Neurological manifestations of chronic graft versus host disease

Allogeneic haemopoietic stem cell transplant (allo-HSCT) is an established therapy for many malignant and non-malignant conditions, with an increasing number of uses and indications in recent decades. Complications including graft versus host disease (GvHD) carry significant morbidity and mortality. Chronic GvHD can affect multiple organs, including skin, gastrointestinal, respiratory and eyes. Neurological involvement is less common and can be a diagnostic and therapeutic challenge for patients and clinicians.

A retrospective analysis was conducted of patients who underwent allo-HSCT for a haematological malignancy with an established diagnosis of chronic GvHD at a major Australian institution.

Out of 455 allo-HSCT cases, 228 (50%) had chronic GvHD, seven (3.1%) of which were identified to have neurological complications. Four cases, each consisting of myasthenia gravis, Bickerstaff brainstem encephalitis, acute disseminated encephalomyelitis and inflammatory meningitis, were selected to illustrate peripheral and central neurological manifestations of chronic GvHD. Presentations, management and outcomes were discussed and contrasted to similar cases available in the literature.

Chronic GvHD infrequently presents with neurological manifestations. Understanding the similarities and differences of these and their idiopathic counterparts allows an enhanced understanding of their pathophysiology within the context of GvHD, as well as some significant differences to conventional management of these neurological conditions.