[A 24-year-old man presenting Garcin syndrome and paraplegia].
We report a 24-year-old man who presented unilateral multiple cranial nerve involvements followed by progressive paraplegia. The patient expired after developing DIC and pneumonia. Post-mortem examination revealed Ewing's sarcoma originated in the pubic bone with extensive metastases including the clivus which was responsible for his cranial nerve lesions. The patient was well until 24 years of age when he noted an onset of pain and a mass in the pubic region. The histology of the biopsy specimen of the tumor suggested Ewing's sarcoma. He was treated with chemotherapy and local radiation. A year after, he noted an onset of nuchal pain, difficulty in tongue movement, dysarthria, deafness in the left ear, and diplopia. On admission to our hospital in July 1990, neurological examination revealed an alert and intelligent Japanese male in no acute distress. The olfactory to the trigeminal nerves appeared intact. He showed complete abducens nerve palsy, facial weakness, mild deafness, and weakness of the soft palate, the sternocleidomastoid muscle and the tongue, all on the left side. The remainder of the neurological examination was unremarkable except for dysesthesia along the left C8 and Th1 dermatoms. Radiological examination revealed a 10 x 10 cm sclerotic mass in the public bone and a high signal mass lesion between the clivus and the pons in the T2-weighted MRI. His clinical course was complicated by acute paraplegia with anesthesia below the Th4 dermatom, DIC, and respiratory distress due to plural effusion. Post-mortem examination revealed a necrotic and hemorrhagic tumor in the pubic bone. The histology was consistent with Ewing's sarcoma.(ABSTRACT TRUNCATED AT 250 WORDS)
Hayashi
A
A
Departments of Neurology, Juntendo University School of Medicine, Tokyo, Japan.
Kogahara
K
K
Kusunose
K
K
Akiyama
K
K
Matsumoto
T
T
Mori
H
H
Kuwabara
N
N
Kondo
T
T
Sato
T
T
Yamauchi
Y
Y
D000328:Adult; D001859:Bone Neoplasms / Q000473:pathology*; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D009362:Neoplasm Metastasis; D010264:Paraplegia / Q000209:etiology; D011630:Pubic Bone / Q000473:pathology; D012512:Sarcoma, Ewing / Q000473:pathology*