Isolated inctracranial Whipple's disease—Report of a rare case and review of the literature

Mohamed, Wazim; Neil, Erin; Kupsky, William J.; Juhász, Csaba; Mittal, Sandeep; Santhakumar, Sunitha

doi:10.1016/j.jns.2011.05.029

Cited by 31 publications

(29 citation statements)

References 41 publications

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“…Focal lesions have been observed on MRI in patients with central effects from Whipple’s disease, suggesting that the infection may anatomically disrupt pathways in the brain important for regulation of orbital and facial muscles. [52] The antibiotic therapy is the mainstay of treatment of Whipple’s disease and associated neurological complications.…”

Section: Whipple’s Diseasementioning

confidence: 99%

Acquired pendular nystagmus

Kang¹,

Shaikh²

2017

Journal of the Neurological Sciences

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Acquired pendular nystagmus is comprised of quasi-sinusoidal oscillations of the eyes significantly affecting gaze holding and clarity of vision. The most common causes of acquired pendular nystagmus include demyelinating disorders such as multiple sclerosis and the syndrome of ocular palatal tremor. However, several other deficits, such as pharmacological intoxication, metabolic and genetic disorders, and granulomatous disorders can lead to syndromes mimicking acquired pendular nystagmus. Study of the kinematic features of acquired pendular nystagmus has suggested a putative pathophysiology of an otherwise mysterious neurological disorder. Here we review clinical features of neurological deficits that co-occur with acquired pendular nystagmus. Subsequent discussion of the pathophysiology of individual forms of pendular nystagmus speculates on mechanisms of the underlying disease while providing insights into pharmacotherapy of nystagmus.

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Section: Whipple’s Diseasementioning

confidence: 99%

Acquired pendular nystagmus

Kang¹,

Shaikh²

2017

Journal of the Neurological Sciences

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“…The term oculo‐facio‐skeletal myorhythmia (OFSM) was introduced in 1988 by Hausser‐Hauw and colleagues, who also noted that limb myorhythmia may accompany craniocervical myorhythmia in WD . Subsequent reports provided additional evidence that OMM, often associated with OFSM, is a highly specific sign for WD …”

Section: Myorhythmia Secondary To Whipple's Disease and Other Centralmentioning

confidence: 99%

Myorhythmia: Phenomenology, etiology, and treatment

2014

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Myorhythmia is defined as repetitive, rhythmic, slow (1-4 Hz) movement affecting chiefly cranial and limb muscles. When occurring in the limbs it may be oscillatory and jerky, whereas oculo-masticatory myorhythmia, typically associated with Whipple's disease, is a slow, repetitive, often asymmetrical, facial and ocular movement. Thus, myorhythmia overlaps phenomenologically with tremor and segmental myoclonus. Although often present at rest, it must be differentiated from parkinsonian or dystonic tremor. Recognition of this movement disorder is important because it is usually associated with lesions involving the brainstem, thalamus, or other diencephalic structures with potentially treatable etiologies. In addition to Whipple's disease, myorhythmia has been described in patients with cerebrovascular disease, listeria encephalitis, anti-N-methyl-d-aspartate receptor encephalitis, steroid-responsive encephalopathy associated with autoimmune thyroiditis, multiple sclerosis, and other disorders. In addition to our own experience, we have systematically reviewed the medical literature, focusing on the phenomenology, pathophysiology, and etiology of this poorly recognized movement disorder. In this review, we aim to highlight the clinical features that differentiate myorhythmia from other movement disorders. Treatment should be directed against the underlying etiology.

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“…It can be in the form of headache, confusion, dementia, ophthalmoplegia, characteristic myoclonus as oculomasticatorymyorhythmia (convergent eye movements with simultaneous chewing movements), gait abnormalities, seizures and finally coma [21,22]. Ocular involvement can be in the form of uveitis, vitritis, keratitis, retinitis, and retinal hemorrhages [23].…”

Section: Clinical Featuresmentioning

confidence: 99%

Whipple’s Disease: A Case Report and Review of Literature

Elkholy¹,

Khaled²,

Aziz³

et al. 2017

MOJCR

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Whipple's disease is a rare systemic disease. It is caused by a gram-positive infectious bacillus called Tropheryma whipplei (T. whipplei). The common presenting feature is malabsorption, but it can also affect multiple organs as heart, central nervous system (CNS), joints, skin, lungs and vascular system. Presentation can be highly variable but mainly the patients are complaining of weight loss, diarrhea, joint pain and arthritis, about 15% of patients do not develop these classic symptoms. Endoscopic picture usually shows pale yellow shaggy mucosa with erythematous eroded patches in the duodenum extending up to the jejunum. Definitive diagnosis is made mainly by endoscopic duodenal biopsies that show characteristic foamy macrophages in the lamina propria containing non-acid fast gram positive bacilli that stain red with Periodic acid-Schiff (PAS) stain. Immunohistochemical staining for antibodies against T. whipplei had been used to detect the organism in a variety of tissues. Polymerase chain reaction (PCR) of T. whipplei is considered a confirmatory test. We present a case of classic Whipple's disease with review of literature for better understanding of the presentation, methods of diagnosis and treatment of the disease.

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Isolated inctracranial Whipple's disease—Report of a rare case and review of the literature

Cited by 31 publications

References 41 publications

Acquired pendular nystagmus

Acquired pendular nystagmus

Myorhythmia: Phenomenology, etiology, and treatment

Whipple’s Disease: A Case Report and Review of Literature

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