[Palpable orbital subcutaneous masses in chronic inflammatory demyelinating polyneuropathy. MRI and neurophysiological study of multiple peripheral nerve swelling].
We report a 46-year-old woman with chronic inflammatory demyelinating polyneuropathy (CIDP) in whom swelling of the first branch of the trigeminal nerves in the bilateral orbits were observed as subcutaneous masses in the upper eyelids. The disease developed when the patient was 33 years old, and weakness of the four limbs, double vision, unilateral hypoglossal neuroparalysis, and unilateral facial paralysis frequently occurred during the course of the illness. On nerve conduction studies, conduction block was detected in the motor nerves. Steroid therapy and immunoglobulin treatment improved the symptoms. At 43 years old, subcutaneous phymas were noted in the bilateral upper eyelids, and fat-suppressed MRI detected the phymas localized along the upper region of the superior straight muscle in the bilateral orbits, and the muscles were slightly compressed downward. The masses branched in the orbits, and were diagnosed as nerve swelling of the supraorbital nerve, the first branch of the trigeminal nerve. Fat-suppressed MRI also identified nerve swelling of the extracranial maxillary and mandibular nerves. However, the patient had no subjective sensory disturbance in the trigeminal nerve region. Blink reflex did not induce R1 and R2 exhibited low amplitude and delayed latency. MRI confirmed asymmetric nerve swelling in the regions of the bilateral median nerves with motor nerve conduction block. Lumbar MRI detected nerve swelling in the peripheral nerves distant from the lumbar ganglion. MRI detected no nerve swelling in the arachnoid space such as the cervical cord, thoracic cord, or cauda equina. Aggravation of CIDP was treated with steroids and immunoglobulin. Diplopia occasionally appeared, but was not consistent with aggravation of CIDP. The bilateral supraorbital nerves remained unchanged for three years on MRI.
Department of Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry.
Toshiyuki
T
Yamamoto
Yasushi
Y
Oya
Osamu
O
Igarashi
Chizuko
C
Toyoda
Masafumi
M
Ogawa
Mitsuru
M
Kawai
D002908:Chronic Disease; D003711:Demyelinating Diseases / Q000473:pathology; D005143:Eyelids / Q000294:innervation*; D005260:Female; D006801:Humans; D008875:Middle Aged; D010525:Peripheral Nerves / Q000473:pathology*; D011115:Polyneuropathies / Q000473:pathology*