроснику «Pain detect» сред-ний показатель после курса лечения составил 22 бал-ла, что свидетельствовало об уменьшении нейропа-тической боли. Согласно литературным данным терапия ПП с MGUS проводится по стандартным схемам лече-ния ХВДП [21,22,23], несмотря на наличие парапро-теинемии. Однако отмечено, что кортикостероиды эффективны только в 30% случаев ПП с MGUS.…”

Chronic Inflammatory Demyelinating Polyneuropathy

“…Weakness is typically symmetric and characteristically involves proximal and distal muscles [9], occasionally affecting face or neck flexor muscles, and typically sparing of extraocular muscles. Except in pure motor presentation, which affects up to 10 % of cases [10], sensory symptoms include numbness, tingling, gait imbalance, and at times painful parasthesias [10][11][12]. The sensory variant affects 35 % of cases while classic sensorimotor presentation occurs in 51 % [10].…”