David Kiefer1, Uta Kiltz1, Jürgen Braun1.
Abstract
HISTORY: A 68-year-old woman presented with chronic back pain, a poor general condition, weight loss of 20 kg in the last 6 months and paretic hands. FINDINGS AND DIAGNOSIS: The patient had pareses of hand- and finger muscles on both sides with corresponding deficits proven by EMG. MRI revealed an intraspinal mass affecting the cervical and thoracic spine with myelon compression. Histologically IgG4-positive plasma cells were detected and IgG4-RD with spinal pseudotumor and resulting cervicothoracic myelopathy were diagnosed. THERAPY AND COURSE: After initiation of high-dose glucocorticoid therapy (100 mg i. v. over 7 days with reduction afterwards) and cyclophosphamide-pulse-therapy (initially 750 mg i. v., cumulative dose of 4500 mg in 6 months), the general condition and motor deficits improved.
CONCLUSIONS: The CNS are a rare manifestation of IgG4-RD. The tumor typically grows displacing, is best visualized on MRI or PET-CT, presenting clinically the corresponding pain and neurological deficits and shows characteristic histology. Steroids and, as in our case helpful, advanced immunosuppression can be a promising treatment option. © Georg Thieme Verlag KG Stuttgart · New York.
HISTORY: A 68-year-old woman presented with chronic back pain, a poor general condition, weight loss of 20 kg in the last 6 months and paretic hands. FINDINGS AND DIAGNOSIS: The patient had pareses of hand- and finger muscles on both sides with corresponding deficits proven by EMG. MRI revealed an intraspinal mass affecting the cervical and thoracic spine with myelon compression. Histologically IgG4-positive plasma cells were detected and IgG4-RD with spinal pseudotumor and resulting cervicothoracic myelopathy were diagnosed. THERAPY AND COURSE: After initiation of high-dose glucocorticoid therapy (100 mg i. v. over 7 days with reduction afterwards) and cyclophosphamide-pulse-therapy (initially 750 mg i. v., cumulative dose of 4500 mg in 6 months), the general condition and motor deficits improved.
CONCLUSIONS: The CNS are a rare manifestation of IgG4-RD. The tumor typically grows displacing, is best visualized on MRI or PET-CT, presenting clinically the corresponding pain and neurological deficits and shows characteristic histology. Steroids and, as in our case helpful, advanced immunosuppression can be a promising treatment option. © Georg Thieme Verlag KG Stuttgart · New York.
Entities:
Mesh：
Year: 2019
PMID： 31311046 DOI： 10.1055/a-0850-0600
Source DB: PubMed Journal: Dtsch Med Wochenschr ISSN： 0012-0472 Impact factor: 0.628