Guillain-Barre syndrome with preserved reflexes and normal cerebrospinal fluid: a case study
Keywords:
Guillain-Barre syndrome, Guillain-Barre Strohl syndrome, Landry-Guillain-Barre-Strohl syndrome, Acute motor axonal neuropathy, Normo-reflexia, Hyper-reflexia, Areflexic motor paralysisAbstract
Guillain-Barre syndrome (GBS) is an acute fulminant polyradiculopathy, which typically manifests as areflexic paralysis with variable sensory and autonomic involvement. Typical cerebrospinal fluid (CSF) picture consists of an elevated CSF protein without pleocytosis (albumin-cytologic dissociation). There have been many case reports of atypical presentations of GBS; with normo-reflexia or even hyper-reflexia from Chinese/Japanese and European population but only a few from Indian Subcontinent. Also the typical CSF picture if not found, makes the diagnosis of GBS even more difficult. A 24-year‑old man presented with weakness of all 4 limbs of 4 days duration with the antecedent history of loose stools and fever. On examination, there was flaccid paralysis involving all the 4 limbs (lower limb weakness more than the upper limb) with preserved reflexes, no sensory or cranial nerve deficit, no bladder-bowel involvement; and a normal CSF study at presentation, which 1 week later showed albumin-cytologic dissociation. On electro-diagnostic studies, it was proven as a case of acute motor axonal neuropathy. Patient was managed with routine empirical antibiotics and intravenous methyl prednisone; after 3 weeks, patient was discharged in a stable condition without any residual deficit. Our understanding about the GBS has changed manifolds over the last few decades with many atypical variants being reported across the world. This case study is to lay stress on the fact that even in the absence of typical clinical features and a normal CSF study the diagnostic possibility of GBS should be kept if there is strong clinical suspicion.
References
Stephen LH, Anthony AA. Guillain-Barre syndrome & other immune-mediated neuropathies. Harrison’s Principles of Internal Medicine. 18th Edition, Volume 2. New York: McGraw Hill; 2012: 3473-7.
Gupta D, Nair M, Baheti NN, Sarma PS, Kuruvilla A., Diplomate-American Board. Electrodiagnostic and clinical aspects of Guillain-Barré syndrome: an analysis of 142 cases. J Clin Neuromuscul Dis. 2008;10(2):42-51.
Yuki N, Kokubun N, Kuwabara S, Sekiguchi Y, Ito M, Odaka M, et al. Guillain-Barré syndrome associated with normal or exaggerated tendon reflexes. J Neurol. 2012;259(6):1181-90.
Kuwabara S, Ogawara K, Koga M, Mori M, Hattori T, Yuki N. Hyperreflexia in Guillain-Barré syndrome: relation with acute motor axonal neuropathy and anti-GM1 antibody. J Neurol Neurosurg Psychiatry. 1999;67(2):180-4.
Asbury AK, Cornblath DR. Preserved tendon reflexes in Campylobacter neuropathy [reply to letter]. Ann Neurol 1998;43:547.
Susuki K, Atsumi M, Koga M, Hirata K, Yuki N. Acute facial diplegia and hyperreflexia: a Guillain-Barré syndrome variant. Neurology. 2004;62(5):825 7.
Somarajan A. Guillain Barre syndrome with brisk reflexes-another variant. Neurol India. 2006;54(2):215-6.
Baheti NN, Manuel D, Shinde PD, Radhakrishnan A, Nair M. Hyperreflexic Guillain-Barré syndrome. Ann Indian Acad Neurol. 2010;13(4):305-7.
Guillain G, Barre JA, Strohl A. Sur un syndrome de radiculo-nevrite avec hyperalbuminose du liquid cephalorachidien sans reaction cellulaire. Remarqes sur les caracteres cliniques et graphiques des reflexes tendineux. Bull Soc Med Hop Paris 1916;25:1462 70.
Dana L, Charles R. Guillain-Barre syndrome. Am Fam Physician 2004;10:2405-10.
Hughes RA, van Der Meche FGA. Corticosteroids for treating Guillain-Barre syndrome. Cochrane Database Syst Rev 2003;(4):CD001446.