Document Detail

Selective immunoadsorption in neurologic complications of systemic lupus erythematosus
MedLine Citation:
PMID:  17342456     Owner:  NLM     Status:  MEDLINE    
Vasculitis of the nervous system is a rare cause of multifocal neurologic symptoms and may involve both the central and peripheral nervous systems. Typical symptoms include headache, encephalopathy with cognitive impairment and psychotic symptoms, epileptic seizures, and peripheral neuropathies. Here we report the case of a 71-year-old female presenting with Raynaud's syndrome and paresthesia of the feet. Several weeks later she was admitted to our hospital with a status epilepticus and complex partial seizures. On admission she had mild aphasia, distal paresis of the arms without sensory deficits, and disorientation with hallucinations. Cerebral MRI revealed small, multifocal infarctions in several arterial territories. Multiple cerebral artery stenoses were detected by ultrasound. Examination of the CSF was unremarkable. Serologic tests for autoimmune disorders detected Ro antibodies compatible with systemic lupus erythematosus or Sjögren's syndrome. A sural nerve biopsy revealed ischemic axonal neuropathy. During administration of i.v. methylprednisolone, the symptom progression stopped but dosages could not be tapered due to severe CNS symptoms (mental decline, disorientation, aphasia, hallucinations). Slow but sustained clinical improvement was achieved by immunoadsorption over 3 weeks followed by a combined high-dose immunosuppressive treatment with cyclophosphamide and prednisolone that paralleled a reduction in anti-Ro titers and normalization of cerebral blood flow velocities as detected by repeated transcranial Doppler sonography. Systemic vasculitis may present with multiple neurologic and psychiatric symptoms due to involvement of the central and peripheral nervous systems. After excluding systemic infection, immunosuppressive therapy should be started early. In our case a combination of high-dose methylprednisolone, immunoadsorption with elimination of Ro antibodies, and cyclophosphamide led to the patient's recovery.
S Harscher; S Rummler; P Oelzner; H-J Mentzel; M Brodhun; O W Witte; C Terborg; S Isenmann
Publication Detail:
Type:  Case Reports; English Abstract; Journal Article    
Journal Detail:
Title:  Der Nervenarzt     Volume:  78     ISSN:  0028-2804     ISO Abbreviation:  Nervenarzt     Publication Date:  2007 Apr 
Date Detail:
Created Date:  2007-04-16     Completed Date:  2007-08-08     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0400773     Medline TA:  Nervenarzt     Country:  Germany    
Other Details:
Languages:  ger     Pagination:  441-4     Citation Subset:  IM    
Klinik für Neurologie, Friedrich-Schiller-Universität, Erlanger Allee 101, 07740, Jena.
Vernacular Title:
Selektive Immunadsorption bei neurologischen Komplikationen eines systemischen Lupus erythematodes.
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MeSH Terms
Antibodies, Antinuclear / immunology*
Brain / drug effects,  immunology*
Immunosorbent Techniques
Immunosuppressive Agents / administration & dosage
Lupus Vasculitis, Central Nervous System / complications,  diagnosis*,  drug therapy,  immunology*
Nervous System Diseases / diagnosis*,  drug therapy,  etiology,  immunology*
Treatment Outcome
Reg. No./Substance:
0/Antibodies, Antinuclear; 0/Immunosuppressive Agents; 0/SS-A antibodies

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