Neurosarcoidosis without systemic sarcoidosis

  • Norbert Sommer
  • Michael Weller
  • Dirk Petersen
  • Horst Wiethölter
  • Johannes Dichgans
Original Articles


Neurosarcoidosis is a well-recognised complication of systemic sarcoidosis but diagnosis may be difficult if there is no clear evidence of an extracerebral manifestation of the disease. We present the case of a 42-year-old woman with clinical features characteristic of cerebral sarcoidosis including tetraparesis, diabetes insipidus, diencephalic hyperphagia, personality changes, and memory loss. Diagnosis was supported by cerebrospinal fluid (CSF) findings and magnetic resonance imaging (MRI): CSF showed mild lymphocytic pleocytosis, intrathecal production of IgG without oligoclonal bands, and a raised level of lysozyme. MRI revealed multiple contrast-enhanced granulomas at the base of the brain with partial involvement of diencephalic and mesencephalic structures and parts of the spinal cord. There was no evidence of systemic manifestation of sarcoidosis. Administration of corticosteroids led to improvement of the symptoms.

Key words

Sarcoidosis Cerebrospinal fluid Lysozyme Magnetic resonance imaging 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Allen KS, Glickstein M, Arger, PH, Bilaniuk L, Levy DW (1988) Cryptococcosis associated with sarcoidosis: CT and MR findings. J Comput Assist Tomogr 12:420–422PubMedGoogle Scholar
  2. 2.
    Borucki SJ, Nguyen BV, Ladoulis CT, McKendall RR (1989) Cerebrospinal fluid immunoglobulin abnormalities in neurosarcoidosis. Arch Neurol 46:270–273PubMedGoogle Scholar
  3. 3.
    Capp MP, Strautman PR, Kricun ME, Modic MT, Moss AA, Webb WR, Wodd BP (1990) Image interpretation session. Case 9. Radiographics 10:149–151Google Scholar
  4. 4.
    Cooper SD, Brady MB, Williams JP, Pilgreen KL, Harp DL, Weissmann JR (1988) Neurosarcoidosis: evaluation using computed tomography and magnetic resonance imaging. J Comput Tomogr 12:96–99CrossRefPubMedGoogle Scholar
  5. 5.
    Delaney P (1977) Neurologic manifestation in sarcoidosis: review of the literature, with a report of 23 cases. Ann Intern Med 87:336–354PubMedGoogle Scholar
  6. 6.
    Hayes WS, Sherman JL, Stern BJ, Citrin CM, Pulaski PD (1987) MR and CT evaluation of intracranial sarcoidosis. Am J Roentgenol 149:1043–1049Google Scholar
  7. 7.
    Kinnman J, Link H (1984) Intrathecal production of oligoclonal IgM and IgG in CNS sarcoidosis. Acta Neurol Scand 69: 97–106PubMedGoogle Scholar
  8. 8.
    Lawrence EC, Brousseau KP, Berger MB, Kurman CC, Marcon L, Nelson DL (1988) Elevated concentrations of soluble interleukin-2 receptors in serum samples and bronchoalveolar lavage fluids in active sarcoidosis. Am Rev Respir Dis 137: 759–746PubMedGoogle Scholar
  9. 9.
    McHugh PR (1986) Satiety and its disorders. In: Asbury AK, McKhann GM, McDonald WI (eds) Disorders of the nervous systems. Heinemann, London, pp 643–650Google Scholar
  10. 10.
    Mende D, Suchenwirth RMA (1990) Neurosarkoidose vergleichende Analyse des klinischen Profils anhand von 537 Beobachtungen aus der Weltliteratur bis 1963 und von 1976–1988. Fortschr Neurol Psychiatr 58:7–18PubMedGoogle Scholar
  11. 11.
    Miller DH, Kendall BE, Barter S, Johnson G, MacManus DG, Logsdail SJ, Ormerod IEC, McDonald WI (1988) Magnetic resonance imaging in central nervous system sarcoidosis. Neurology 38:378–383PubMedGoogle Scholar
  12. 12.
    Mitchell JD, Yap PL, Milne LA, Lachmann PJ, Pentland B (1985) Immunological studies on the cerebrospinal fluid in neurological sarcoidosis. J Neuroimmunol 7:249–253CrossRefPubMedGoogle Scholar
  13. 13.
    Nakao K, Noma K, Sato B, Yano S, Yamamura Y, Tachibana T (1978) Serum prolactin levels in 80 patients with sarcoidosis. Eur J Clin Invest 8:37–40PubMedGoogle Scholar
  14. 14.
    Nesbit GM, Miller GM, Baker HL, Ebersold MJ, Scheithauer BW (1989) Spinal cord sarcoidosis: a new finding at MR imaging with Gd-DTPA enhancement. Radiology 173:839–843PubMedGoogle Scholar
  15. 15.
    Oksanen V (1986) Neurosarcoidosis: clinical presentations and course in 50 patients. Acta Neurol Scand 73:283–290PubMedGoogle Scholar
  16. 16.
    Oksanen V, Fyhrquist F, Grönhagen-Riska C, Somer H (1985) CSF angiotensin-converting enzyme in neurosarcoidosis. Lancet I:1050–1051CrossRefGoogle Scholar
  17. 17.
    Oksanen V, Fyhrquist F, Somer H, Grönhagen-Riska C (1985) Angiotensin converting enyme in cerebrospinal fluid: a new assay. Neurology 35:1220–1223PubMedGoogle Scholar
  18. 18.
    Oksanen V, Grönhagen-Riska C, Tikanoja S, Somer H, Fyhrquist F (1986) Cerebrospinal fluid lysozyme and β2-microglobulin in neurosarcoidosis. J Neurol 73:79–87Google Scholar
  19. 19.
    Schlegel U (1987) Neurosarkoidose: Diagnostik und Therapie. Fortschr Neurol Psychiatr 55:1–15PubMedGoogle Scholar
  20. 20.
    Scott TF, Seay AR, Goust JM (1989) Pattern and concentration of IgG in cerebrospinal fluid in neurosarcoidosis. Neurology 39:1637–1639PubMedGoogle Scholar
  21. 21.
    Smith AS, Meisler DM, Weinstein MA, Tomsak RL, Hanson MR, Rudick RA, Farris BK, Ransohoff RM (1989) High-signal periventricular lesions in patients with sarcoidosis: neurosarcoidosis or multiple sclerosis. Am J Roentgenol 153:147–152Google Scholar
  22. 22.
    Stern BJ, Krumholz A, Johns C, Scott P, Nissim J (1985) Sarcoidosis and its neurological manifestation. Arch Neurol 42: 909–917PubMedGoogle Scholar
  23. 23.
    Stern BJ, Griffin DE, Luke RA (1987) Neurosarcoidosis: cerebrospinal fluid lymphocyte subpopulations. Neurology 37: 878–881PubMedGoogle Scholar
  24. 24.
    Strausz J, Müller-Quenheim J, Ferlinz R (1989) Sezernierter Interleukin-2-Rezeptor als Aktivitätsparameter der Sarkoidose. Dtsch Med Wochenschr 114:744–749PubMedGoogle Scholar
  25. 25.
    Stuart CA, Neelon A, Lebovitz HE (1978) Hypothylamic insufficiency: the cause of hypopituitarism in sarcoidosis. Ann Intern Med 88:589–594PubMedGoogle Scholar
  26. 26.
    Wiederholt WC, Siekert RG (1965) Neurological manifestations of sarcoidosis. Neurology 15:1147–1154PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1991

Authors and Affiliations

  • Norbert Sommer
    • 1
  • Michael Weller
    • 1
  • Dirk Petersen
    • 2
  • Horst Wiethölter
    • 1
  • Johannes Dichgans
    • 1
  1. 1.Department of NeurologyUniversity of TübingenTübingenFederal Republic of Germany
  2. 2.Department of NeuroradiologyUniversity of TübingenTübingenFederal Republic of Germany

Personalised recommendations