Clinical Rheumatology

, Volume 30, Issue 12, pp 1631–1639 | Cite as

The challenge of profound hypoglycorrhachia: two cases of sarcoidosis and review of the literature

  • Harini Sarva
  • Rachel Chapman
  • Eghosa Omoregie
  • Charles Abrams
Case Based Review


We report two cases of sarcoidosis which were initially misdiagnosed due to profound hypoglycorrhachia. Sarcoidosis is a systemic inflammatory disorder which can mimick other entities and has a vast array of presentations including altered mentation and signs of increased intracranial pressure. Though hypoglycorrhachia has been described previously, these two cases are unique in not only the level of hypoglycorrhachia but also the diagnostic challenge presented. We propose that sarcoidosis should be in the differential of all cases of CNS diseases with any level of hypoglycorrhachia.


Hypoglycorrhachia Mass lesion Pituitary Sarcoidosis 


  1. 1.
    Scott TF (1993) Neurosarcoidosis: progress and clinical aspects. Neurology 43:8–12PubMedGoogle Scholar
  2. 2.
    Nowak DA, Widenka DC (2001) Neurosarcoidosis: a review of its intracranial manifestation. J Neurol 248:363–372PubMedCrossRefGoogle Scholar
  3. 3.
    Hoitsma E, Faber CG, Drent M, Sharma OP (2004) Neurosarcoidosis: a clinical dilemma. Lancet Neurol 3:397–407PubMedCrossRefGoogle Scholar
  4. 4.
    Iannuzzi MC, Rybicki BA, Teirstein AS (2007) Medical progress: sarcoidosis. New Engl J Med 357(21):2153–2165PubMedCrossRefGoogle Scholar
  5. 5.
    Ricker W, Clark M (1949) Sarcoidosis: a clinical pathologic review of 300 cases. Am J Clin Pathol 19:725–749PubMedGoogle Scholar
  6. 6.
    Burns TM (2003) Neurosarcoidosis. Arch Neurol 60:1166–1168PubMedCrossRefGoogle Scholar
  7. 7.
    Heerfordt CF (1909) Über eine febris uveo-parotidea subchronica, an der glandula parotis und der uvea des auges lokalisiert und ha¨ufig mit paresen cerebrospinaler nerven kompliziert. Graefes Arch Ophthalmol 70:254–273CrossRefGoogle Scholar
  8. 8.
    Colover J (1948) Neurosarcoidosis with involvement of the nervous system. Brain 71:451–475PubMedCrossRefGoogle Scholar
  9. 9.
    Widerholt WC, Siekert RG (1965) Neurologic manifestations of sarcoidosis. Neurology 15:1147–1154Google Scholar
  10. 10.
    Kumar G, Kang CA, Gianni C (2007) Neurosarcoidosis presenting as a cerebellar mass. J Gen Intern Med 22:1373–1376PubMedCrossRefGoogle Scholar
  11. 11.
    Pennell WH (1951) Boeck’s sarcoid with involvement of the central nervous system. Arch Neurol Psychiatry 66:728–737Google Scholar
  12. 12.
    Meyer JS, Foley JM, Campagna-Pinto D (1953) Granulomatous angiitis of the meninges in sarcoidosis. Arch Neurol 69:587–600Google Scholar
  13. 13.
    Bihan H, Christozova V, Dumas J, Jomaa R, Valeyre D, Tazi A, Reach G, Krivitzky A, Cohen R (2007) Sarcoidosis: clinical, hormonal, and magnetic resonance imaging (MRI) manifestations of hypothalamic–pituitary disease in 9 patients and review of the literature. Medicine 86:259–268PubMedCrossRefGoogle Scholar
  14. 14.
    Stuart C, Neelon F, Lebovitz H (1978) Hypothalamic insufficiency: the cause of hypopituitarism in sarcoidosis. Ann Intern Med 5:589–594Google Scholar
  15. 15.
    Porter N, Beynon HL, Randeva HS (2003) Endocrine and reproductive manifestations of sarcoidosis. QJM: Oxford J 96:553–561CrossRefGoogle Scholar
  16. 16.
    Smith D, Cullen MJ (2005) Two cases of hypothalamic–pituitary sarcoidosis. J Royal Soc Med 98:167–169CrossRefGoogle Scholar
  17. 17.
    Bullmann C, Faust M, Hoffmann A, Heppnerl C, Jockenhövel, Dirk Müller-Wieland F, Krone W (2000) Five cases with central diabetes insipidus and hypogonadism as first presentation of neurosarcoidosis. Eur J Endocrinol 142:365–372PubMedCrossRefGoogle Scholar
  18. 18.
    Hildebrand J, Aoun M (2003) Chronic meningitis: still a diagnostic challenge. J Neurol 250:653–660PubMedCrossRefGoogle Scholar
  19. 19.
    Stein JH, Eisenberg JM (1998) Part III: pulmonary and critical care medicine. Chapter IV: specific diseases. Internal medicine, 5th edn. Mosby, St. Louis, p 458Google Scholar
  20. 20.
    Viola GM (2010) Case study: extreme hypoglycorrhachia: not always bacterial meningitis. Nat Rev Neurol 6:637–641PubMedCrossRefGoogle Scholar
  21. 21.
    Petersdorf RG, Harter DH (1961) The fall in cerebrospinal fluid sugar in meningitis. Arch Neurol 4:21–29PubMedGoogle Scholar
  22. 22.
    Quagliarello VJ, Long WJ, Scheld WM (1986) Morphologic alterations in the blood brain barrier with experimental meningitis in the rat: temporal sequence and role of encapsulation. J Clin Investig 77:1084–1095PubMedCrossRefGoogle Scholar
  23. 23.
    Quagliarello VJ, Wispelwey B, Long WJ, Scheld WM (1991) Recombinant human interleukin-1 induces meningitis and blood-brain barrier injury in the rat: characterization and comparison with tumor necrosis factor alpha. J Clin Investig 87:1360–1366PubMedCrossRefGoogle Scholar
  24. 24.
    Moller K, Skinhoj P, Knudsen GM, Larsen FS (2000) Effect of short-term hyperventilation on cerebral blood flow autoregulation in patients with acute bacterial meningitis. Stroke 31:1116–1122PubMedCrossRefGoogle Scholar
  25. 25.
    Muller M, Merkelbach S, Huss GP, Schimrigk K (1995) Clinical relevance and frequency of transient stenoses of the middle and anterior cerebral arteries in bacterial meningitis. Stroke 26:1399–1403PubMedCrossRefGoogle Scholar
  26. 26.
    Veres L, Utz JP, Houser OW (1997) Sarcoidosis presenting as a central nervous system lesion. Chest 111:518–521PubMedCrossRefGoogle Scholar
  27. 27.
    Bakshi R, Fensternmaker RA, Bates VE, Ravichandran TP, Goodloe S, Kinkel WR (1998) Neurosarcoidosis presenting as a large suprasellar mass magnetic resonance imaging findings. Clin Imaging 22:323–326PubMedCrossRefGoogle Scholar
  28. 28.
    Sponsler JL, Werz MA, Maciunas R, Cohen M (2005) Neurosarcoidosis presenting with simple partial seizures and solitary enhancing mass: case reports and review of the literature. Epilepsy Behav 6:623–630PubMedCrossRefGoogle Scholar
  29. 29.
    Elias WJ, Lanzino G, Reitmeye M, Jane JA (1999) Solitary sarcoid granuloma of the cerebellopontine angle. Surg Neurol 51:185–190PubMedCrossRefGoogle Scholar
  30. 30.
    Strickland-Marmol LB, Fessler RG, Rojiani AM (2000) Necrotizing sarcoid granulomatosis mimicking an intracranial neoplasm: clinicopathologic features and review of the literature. Mod Pathol 13(8):909–913PubMedCrossRefGoogle Scholar
  31. 31.
    Zajicek JP, Scolding NJ, Foster O, Rovaris M, Evanson J, Moseley IF, Scadding JW, Thompson EJ, Chamoun V, Miller DH, McDonald WI, Mitchell D (1999) Central nervous system sarcoidosis—diagnosis and management. Q J Med: An Int J Med 92:103–117Google Scholar
  32. 32.
    Androdias A, Maillet D, Marignier M, Pinede L, Confavreux C, Broussolle C, Vukusic S, Seve P (2011) Mycophenolate mofetil may be effective in CNS sarcoidosis but not in sarcoid myopathy. Neurology 76:1168–1172PubMedCrossRefGoogle Scholar
  33. 33.
    Marangoni S, Argentiero V, Tavolato B (2006) Neurosarcoidosis clinical description of 7 cases with a proposal for a new diagnostic strategy. J Neurol 253:488–495PubMedCrossRefGoogle Scholar
  34. 34.
    Mayer SA, Yim GK, Onesti ST, Lynch T, Faust PL, Marder K (1993) Biopsy-proven isolated sarcoid meningitis. J Neurosurg 78:994–996PubMedCrossRefGoogle Scholar
  35. 35.
    Pawate S, Moses H, Sriram S (2009) Presentation and outcomes of neurosarcoidosis: a study of 54 cases. Q J Med: An Int J Med 102:449–460Google Scholar
  36. 36.
    Joseph FG, Scolding NJ (2008) Neurosarcoidosis: a study of 30 new cases. J Neurol Neurosurg Psychiatry 80:297–304PubMedCrossRefGoogle Scholar
  37. 37.
    Gaines JD, Eckman PB, Remington JS (1970) Low CSF glucose level in sarcoidosis involving the central nervous system. Arch Intern Med 125:333–336PubMedCrossRefGoogle Scholar
  38. 38.
    Douglas AC, Maloney FJ (1973) Sarcoidosis of the central nervous system. J Neurol Neurosurg Psychiatry 36:1024–1033PubMedCrossRefGoogle Scholar
  39. 39.
    Stern BJ, Krumholz A, Johns C, Scott P, Nissim J (1985) Sarcoidosis and its neurological manifestations. Arch Neurol 42:909–917PubMedGoogle Scholar
  40. 40.
    Sommer N, Weller M, Peterson D, Weitholter H, Dichgans J (1991) Neurosarcoidosis without systemic sarcoidosis. Eur Arch Psychiatry Clin Neurosci 240:334–338PubMedCrossRefGoogle Scholar
  41. 41.
    Takahiro M, Shoichi I, Masahiro M, Satoshi K (2009) Diffuse neurosarcoidosis involving only the leptomeninges of the brainstem and spinal cord. Intern Med 48(21):1909–1913CrossRefGoogle Scholar
  42. 42.
    Joerg-Stubgen (1995) Neurosarcoidosis presenting as a retroclival mass. Surg Neurol 43:85–88CrossRefGoogle Scholar
  43. 43.
    Sattelmeyer VM, Vernet O, Janzer R, de Tribolet N (1999) Neurosarcoidosis presenting as an isolated mass of the quadrigeminal plate. J Clin Neurosci 6(3):259–261PubMedCrossRefGoogle Scholar
  44. 44.
    Westhout FD, Linkskey ME (2008) Obstructive hydrocephalus and progressive psychosis: rare presentations of neurosarcoidosis. Surg Neurol 68:288–292CrossRefGoogle Scholar

Copyright information

© Clinical Rheumatology 2011

Authors and Affiliations

  • Harini Sarva
    • 1
  • Rachel Chapman
    • 1
  • Eghosa Omoregie
    • 1
  • Charles Abrams
    • 1
  1. 1.SUNY Downstate Medical Center450 Clarkson AvenueBrooklynUSA

Personalised recommendations