Cognitive and Behavioral Abnormalities in a Case of Central Nervous System Whipple Disease

Manzel, Kenneth; Tranel, Daniel; Cooper, Gregory S.

doi:10.1001/archneur.57.3.399

Cited by 32 publications

(16 citation statements)

References 23 publications

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“…They tend to progress insidiously and are usually accompanied by depression, personality and behavioral changes. Previous reports identified disabling impairment in the domains of sustained attention, memory, executive function and constructional praxis 15 . At later stages, these deficits completely fulfill criteria for dementia (much like Alzheimer´s disease) and steadily worsen prognosis.…”

Section: Discussionmentioning

confidence: 98%

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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Section: Discussionmentioning

confidence: 98%

Whipple's disease with neurological manifestions: case report

França

Castro

Balthazar

et al. 2004

Arq. Neuro-Psiquiatr.

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“…The cognitive changes are generally of insidious onset, and include memory loss, confusion, and impairments in attention, praxis, and executive function [7]. These cognitive changes are often associated with depression, personality changes, or other psychiatric disturbances (paranoia, disinhibition, or confabulation) [10]. In Louis' study [6], 47% of patients with cognitive symptoms also had psychiatric signs.…”

Section: Cns Whipple's Diseasementioning

confidence: 99%

“…Pretreatment deficits included impairments in anterograde and retrograde memory, temporal and autobiographical information, sustained attention, information processing speed, and visual-spatial functioning, as well as confabulation, poor impulse control, and perseverative behavior [10,11]. In the first case, after antibiotic treatment, the patient was noted to have significant improvement in orientation, attention, abstract thinking, and visual-spatial function, with some residual deficits in long-term memory [11], whereas in the second case the patient demonstrated improvements in attention and emotional control, but still continued to have significant impairment in anterograde memory, working memory, and executive functioning [10]. Due to the nonspecific nature of many of the cognitive and psychiatric symptoms in CNS Whipple's disease, it is easy for findings to be dismissed as consequences of aging or nonreversible dementia such as Alzheimer's disease [12].…”

Section: Cns Whipple's Diseasementioning

confidence: 99%

Neurologic manifestations of Whipple’s disease

Lugassy

Louis

2006

Curr Infect Dis Rep

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Whipple's disease is a rare, multisystem, infectious disorder caused by the bacterium Tropheryma whippelii. Patients with central nervous system (CNS) involvement may present in a variety of ways, including cognitive impairment, psychiatric manifestations, gaze palsies, upper motor neuron signs, and hypothalamic dysfunction. A tissue-based diagnosis of Whipple's disease is made based on the presence of periodic acid-Schiff-stained material or by the identification of bacterial RNA using polymerase chain reaction. To avoid relapse, patients should be treated with antimicrobial agents that penetrate the CNS, and therapy should be continued for at least 1 year.

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“…Die kognitiven Einbußen beginnen oft schleichend und lassen sich phänomenologisch nicht von [24]. ᭤Supranukleäre Augenbewegungsstörungen sind das zweithäufigste Symptom des zerebralen M.Whipple.…”

Section: Klinik Des Zentralen Mwhippleunclassified

“…Neuroradiologisch nachweisbare Veränderungen sind typischerweise im medialen Temporallappen, im Hypothalamus oder in der Pons zu finden, Signalabweichungen im Rückenmark sind die Ausnahme [4]. Unter einer Behandlung sind die Läsionen oft rückläufig [17,24]. Allerdings wurden auch Fälle mit zerebralem M. Whipple mit unauffälligem MRT beschrieben [33].…”

Section: Diagnostik Beim Zentralen Mwhippleunclassified

Neurologische Komplikationen bei entzündlichen Darmerkrankungen

2002

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Some inflammatory diseases of the gastrointestinal system are associated with neurological symptoms which, in rare cases, may precede the onset of the gastrointestinal manifestation of the disease. Celiac sprue is characterized by an intolerance to the wheat protein gluten. The typical neurological manifestation of celiac sprue is cerebellar ataxia. The ataxia as well as the gastrointestinal symptoms can be treated with a strictly gluten-free diet. The neurological symptoms of Whipple's disease, a disorder caused by gram-positive bacilli, are variable. Typical symptoms of cerebral Whipple's disease include dementia, ophthalmoplegia, movement disorders, and signs of hypothalamic dysfunction. Nowadays, the diagnosis of cerebral Whipple's disease is made by PCR detection of the bacilli in the CSF. Crohn's disease and ulcerative colitis are associated with neurological symptoms to a similar proportion. Various forms of polyneuropathies have been observed. The CNS manifestations of inflammatory bowel diseases include thromboembolic stroke, cerebral venous thrombosis, and cerebral vasculitis.

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Cognitive and Behavioral Abnormalities in a Case of Central Nervous System Whipple Disease

Cited by 32 publications

References 23 publications

Whipple's disease with neurological manifestions: case report

Whipple's disease with neurological manifestions: case report

Neurologic manifestations of Whipple’s disease

Neurologische Komplikationen bei entzündlichen Darmerkrankungen

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