Neuromuscular Case Vignettes

The patient presents with a clinical picture consistent with Guillain Barre Syndrome (GBS), considering the fact that the peak of weakness occurred within a period of one month followed by spontaneous improvement. Follow up nerve conduction study (demonstrated below), showed absent sensory nerve action potentials, very prolonged distal latencies and demyelinating range slowing of the motor conduction velocities, which along with the clinical tempo of the disease confirms the diagnosis of GBS. She also has lymphadenopathy, mild leukopenia, polyclonal hypergammaglobulinemia, and CSF pleocytosis which suggests a systemic disease as the cause of GBS. Ddx therefore includes GBS associated with: 1)  Lymphoma: Hodgkin disease and non-Hodgkin Lymphoma rarely present with GBS 1-3 ;2) Neurosarcoidosis should also be considered as she also had high serum ACE level. Neurosarcoidosis typically presents with polyradiculopathy, mononeuritis multiplex, or polyradiculopathy, but association with GBS has rarely been reported 4; 3) Ebstein Barre virus (EBV) infection which is a cause for lymphadenopathy and is one of the antecedent infections associated with GBS 5; 4) HIV infection. CSF pleocytosis is uncharacteristic for garden variety GBS and is suggestive for GBS after HIV infection. HIV infection is also associated with both lymphadenopathy and polyclonal hypergammaglobulinemia 6

Click here for a follow-up test that resulted in the diagnosis.

Reference:

1.              Lisak RP, Mitchell M, Zweiman B, Orrechio E, Asbury AK. Guillain-Barre syndrome and Hodgkin’s disease: three cases with immunological studies. Annals of neurology. 1977;1:72-78.

2.              Anderson D, Beecher G, Steve TA, Jen H, Camicioli R, Zochodne DW. Neurological Nuance: Hodgkin lymphoma presenting with Guillain-BarrE syndrome. Muscle & nerve. 2017;55:601-604.

3.              Polo-Romero FJ, Sanchez-Beteta P, Perona-Buendia P, Perez-Garcia AM. Guillain-Barre syndrome as first presentation of non-Hodgkin lymphoma. Neurologia. 2012;27:511-513.

4.              Miller R, Sheron N, Semple S. Sarcoidosis presenting with an acute Guillain-Barre syndrome. Postgraduate medical journal. 1989;65:765-767.

5.              Yuki N, Hartung HP. Guillain-Barre syndrome. The New England journal of medicine. 2012;366:2294-2304.

6.              De Milito A, Nilsson A, Titanji K, et al. Mechanisms of hypergammaglobulinemia and impaired antigen-specific humoral immunity in HIV-1 infection. Blood. 2004;103:2180-2186.

CT scan axillary, arrow points to enlarged lymph nodes

Pelvic CT scan, arrow points to enlarged lymph node