A Combined Central and Peripheral Demyelination (CPPD) Syndrome After COVID-19 Vaccination
Lydia Kauffman1, Nader El Seblani1, James Grogan2, Jikku Jose Zachariah3, Puneet Kochar4, Rae Bacharach4
1Penn State Milton S. Hershey Medical Center, 2Pennsylvania State University Hershey Medical Center, 3Pennsylvania State University-Hershey Medical Center, 4Penn State University, Milton S Hershey Medical Center
Objective:
Describe a novel case of combined central and peripheral demyelinating disease.
Background:
Combined Central Nervous System and Peripheral Nervous System Demyelination (CCPD) is a rare phenomenon whose pathogenesis remains largely unknown. The aim of this report is to describe a case of CCPD with the clinical features, diagnostics results, treatments and outcomes.
Design/Methods:
A 50-year-old female, without remarkable medical history initially presented with subacute right sided weakness, dysarthria and ataxia as well as hand and foot paresthesias. Brain MRI demonstrated bilateral enhancing lesions in midbrain tegmentum. Extensive studies including CSF basic cell counts, cytology, cytometry, and paraneoplastic/autoimmune panel, heavy metals testing were all nonconclusive. Serum testing for ganglioside disorders and nodopathies was negative. She was treated with a three-day course of high dose IV steroids with significant improvement of her symptoms. One month later, the patient experienced short episodes of severe ataxia and weakness lasting 10-15 seconds despite improvement in midbrain lesion on MRI brain. Additionally, her paresthesias worsened. Nerve conduction studies were performed and demonstrated a length-dependent, sensorimotor, predominately demyelinating polyneuropathy. The patient was empirically treated with a second round of IV steroids with significant improvement.
Conclusions:
CCPD cases are rarely reported likely due to being underdiagnosed with unclear pathologies and guidelines for treatment. This case exhibits a midbrain demyelinating lesion followed by sensorimotor and demyelinating polyneuropathy. COVID-19 infection has been linked to both acute and central peripheral demyelinating pathologies. Retrospectively, three months before developing her symptoms, our patient acquired COVID-19 infection. One month prior to symptom onset she received Ad26COV2.S Janssen vaccination. Development of deficits after vaccination rather than after the viral infection may suggest a role for the immune complex mechanisms in CPPD pathogenesis, a theory that has yet to be verified with future cohort studies and advanced laboratory testing.