Systemic Sarcoidosis Presenting with Headache and Stroke-Like Episodes

Sarcoidosis is a multisystem granulomatous disorder. Neurological manifestations as a presenting symptom are relatively rare. A 26-year-old male presented with a five-week history of headache suggestive of raised intracranial pressure. He subsequently developed transient episodes of mild right-sided hemiparesis and numbness. Magnetic resonance imaging (MRI) of brain revealed widespread inflammatory white matter lesions, an ischaemic focus in the left corona radiata, and widespread microhaemorrhages consistent with a more diffuse vasculopathy. Serum angiotensin-converting enzyme (ACE) level was normal. Lumbar puncture revealed an elevated opening pressure (36 cmH2O) and inflammatory cerebrospinal fluid (CSF). Computerised tomography (CT) of chest, abdomen, and pelvis revealed widespread lymphadenopathy and biopsy of axillary lymph nodes revealed the presence of noncaseating granulomata in keeping with systemic sarcoidosis. The patient responded well to corticosteroids. This case highlights the importance of considering sarcoidosis to be a rare but potentially treatable cause of stroke in younger patients.

[1]  Yuebing Li,et al.  Longitudinal ultra-extensive transverse myelitis as a manifestation of neurosarcoidosis , 2015, Journal of the Neurological Sciences.

[2]  A. Prasse,et al.  Sarcoidosis , 2014, The Lancet.

[3]  J. Reich.  A critical analysis of sarcoidosis incidence assessment , 2013, Multidisciplinary Respiratory Medicine.

[4]  D. Collins,et al.  Neurosarcoidosis‐related intracranial haemorrhage: three new cases and a systematic review of the literature , 2013, European journal of neurology.

[5]  I. González-Aramburu,et al.  Sarcoidosis presenting as transient ischemic attack status. , 2012, Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association.

[6]  M. Mochizuki,et al.  Validation of international criteria for the diagnosis of ocular sarcoidosis proposed by the first international workshop on ocular sarcoidosis , 2010, Japanese Journal of Ophthalmology.

[7]  陶仲为.  Sarcoidosis , 2009 .

[8]  B. Milleron,et al.  [Brain and medullar neurosarcoidosis, complicated by stroke with local vasculitis]. , 2009, Revue neurologique.

[9]  M. Raftery,et al.  Sarcoid tubulo-interstitial nephritis: long-term outcome and response to corticosteroid therapy. , 2006, Kidney international.

[10]  R. Maciunas,et al.  Neurosarcoidosis presenting with simple partial seizures and solitary enhancing mass: case reports and review of the literature , 2005, Epilepsy & Behavior.

[11]  G. Ishak,et al.  Intracerebral Bleeding in a Patient With Neurosarcoidosis While on Corticosteroid Therapy , 2005, Southern medical journal.

[12]  A. Steck,et al.  Successful treatment of steroid-refractory neurosarcoidosis with infliximab , 2004, Journal of Neurology.

[13]  D. Mimouni,et al.  Mycophenolate mofetil may serve as a steroid‐sparing agent for sarcoidosis , 2003, The British journal of dermatology.

[14]  J. Piette,et al.  Central nervous system sarcoidosis: follow-up at MR imaging during steroid therapy. , 2000, Radiology.

[15]  D. Moller.  Cells and cytokines involved in the pathogenesis of sarcoidosis. , 1999, Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG.

[16]  M. Rovaris,et al.  Central nervous system sarcoidosis--diagnosis and management. , 1999, QJM : monthly journal of the Association of Physicians.

[17]  O. Sharma.  Effectiveness of chloroquine and hydroxychloroquine in treating selected patients with sarcoidosis with neurological involvement. , 1998, Archives of neurology.

[18]  M. Iannuzzi,et al.  Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. , 1997, American journal of epidemiology.

[19]  M. Angi,et al.  Uveitis as a presenting feature of chronic sarcoidosis. , 1996, The European respiratory journal.

[20]  B. Stern,et al.  The treatment of neurosarcoidosis with cyclosporine. , 1992, Archives of neurology.

[21]  M. Swash,et al.  Sarcoidosis presenting with stroke. , 1989, Stroke.

[22]  R. Mckendall,et al.  Cerebrospinal fluid immunoglobulin abnormalities in neurosarcoidosis. , 1989, Archives of neurology.

[23]  D. MacManus,et al.  Magnetic resonance imaging in central nervous system sarcoidosis , 1988, Neurology.

[24]  V. Oksanen.  Neurosarcoidosis: clinical presentations and course in 50 patients , 1986, Acta neurologica Scandinavica.

[25]  E. Spokes,et al.  NEUROSARCOIDOSIS , 1985, The Lancet.

[26]  B. Stern,et al.  Sarcoidosis and its neurological manifestations. , 1985, Archives of neurology.

[27]  G. Schwesinger,et al.  [Sarcoidosis of the central nervous system]. , 1976, Zeitschrift fur die gesamte innere Medizin und ihre Grenzgebiete.

[28]  E. Reske‐Nielsen,et al.  PERIANGIITIS AND PANANGIITIS AS A MANIFESTATION OF SARCOIDOSIS OF THE BRAIN: REPORT OF A CASE , 1962, The Journal of nervous and mental disease.

[29]  R. L. Williams,et al.  Neurosarcoidosis presenting as acute infarction on diffusion-weighted MR imaging: summary of radiologic findings. , 2007, AJNR. American journal of neuroradiology.

[30]  F. Berenbaum,et al.  Muscle involvement in sarcoidosis: a retrospective and followup studies. , 2006, The Journal of rheumatology.

[31]  S. Kiechl,et al.  Subarachnoid hemorrhage as presenting feature of isolated neurosarcoidosis , 2004, The clinical investigator.

[32]  A. Michotte,et al.  Focal neurological deficit with sudden onset as the first manifestation of sarcoidosis: a case report with MRI follow-up. , 1991, European neurology.

[33]  B. Stern,et al.  Neurosarcoidosis and stroke. , 1990, Stroke.

[34]  A. Hartmann,et al.  Pentoxifylline alters the natural course of acute nonhemorrhagic stroke. , 1990, Stroke.