Central Nervous System Involvement as Relapse in Undiagnosed Whipple’s Disease with Atypical Symptoms at Onset

Abstract

Whipple’s disease (WD) is a rare systemic disease caused by the gram-positive bacillus Tropheryma Whipplei and mostly characterized by arthralgias, chronic diarrhea, weight loss, fever and abdominal pain. Central Nervous System involvement is not uncommon and it may precede other disease manifestations, appear after treatment and improvement of gastrointestinal signs or rarely be the only WD symptom. We report a case in a middle-aged male with unexplained neurological signs and symptoms which were presented as relapse of previously undiagnosed WD with atypical symptoms at onset. After diagnosis confirmation, the patient was appropriately treated which resulted in improvement of major symptoms.

Systematic review of movement disorders and oculomotor abnormalities in Whipple's disease

“…The percentage of clinical CNS involvement varies considerably, ranging from 6% to 63%, with most estimates of 10%‐43%. In approximately 5% of cases, the initial manifestations are purely neurological . CNS‐WhD can mimic almost any neurological disorder; its most common neurological manifestations include cognitive changes, an encephalopathy, a neuropsychiatric syndrome, hypothalamic manifestations, and oculomotor abnormalities, particularly vertical supranuclear gaze palsies (SGPs).…”

“…In approximately 5% of cases, the initial manifestations are purely neurological . CNS‐WhD can mimic almost any neurological disorder; its most common neurological manifestations include cognitive changes, an encephalopathy, a neuropsychiatric syndrome, hypothalamic manifestations, and oculomotor abnormalities, particularly vertical supranuclear gaze palsies (SGPs). Movement disorders (MDs) are common and account for about half of CNS‐WhD syndromes .…”