Demyelinating disease

Abstract

Patients with chronic inflammatory demyelinating polyneuropathy (CIDP) experience proximal- and distal-extremity weakness, sensory loss, and often hyporeflexia or areflexia. CIDP is associated with a variety of concomitant medical illnesses, which often manifest weeks before the onset of muscle weakness and paresis. We describe what we believe is the first reported case of an association between CIDP and recurrent acute adenotonsillitis, which we observed in an 11-year-old girl. Following adenotonsillectomy, the patient's CIDP went into remission and her overall physical condition improved with physiotherapy.

Introduction

Chronic inflammatory demyelinating polyneuropathy (CIDP) is similar to Guillain-Barre syndrome in that it causes proximal- and distal-extremity weakness, sensory loss, and often hyporeflexia or areflexia. (1) Among the many treatments for CIDP are plasmapheresis, intravenous immunoglobulin (IV Ig), corticosteroids, and other immunomodulators such as azathioprine, cyclosporine, and cyclophosphamide. (1,2) The association of CIDP with a concomitant medical illness has been reported quite frequently in the literature. (1-4) In this article, we describe what we believe is the first reported case of an association between CIDP and recurrent acute adenotonsillitis. Following adenotonsillectomy, the patient's CIDP went into remission and her overall physical condition improved.

Case report

An 11-year-old girl with CIDP was brought to us for evaluation. At the age of 4 years, she developed a generalized paralysis that necessitated hospital admission and prolonged ventilatory support. At that time, she was initially diagnosed with Guillain-Barre syndrome. However, following a prolonged recovery from this initial acute event and after frequent and intermittent relapses of weakness and paresis, the diagnosis of CIDP was established.

The patient later experienced two relapses, and she was treated with IV Ig. Treatment had increased the speed with which her weakness resolved, but she continued to experience relapses. Her episodes of weakness and paresis were generally preceded by fever or illness. Her most common systemic illness was recurrent acute adenotonsillitis, which had occurred approximately four times a year. Each episode of adenotonsillitis was followed in 2 to 6 weeks by increased weakness, primarily in her lower extremities, that necessitated the use of a wheelchair.

On physical examination, the patient had slightly enlarged tonsils for her age and adenoids that were 30% obstructive of the choanae. A standard adenotonsillectomy was performed. At the 1-year postoperative follow-up, both the patient and her family reported no episodes of sore throat or upper respiratory illness, no further relapses of acute paralysis, and an improvement in her baseline strength and agility with physiotherapy.

Discussion

Dyck et al coined the phrase chronic inflammatory demyelinating polyneuropathy (5) several years after they reported the first case series of 53 patients with this disease complex. (6) In 1989, Barohn et al (7) published diagnostic criteria, which were later revised by an ad hoc subcommittee of the American Academy of Neurology. (8)

CIDP is characterized by the presence of distal- and proximal-extremity weakness, more often in the lower extremities. Some patients are also afflicted with a sensory impairment, and patients frequently experience hyporeflexia or areflexia. (1) Although most patients have a slowly progressive form of the disease, a relapsing-remitting course has also been described. (1) In a recently published retrospective study, Bouchard et al found that three of 100 patients with CIDP initially had acute-onset Guillain-Barre syndrome; this was followed by a slow progression of CLDP in two of these patients and a relapsing-remitting course in the third. (4) Most patients who initially have Guillain-Barre syndrome are children, and children are more likely than adults to have a relapsing and quickly fluctuating form of the disease. (9)

Studies have also found that infectious events occur in 16 to 21% of patients during the weeks prior to the onset of neurologic manifestations. (3,4) There is a well-documented association between CIDP and a number of concurrent infections (e.g., human immunodeficiency virus infection and viral hepatitis) as well as systemic medical illnesses that alter the immune system (e.g., Charcot-Marie-Tooth disease, inflammatory bowel disease, lymphoma, and diabetes mellitus). (1-4) As is the case with Guillain-Barre syndrome, the pathophysiology of CIDP is believed to be of autoimmune origin. Histopathologic analysis of nerve biopsies often reveals demyelination, remyelination, and inflammation. (2)

The treatment options for CLDP are all centered on immunomodulation, and randomized, controlled trials have demonstrated the efficacy of plasmapheresis, IV Ig, corticosteroids, and other immunomodulators. (1,2) Currently, IV Ig and corticosteroid therapy are considered first-line treatments. (1,4,9) Hahn recently proposed that IV Ig is the treatment of choice in children because of its relative ease of administration and its relative lack of side effects. (9) Plasmapheresis and more aggressive immunomodulating medications (e.g., azathioprine, cyclosporine, and cyclophosphamide) are generally reserved for cases that are refractory to initial treatment or that relapse following treatment with a first-line modality. (1,4) Initial therapy with one of these modalities has been reported to be successful in alleviating symptoms in 39 to 95% of patients. (1-3) However, about 50% of those who initially respond to therapy will relapse. (2,3) In addition, only approximately one-third of all patients with CIDP will remai n symptom-free when all medications and treatments are discontinued. (1,2)

Our patient had been treated with IV Ig twice prior to her visit to our clinic. These treatments were effective in quickly resolving her acute weakness, but they were unable to bring about a complete remission of her CLDP. Recurrent adenotonsillitis was the associated event that preceded her disease exacerbations. The removal of her tonsils and adenoids brought about a prolonged remission of her CIDP and allowed for improvement in her strength and agility through physiotherapy. This case is a good example of the principle that treatment of the underlying concurrent illness will often bring about remission of or improvement in CIDP.

References

(1.) Saperstein DS. Katz JS, Amato AA, Barohn RJ. Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve 2001;24:311-24.

(2.) Barohn RJ, Saperstein DS. Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy. Semin Neurol 1998;18:49-61.

(3.) Gorson KC, Allam G, Ropper AH. Chronic inflammatory demyelinating polyneuropathy: Clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology 1997;48:321-8.

(4.) Bouchard C, Lacroix C. Plante V, et al. Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neurology 1999;52:498-503.

(5.) Dyck PJ, O'Brien PC, Oviatt KF, et al. Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol 1982;11:136-41.

(6.) Dyck PJ, Lais AC, Ohta M, et al. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 1975;50:621-37.

(7.) Barohn RJ, Kissel JT, Warmolts JR, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics. course, and recommendations for diagnostic criteria. Arch Neurol 1989;46:878-84.

(8.) Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. Neurology 1991;41:617-18.

(9.) Hahn AF. Treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulin. Neurology 1998;51:S16-S21.

From the Department of Otolaryngology--Head and Neck Surgery. Madigan Army Medical Center, Tacoma, Wash.

Reprint requests: Wayne J. Harsha, MD, Department of Otolaryngology--Head and Neck Surgery, Madigan Army Medical Center, Attn: MCHJ-SET, Tacoma, WA 98431-5000. Phone: (253) 9684420; fax: (253) 968-3154; e-mail: wayne.harsha@nw.amedd.army.mil

The opinions and assertions expressed herein are those of the authors and do not necessarily reflect the views of the U.S. Army or the Department of Defense.

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