Whipple's disease confined to the central nervous system

Coene, Beatrijs De; Duprez, Thierry; Gilliard, Claude; Indekeu, P.; Trigaux, Jean-Paul

doi:10.1007/bf00596579

Cited by 26 publications

(20 citation statements)

References 12 publications

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“…Occasional inclusions within Schwann cells, probably resulting from the late complications in the CNS, have also been reported (46,50). Neurologic symptoms may appear with or without gastrointestinal (30,31,38,46,49,50,59,210) or joint (39) manifestations. After initial successful treatment for intestinal manifestations, a number of patients relapsed with progressive neurologic involvement (22,168,197,198).…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Whipple's Disease and “Tropheryma whippelii”

2001

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SUMMARY Whipple's disease is a rare bacterial infection that may involve any organ system in the body. It occurs primarily in Caucasian males older than 40 years. The gastrointestinal tract is the most frequently involved organ, with manifestations such as abdominal pain, malabsorption syndrome with diarrhea, and weight loss. Other signs include low-grade fever, lymphadenopathy, skin hyperpigmentation, endocarditis, pleuritis, seronegative arthritis, uveitis, spondylodiscitis, and neurological manifestations, and these signs may occur in the absence of gastrointestinal manifestations. Due to the wide variability of manifestations, clinical diagnosis is very difficult and is often made only years or even decades after the initial symptoms have appeared. Trimethoprim-sulfamethoxazole for at least 1 year is usually considered adequate to eradicate the infection. The microbiological diagnosis of this insidious disease is rendered difficult by the virtual lack of culture and serodiagnostic methods. It is usually based on the demonstration of periodic acid-Schiff-positive particles in infected tissues and/or the presence of bacteria with an unusual trilaminar cell wall ultrastructure by electron microscopy. Recently, the Whipple bacteria have been characterized at the molecular level by amplification of their 16S rRNA gene(s). Phylogenetic analysis of these sequences revealed a new bacterial species related to the actinomycete branch which was named “Tropheryma whippelli.” Based on its unique 16S ribosomal DNA (rDNA) sequence, species-specific primers were selected for the detection of the organism in clinical specimens by PCR. This technique is currently used as one of the standard methods for establishing the diagnosis of Whipple's disease. Specific and broad-spectrum PCR amplifications mainly but not exclusively from extraintestinal specimens have significantly improved diagnosis, being more sensitive than histopathologic analysis. However, “T. whippelii” DNA has also been found in persons without clinical and histological evidence of Whipple's disease. It is unclear whether these patients are true asymptomatic carriers or whether differences in virulence exist among strains of “T. whippelii” that might account for the variable clinical manifestations. So far, six different “T. whippelii” subtypes have been found by analysis of their 16S-23S rDNA spacer region. Further studies of the pathogen “T. whippelii” as well as the host immune response are needed to fully understand this fascinating disease. The recent cultivation of the organisms is a promising major step in this direction.

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Section: Clinical Manifestationsmentioning

confidence: 99%

Whipple's Disease and “Tropheryma whippelii”

2001

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“…Such relapses of WD were frequently reported when initial antibiotic treatment had not been adequate and frequently included cerebral manifestations. Short-term use of SMZ-TMP or tetracycline (which does not cross the blood-brain barrier) were particularly related to CNS relapses 11,12 and a worse clinical course. Feldman et al 13 reported a patient disclosing acute meningoencephalitis during a relapse of the disease whose abnormalities closely recalled our own patient.…”

Section: Discussionmentioning

confidence: 99%

“…A few cases presenting with rapidly evolving cranial hypertension were reported, most of them caused by tumorlike lesions or acute hydrocephalus (Sylvius aqueduct stenosis) 3,11 . Various forms of peripheral neuropathy have been observed as in other chronic inflammatory bowel diseases, probably associated to malabsorptive nutritional deficiencies 20 .…”

Section: Discussionmentioning

confidence: 99%

Whipple's disease with neurological manifestions: case report

França

Castro

Balthazar

et al. 2004

Arq. Neuro-Psiquiatr.

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-Whipple´s disease (WD) is an uncommon multisystem condition caused by the bacillus Tropheryma whipplei. Central nervous system involvement is a classical feature of the disease observed in 20 to 40% of the patients. We report the case of a 62 yeards old man with WD that developed neurological manifestations during its course, and discuss the most usual signs and symptoms focusing on recent diagnostic criteria and novel treatment regimens.KEY WORDS: Whipple´s disease, inflammatory bowel disease, dementia, polyneuropathy. Doença de Whipple com manifestações neurológicas: relato de casoRESUMO -A doença de Whipple (DW) é distúrbio multissistêmico raro causado pelo bacilo Tropheryma whipplei. O envolvimento do sistema nervoso central é um aspecto clássico da doença, sendo observado em 20 a 40% dos pacientes. Relatamos o caso de homem de 62 anos com DW que desenvolveu manifestações neurológicas durante sua evolução, com o objetivo de discutir os sinais e sintomas mais comuns e destacar os critérios diagnósticos e propostas terapêuticas mais recentes. PALAVRAS-CHAVE: doença de Whipple, doença inflamatória intestinal, demência, polineuropatiaWhipple´s disease (WD) or intestinal lypodystrophy was first described in 1907 1 . It is a relatively rare multisystem disorder caused by the gram-positive bacillus Tropheryma whipplei.The usual presenting complaints include pronounced weight loss, mal-absorptive diarrhea (sometimes accompanied by abdominal cramping and bloody stool), recurrent non-deforming polyarthritis and longstanding low grade fever 2 . Cutaneous hyperpigmentation and lymphadenopathy are also common clinical signs. Central nervous system (CNS) involvement is a classical feature of WD observed in 20 to 40% of cases. CNS manifestations are myriad and usually develop in later stages of the illness 3 , often with cranial nerve and cognitive complaints. Approximately 5% of the patients follow an unusual presentation with isolated CNS symptoms 4 . Available data on CNS WD are scant, consisting basically of isolated case reports. Gerard et al. 3 found only 122 reported cases in the literature since 1960, most of them in Europe and North America. To our knowledge, the following case is the first to be reported in Brazil. CASEA 62-year-old white man, retired electrician, came to our Gastroenterologic Clinic in 1989 complaining of increased stool frequency and liquidity, associated with pronounced weight loss (20 Kg) in the last 2 years. Subsequent investigation disclosed a mal-absorptive syndrome, steatorrhoea and hepatosplenomegaly. Six months later, he developed bilateral knee arthritis which subsided spontaneously in two weeks. In 1990, an upper digestive endoscopy combined with multiple gastric and duodenal biopsies was performed. Histologic examination of the bowel specimens revealed flattened villi, small intestinal mucosa laden with distended foamy macrophages in the lamina propria and intracellular periodic acid Schiff (PAS) positive granules ( Figure 1A -1B). Acid-fast bacilli were not identified in Z...

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“…These can include speech disturbances, aphasia, partial seizures, hemiparesis, dysarthria, facial palsy, and upper motor neuron signs including increased tone, hyperactive reflexes and extensor plantar reflexes [20,24,25]. Unilateral clonic movements have also been described [13].…”

Section: Clinical Presentation Of Cerebral Whipple's Diseasementioning

confidence: 99%