| 제목 |
Juvenile onset relapsing Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Case Report |
| 소속 |
Korea University College of Medicine, Department of Physical Medicine and Rehabilitation1, Korea University College of Medicine, Department of Pediatrics2 |
| 저자 |
Jei Hak Myung1*, Soon Woo Kwon1, Jung Hye Byeon2, Sung Bom Pyun1† |
Abstract
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is symmetric, motor-dominant, and sensory neuropathy that results in both proximal and distal limb muscle weakness, which usually develops over an 8-week period. It is a rare chronic neuropathy, with its prevalence ranging from 4.8 to 8.9 cases per 100,000 persons and children being estimated at 0.5 cases per 100,000 persons. According to some previous cases, juvenile-onset CIDP, has a more rapid onset, relapsing-remitting progress, but has a more favorable longterm outcome than adult-onset CIDP. Because of its rarity, little is known about juvenile-onset CIDP, and few cases were reported in Korea.
A previously healthy 12-year-old girl presented with a 6-month history of progressive and fluctuating bilateral upper and lower extremities weakness and gait disturbance. Clinical examination revealed the symmetrical motor weakness of Medical Research Council grade 4 & 5 in the proximal & distal upper and 3 & 4 in the proximal & distal lower extremities. Sensory ataxia, hypoesthesia and the decreased deep tendon reflexes of bilateral lower extremities were also noted.
In the initial nerve conduction study (NCS), the compound muscle action potentials (CMAPs) were of prolonged latencies with temporal dispersion (Fig. 1.), low amplitudes, and decreased conduction velocities in all the tested nerves. The sensory nerve action potentials (SNAPs) were unobtainable in all the examined nerves. In the initial needle electromyography (EMG), fibrillation potentials and positive sharp waves were noted in all the examined muscles. Polyphasic motor unit action potentials with reduced recruitment patterns were also noted. All of these findings were compatible with the criterion of CIDP (Table 1). Moreover, the high level of protein in the cerebrospinal fluid supported the diagnosis. Treatment was initiated with the combination of intravenous immunoglobulin (IVIG) therapy and steroid pulse therapy. Second NCS/EMG after the first-line treatment revealed somewhat improved results in CMAPs but not in SNAPs. Clinically, she showed nearly normal strength in both upper and lower extremities.
Nevertheless, continuous re-aggravation of motor weakness happened although she was treated with repetitive IVIG, plasmapheresis, steroid pulse and immunosuppressive therapies with several admissions. Between relapses, complete recovery was not seen both clinical and in EMG/NCS. Serial NCS revealed continuously improving/deteriorating results in distal motor latencies with the evidence of demyelination remaining (Fig. 2).
Conclusion
The disease course of juvenile-onset CIDP is more fluctuating than the typical CIDP in adults. Follow up of electrodiagnostic study is useful for evaluation of relapsing clinical course and response after treatment. |
|
Table 1. Initial (6 months after onset) Nerve Conduction Study and Needle Electromyography // 주석: *: Abnormal values, NR: no response, IIA: increased insertional activities, APB: Abductor pollicis brevis, ADM: Abductor digiti minimi, EDB: extensor digitorum brevis, F&P: fibrillation potentials and positive sharp waves, TA: tibialis anterior, AH: abductor hallucis, Sup.peroneal: Superficial peroneal nerve
Figure 1. Waveform morphologies of the right ulnar (A) and the common fibular nerve (B) motor conduction studies showing prolonged latencies and prominent temporal dispersions.
Figure 2. Serial distal latencies of right (left) ulnar nerve and timetable of serial treatments
|
