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

Abstract

Whipple's disease, affecting the CNS, can cause a wide variety of symptoms. Movement disorders are very prevalent, and some are pathognomonic of the disease. This systematic review analyzed all published cases of movement disorders because of CNS Whipple's disease, providing detailed information on clinical and associated features. We have also attempted to address sources of confusion in the literature, particularly related to differing uses of the terminology of movement disorder. This comprehensive overview of Whipple's disease‐induced movement disorders aims to aid neurologists in recognizing this very rare disorder and successfully reaching a laboratory‐confirmed diagnosis in order to initiate appropriate therapy. © 2018 International Parkinson and Movement Disorder Society

Pearls & Oy-sters: A rare presentation of Whipple disease

“…7,8 Other frequently described neurologic signs are disturbances in ocular movement like supranuclear ophthalmoplegia (32%-59%). 8,9 Oculomasticatory myorhythmia or oculofacioskeletal myorhythmia, a slow smooth convergent-divergent pendular nystagmus associated with synchronous contractions of other body parts, occur less frequently (8%-24%) but are considered pathognomonic for Whipple disease. 8,9 Dysfunction of the hypothalamus occurs in 11%-19% of patients and can manifest as sleep disorders (41%) like insomnia or hyperinsomnia.…”

“…8,9 Oculomasticatory myorhythmia or oculofacioskeletal myorhythmia, a slow smooth convergent-divergent pendular nystagmus associated with synchronous contractions of other body parts, occur less frequently (8%-24%) but are considered pathognomonic for Whipple disease. 8,9 Dysfunction of the hypothalamus occurs in 11%-19% of patients and can manifest as sleep disorders (41%) like insomnia or hyperinsomnia. 8,9 Infection of different brain regions can lead to several neurologic signs: cerebellar ataxia (11%-39%), pyramidal symptoms (10%-44%), myoclonic signs (15%-19%), seizures (11%-17%), headache (28%), or signs of myelopathy (11%).…”

“…8,9 Dysfunction of the hypothalamus occurs in 11%-19% of patients and can manifest as sleep disorders (41%) like insomnia or hyperinsomnia. 8,9 Infection of different brain regions can lead to several neurologic signs: cerebellar ataxia (11%-39%), pyramidal symptoms (10%-44%), myoclonic signs (15%-19%), seizures (11%-17%), headache (28%), or signs of myelopathy (11%). [7][8][9] Diagnosis of neuro-Whipple can be difficult due to the broad spectrum of nonspecific clinical features and its rarity.…”

“…8,9 Infection of different brain regions can lead to several neurologic signs: cerebellar ataxia (11%-39%), pyramidal symptoms (10%-44%), myoclonic signs (15%-19%), seizures (11%-17%), headache (28%), or signs of myelopathy (11%). [7][8][9] Diagnosis of neuro-Whipple can be difficult due to the broad spectrum of nonspecific clinical features and its rarity. 10 The reported range between first symptoms and diagnosis is 0-30 years, with an average of 6.4 years.…”

Thrombocytopenia and endocarditis in a patient with Whipple’s disease: case report

“…Whether his mental changes were secondary to the embolic events or WD or a combination is unknown but considering the presentation it is more likely the embolic events are directly responsible. CNS (central nervous system) findings have been reported in 10 to 40% of patients with classic WD, however, and include altered mental status and confusion [6]. Nonetheless, the WD was indirectly responsible since the vegetations on the heart valves are the presumed source of the emboli and they were probably caused by T. whipplei.…”