Central Nervous System Relapse of Whipple's Disease

Uryu, Kiyoaki; Sakai, Takashi; Yamamoto, Tomonori; Mae, Takahito; Willard, Yoshie Iwasaki; Sugita, Hiroji; Asano, Koukichi; Yamashita, Kenichi; Harada, Hiromasa; Fukuda, Kou

doi:10.2169/internalmedicine.51.7304

Cited by 12 publications

(6 citation statements)

References 13 publications

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“…Given the broad differential diagnoses and the disease's rarity in Japan, Whipple's disease can be difficult to diagnose ( 6 , 9 ). The differential diagnoses include inflammatory rheumatic diseases, malabsorption with small intestine involvement (e.g., celiac disease, sarcoidosis, and lymphoma), Addison's disease, and connective tissue disorders ( 1 ).…”

Section: Discussionmentioning

confidence: 99%

“…We performed repeated follow-up gastrointestinal endoscopy, confirming that the changes in the duodenal mucosa improved in the early stage of treatment, whereas macrophage infiltration in the lamina propria of the duodenum persisted. Uryu et al reported a case with neurologic recurrence of Whipple's disease, where the accumulation of macrophages in the lamina propria of the duodenum continued for six years, even after the symptoms had disappeared ( 9 ). Although the relevance of this finding to the clinical course and risk of recurrence is unknown, it may suggest that macrophage replication and the anti-inflammatory function of the lamina propria remain abnormal.…”

Section: Discussionmentioning

confidence: 99%

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Whipple's Disease with Long-term Endoscopic Follow-up

et al. 2018

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A 72-year-old man presented with anorexia and 15-kg weight loss over 3 years. Endoscopy revealed yellow, shaggy mucosa alternating with erythematous, eroded mucosa in the duodenum. Biopsy specimens showed massive infiltration of periodic acid-Schiff-positive macrophages in the lamina propria, consistent with Whipple's disease. The patient was treated with intravenous ceftriaxone for four weeks, followed by oral trimethoprim-sulfamethoxazole. His condition improved, and he gradually gained weight. Although the endoscopic findings improved with continuous trimethoprim-sulfamethoxazole administration, macrophage infiltration of the duodenal mucosa persisted. However, the patient has been symptom-free for eight years.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Whipple's Disease with Long-term Endoscopic Follow-up

et al. 2018

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“…Whipple's disease (WhD) is a rare but treatable infectious disease from Tropheryma whipplei (TW), with an estimated incidence of 0.4 to 1.0 per 1,000,000 a year . The brain follows the gastrointestinal tract as the second major site of involvement, with CNS involvement on postmortem found in up to 50% of cases, most frequently in a periventricular and periaqueductal distribution .…”

mentioning

confidence: 99%

“…The brain follows the gastrointestinal tract as the second major site of involvement, with CNS involvement on postmortem found in up to 50% of cases, most frequently in a periventricular and periaqueductal distribution . The percentage of clinical CNS involvement varies considerably, ranging from 6% to 63%, with most estimates of 10%‐43%. In approximately 5% of cases, the initial manifestations are purely neurological .…”

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confidence: 99%

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

et al. 2018

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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

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“…Whipple disease was considered as a potential diagnosis, although only a few patients with this disease had been reported in Japan. 3 – 5 However, the absence of periodic acid-Schiff (PAS)–positive macrophages in duodenal biopsy argued against Tropheryma whipplei infection. Subsequently brain biopsy was performed and the potential infectious organism was ultrastructurally and genetically characterized.…”

Section: Methodsmentioning

confidence: 99%

New type of encephalomyelitis responsive to trimethoprim/sulfamethoxazole treatment in Japan

Sakiyama

Kanda

Higuchi

et al. 2015

Neurol Neuroimmunol Neuroinflamm

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Objective:To determine the causative pathogen and investigate the effective treatment of a new type of encephalomyelitis with an unknown pathogen in Japan and report the preliminary ultrastructural and genomic characterization of the causative agent.Methods:From 2005 to 2012, we treated 4 Japanese patients with geographic clustering and comparable clinical features, serum/CSF cytology, and radiologic findings. Brain biopsy was conducted in all patients to analyze neuropathologic changes by histology, and electron microscopy was applied to reveal the features of the putative pathogen. Genomic DNA was obtained from the affected brain tissues and CSF, and an unbiased high-throughput sequencing approach was used to screen for specific genomic sequences indicative of the pathogen origin.Results:All patients exhibited progressive dementia with involuntary tongue movements. Cytologic examination of CSF revealed elevated mononuclear cells. Abnormal MRI signals were observed in temporal lobes, subcortical white matter, and spinal cord. Biopsied brain tissue exhibited aggregated periodic acid-Schiff–positive macrophages and 2–7 μm diameter round/oval bodies without nuclei or cell walls scattered around the vessels. Unbiased high-throughput sequencing identified more than 100 archaea-specific DNA fragments. All patients were responsive to trimethoprim/sulfamethoxazole (TMP-SMX) plus corticosteroid therapy.Conclusions:We report 4 cases of encephalomyelitis due to an unknown pathogen. On the basis of ultrastructural and genomic studies, we propose a new disease entity resulting from a causative pathogen having archaeal features. TMP-SMX therapy was effective against this new type of encephalomyelitis.

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Central Nervous System Relapse of Whipple's Disease

Cited by 12 publications

References 13 publications

Whipple's Disease with Long-term Endoscopic Follow-up

Whipple's Disease with Long-term Endoscopic Follow-up

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

New type of encephalomyelitis responsive to trimethoprim/sulfamethoxazole treatment in Japan

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