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Paraneoplastic fasciitis–panniculitis syndrome: a neurological point of view

Nature Clinical Practice Neurology volume 5, pages 113–117 (2009)Cite this article

Abstract

Background A 54-year-old woman who had undergone gastrectomy to treat gastric adenocarcinoma 5 years previously and had since been in remission presented to a neuromuscular clinic complaining of stiffening and painful spasms of the legs and abdomen. Repeat pan-CT scans and gastric biopsy confirmed the recurrence of poorly differentiated signet ring gastric adenocarcinoma. Her symptoms improved remarkably on initiation of chemotherapy and worsened on discontinuation of chemotherapy.

Investigations Neurological examination, MRI of the abdomen and lower extremities, whole-body fluorodeoxyglucose PET, pan-CT scan, electromyography, muscle biopsy, upper gastrointestinal tract radiography, esophagogastroduodenoscopy, and immunophenotyping (paraneoplastic, rheumatological and autoimmune diseases panels).

Diagnosis Paraneoplastic fasciitis–panniculitis syndrome associated with the recurrence of poorly differentiated signet ring gastric adenocarcinoma.

Management Chemotherapy of recurrent gastric adenocarcinoma and symptomatic management of painful spasms.

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Figure 1: Bilateral MRI scans of the patient's thighs and legs.
Figure 2: FDG–PET/CT images of the patient's abdomen and thighs.
Figure 3: Biopsy sample from the patient's thigh muscle.

References

  1. Naschitz JE et al. (1992) The fasciitis-panniculitis syndrome: clinical spectrum and response to cimetidine. Semin Arthritis Rheum 21: 211–220

    Article  CAS  Google Scholar 

  2. Shulman LE (1975) Diffuse fasciitis with eosinophilia: a new syndrome? Trans Assoc Am Physicians 88: 70–86

    CAS  PubMed  Google Scholar 

  3. Naschitz JE et al. (1994) Cancer-associated fasciitis panniculitis. Cancer 73: 231–235

    Article  CAS  Google Scholar 

  4. Naschitz JE et al. (1996) The fasciitis–panniculitis syndromes: clinical and pathologic features. Medicine (Baltimore) 75: 6–16

    Article  CAS  Google Scholar 

  5. Ido T et al. (2006) Fasciitis-panniculitis syndrome and advanced gastric adenocarcinoma in association with antibodies to single-stranded DNA. Br J Dermatol 155: 640–641

    Article  CAS  Google Scholar 

  6. Graus F et al. (2004) Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 75: 1135–1140

    Article  CAS  Google Scholar 

  7. Dalakas MC et al. (2000) The clinical spectrum of anti-GAD antibody-positive patients with stiff-person syndrome. Neurology 55: 1531–1535

    Article  CAS  Google Scholar 

  8. Pittock SJ et al. (2005) Amphiphysin autoimmunity: paraneoplastic accompaniments. Ann Neurol 58: 96–107

    Article  Google Scholar 

  9. Risk WS et al. (1981) Chronic tetanus: clinical report and histochemistry of muscle. Muscle Nerve 4: 363–366

    Article  CAS  Google Scholar 

  10. Harding AE (1981) Hereditary pure spastic paraplegia: a clinical and genetic study of 22 families. J Neurol Neurosurg Psychiatry 44: 871–883

    Article  CAS  Google Scholar 

  11. Rowland LP (1985) Cramps, spasms and muscle stiffness [French]. Rev Neurol (Paris) 141: 261–273

    CAS  Google Scholar 

  12. Isaacs H (1961) A syndrome of continuous muscle-fibre activity. J Neurol Neurosurg Psychiatry 24: 319–325

    Article  CAS  Google Scholar 

  13. Lee EK et al. (1998) Morvan's fibrillary chorea: a paraneoplastic manifestation of thymoma. J Neurol Neurosurg Psychiatry 65: 857–862

    Article  CAS  Google Scholar 

  14. Derk CT and Jimenez SA (2003) Systemic sclerosis: current views of its pathogenesis. Autoimmun Rev 2: 181–191

    Article  CAS  Google Scholar 

  15. Cowper SE et al. (2000) Scleromyxoedema-like cutaneous diseases in renal-dialysis patients. Lancet 356: 1000–1001

    Article  CAS  Google Scholar 

  16. Lakhanpal S et al. (1988) Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum 17: 221–231

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. P Dinakar is pursuing a Harvard Combined Pain Fellowship at the Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.,

    Pradeep Dinakar

  2. A Höke is an Associate Professor of Neurology and Neuroscience and the Director of the Neuromuscular Division in the Department of Neurology at Johns Hopkins Hospital, Baltimore, MD.,

    Ahmet Höke

Authors
  1. Pradeep Dinakar
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  2. Ahmet Höke
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Correspondence to Pradeep Dinakar.

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The authors declare no competing financial interests.

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Dinakar, P., Höke, A. Paraneoplastic fasciitis–panniculitis syndrome: a neurological point of view. Nat Rev Neurol 5, 113–117 (2009). https://doi.org/10.1038/ncpneuro0999

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  • DOI: https://doi.org/10.1038/ncpneuro0999

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