Whipple's disease and the central nervous system a case report and a review of the literature

doi:10.1016/0303-8467(96)00035-2

Clinical Neurology and Neurosurgery

Volume 98, Issue 4, November 1996, Pages 299-304

https://doi.org/10.1016/0303-8467(96)00035-2 Get rights and content

Abstract

A 65-year-old man was suffering from recurrent manic psychosis accompanied by weight loss. He also had a history of pleural effusion, aspecific migratory non-deforming seronegative polyarthritis, sensorineural hearing loss and semicircular canal paresis. Whipple's disease (WD) had been diagnosed at the age of 63 years. On admission to hospital)he had weight loss, diarrhoea in combination with an organic, brain syndrome, hemiparesis and ophthalmoplegia, including internuclear ophthalmoplegia (INO). A clinical diagnosis of central nervous system (CNS) WD was made. MRI revealed a thalamus lesion that halved in size during sulfamethoxazole-trimethop:rim treatment. The organic brain syndrome and ophthalmoplegia diminished also, as did the cerebrospinal fluid (CSF) IgG level. A review of CNS WD is presented and implications for treatment are discussed.

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This chapter covers eye movement disorders that are characterized by intact alignment but in which the eyes either have restricted motility, move too slowly, or are misdirected. In neuro-ophthalmic terminology, these include horizontal and vertical conjugate gaze limitations, voluntary smooth pursuit and saccadic deficits, and involuntary conjugate gaze deviations.
These conditions tend to result from impaired supranuclear input upon the ocular motor nuclei. Thus, for the most part, third, fourth, and sixth nerve function, as well the vestibuloocular reflex, are intact except in the situations noted. In contrast, disorders in which the eyes tend to be misaligned are covered in Chapters 15 and 18. In Chapter 17, conditions characterized by excessive or inaccurate saccades are reviewed.
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Recurrent strokes may be secondary to primary WD endocarditis. Wernicke's encephalopathy has been reported [51,68–70], meningitis [71] with PAS positive macrophages in the meninges [72], cranial nerves involvement like hearing loss and vestibulo-ocular reflex impairment which might be also associated with peripheral labyrinthine [73]. Acute myelopathy with secondary cerebral involvement suggesting CNS vasculitis has also been reported [74].
Whipple's disease is a rare, chronic, systemic infectious disorder with prominent intestinal manifestations. It presents with weight loss, arthralgia, diarrhea, and abdominal pain. There are different entities of infection or carriage, respectively, classical Whipple's disease, localized WD, and Isolated Neurological WD. The disease is commonly diagnosed by biopsy of lymph node or small-bowel. Histological detection within duodenal biopsies with “Periodic acid Schiff” (PAS) staining still is first choice for the diagnosis of classical Whipple's disease. PCR or immunohistochemistry can identify the agent more specifically, and DNA sequencing for Tropheryma whipplei on lymphocytes from blood and cerebrospinal fluid from PCR-positive specimens, is essential. Cell-mediated immunity in active and inactive Whipple's disease has subtle defects that might predispose some individuals to symptomatic infection with this bacillus. Successful treatment can be achieved in most of the cases by antimicrobial therapy. WD can be progressive lethal. Immune reconstitution inflammatory syndrome (IRIS) might complicate the course of treatment and in worst case end fatal.
Rare occurrence of whipple disease in a young female patient with a fatal outcome
2013, Egyptian Journal of Forensic Sciences
Whipple Disease is a rare chronic multi-systemic disease caused by the ubiquitous environmental Gram-positive bacterium Tropheryma whipplei. It can be fatal if untreated. Here we describe the fatal outcome in a 27-year-old Caucasian female patient with a three-month history of persistent fever, anemia, weight loss and diarrhea. The final resolution of the diagnostic process was only reached after the patient’s death thanks to autopsy. The case depicted is a classic Whipple Disease histologically characterized by digestive involvement based on positive periodic acid-Schiff (PAS) staining and immunohistochemical analysis for T. whipplei and systemic involvement (pericarditis, brain lipid storage, renal fat embolism). The case reported here is of interest because the patient was a woman and also young, characteristics which do not meet the classical epidemiological features of this elusive and troubling disease.
An atypical case of neuro-Whipple: Clinical presentation, magnetic resonance spectroscopy and follow-up
2010, Journal of the Neurological Sciences
Citation Excerpt :
WD, first described by George H. Whipple in 1907 [4], is an infectious disease that usually affects middle-aged Caucasian men. The CNS may be affected in as many as 43% of patients with WD during the disease course [5]. Peripheral neuropathy is a very rare symptom of WD, often caused by malabsorption.
We report a case of a 53-year-old man with a 2-year history of progressive gait and balance disturbance, supranuclear ophthalmoparesis, mild dysarthria and dysmetria. EMG revealed a lower limb axonal sensory–motor neuropathy, while MR imaging demonstrated a small focal lesion in the right frontal lobe, mild diffuse hyperintensity of the periventricular white matter and diffuse brain atrophy. Magnetic resonance spectroscopy revealed a mild decrease in N-acetyl-aspartate peak and an increase in the choline peak in the small right frontal lesion and within 6 voxels of interest in normal appearing cerebral tissue. According to the clinical picture the diagnosis of WD was made by the positivity of PCR for T. whipplei DNA on CSF. After treatment the patient showed a mild clinical improvement although MR images and laboratory test remained unchanged. The MRS findings suggest that the pathological process of the disease diffusely involves the brain. Despite the absence of gastrointestinal involvement WD should be suspected in all complex and atypical neurological pictures, even in presence of peripheral involvement, in order to be able to start treatment promptly.
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A case of Whipple's disease with development of antibiotic resistance is reported. The patient's symptomatology correlated with evolution of diffusion abnormality rather than with lesion enhancement. The lesion demonstrated no hyperperfusion, moderately elevated choline, and decreased N-acetylaspartate.
Conventional magnetic resonance (MR) imaging findings of central nervous system Whipple's disease are nonspecific and may mimic neoplasm. MR perfusion and spectroscopy findings are reported, which may assist in diagnosis. Change in diffusion restriction appears to be a potential imaging indicator of clinical progression and response to therapy.
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Case reportWhipple's disease and the central nervous system a case report and a review of the literature

Abstract

Am. J. Med. Sci.

Gastroenterology

Gastroenterology

Gastroenterology

Gastroenterology

A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues

Bull. Johns Hopkins Hosp.

Whipple's disease: A review of 19 patients from one hospital and a review of the literature since 1950

Medicine (Baltimore)

Whipple's disease: Etiopathogenesis, treatment, diagnosis, and clinical course; case report and review of the world literature

Acta Hepato-gastroenterol. (Stuttg.)

Cranial computed tomography in Whipple's disease

J. Comput. Assist. Tomogr.

The arthritis of Whipple's disease

Arthritis Rheum.

Morbus Whipple

Fortschr. Neurol. Psychiatr.

Die Whipplesche Erkrankung. Gegenwärtiger Stand von Diagnostik and Therapie

Med. Klin.

Central nervous system involvement in Whipple's disease

J. Neuropathol. Exp. Neurol.

Whipple's disease: a review of the literature and report of fifteen patients

Virchows Arch. Path. Anat.

Encephalopathy in Whipple's disease. P, histochemical study

Neurology

Granulomatous encephalitis in Whipple's disease. Electron microscopic observations

Acta Neuropath. (Berl.)

Cerebral manifestations of Whipple's disease

Acta Neurol. Belg.

Ultrastructure of bacilli and the bacillary origin of the macrophagic inclusions in Whipple's disease

J. Gen. Microbiol.

Identification of the uncultured bacillus of Whipple's disease

N. Engl. J. Med.

Encephalitis with myoclonus in Whipple's disease

J. Neurol. Neurosurg. Psychiatry

Brain involvement in Whipple's disease. A case report

Acta Neuropalhol. (Berl.)

Neurologic disease in patients with treated Whipple's disease

Medicine

Whipple's disease confined to the brain: A case studied clinically and pathologically

J. Neurol. Neurosurg. Psychiatry

Whipple's disease confined to the central nervous system

Ann. Neurol.

Whipple's disease confined to the nervous system

J. Neurol. Neurosurg. Psychiatry

Whipple disease of the nervous system

Neurology

Whipple's disease confined to the CNS presenting with multiple intracerebral mass lesions

J. Neurol. Neurosurg. Psychiatry

Case report
Whipple's disease and the central nervous system a case report and a review of the literature