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Primary Whipple disease of the brain: case report with long-term clinical and MRI follow-up.

Peregrin J, Malikova H - Neuropsychiatr Dis Treat (2015)

Bottom Line: The first neurological manifestations developed 2 years later despite recommended antibiotic treatment, with cognitive impairment developing more than 10 years later.According to the MRI findings and clinical course, the disease was active for several years when multiple lesions on MRI appeared despite antibiotic therapy.In the discussion, we compare the present case with similar cases previously reported and we elaborate on the similarities and discrepancies in clinical features, diagnostic procedures, results, and treatment options.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Charles University, Prague, Czech Republic.

ABSTRACT
Whipple disease (WD) is a rare systemic disorder caused by the bacteria Tropheryma whipplei. In its classic form, it manifests with gastrointestinal problems including diarrhea, abdominal pain, and weight loss. However, various other systems can be affected, including the central nervous system (CNS). Even more rarely, the CNS is primarily affected without gastrointestinal symptoms and with a negative small bowel biopsy. The incidence of primary CNS WD is unknown. We report the case of a young female with the primary CNS form of WD. In this report, we highlight the main clinical features and diagnostic procedures that lead to the diagnosis and comment on the treatment and clinical response. We stress the importance of neuroimaging and brain biopsy. A unique feature of this case is that the patient has been followed up for 12 years. At the time of diagnosis, no neurological manifestations were detected, although a tumor-like lesion in the right temporal lobe and hypothalamic infiltration were present on magnetic resonance imaging (MRI). The first neurological manifestations developed 2 years later despite recommended antibiotic treatment, with cognitive impairment developing more than 10 years later. According to the MRI findings and clinical course, the disease was active for several years when multiple lesions on MRI appeared despite antibiotic therapy. In the discussion, we compare the present case with similar cases previously reported and we elaborate on the similarities and discrepancies in clinical features, diagnostic procedures, results, and treatment options.

No MeSH data available.


Related in: MedlinePlus

Initial post-gadolinium MRI (2003).Notes: There is nonhomogenous irregular post-gadolinium enhancement in the mass-like lesion. Also, enhancing infiltration in the hypothalamus is clearly depicted. (SE T1 WI after gadolinium enhancement, transversal).Abbreviations: MRI, magnetic resonance imaging; SE T1 WI, spin echo T1 weighted images.
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f2-ndt-11-2461: Initial post-gadolinium MRI (2003).Notes: There is nonhomogenous irregular post-gadolinium enhancement in the mass-like lesion. Also, enhancing infiltration in the hypothalamus is clearly depicted. (SE T1 WI after gadolinium enhancement, transversal).Abbreviations: MRI, magnetic resonance imaging; SE T1 WI, spin echo T1 weighted images.

Mentions: The 33-year-old Caucasian female was first admitted to our hospital in 2003. At that time, the patient had a history of two spontaneous abortions followed by amenorrhea and galactorrhea, and also a history of migratory arthralgia of the large joints. She suffered from nonspecific symptoms including lower back pain accompanied by mild fever. Despite a lack of neurological manifestations, the patient underwent several investigations including brain computerized tomography (CT) and MRI, which surprisingly showed a mass-like lesion in the right temporal lobe with cystic and infiltrative portions that partially enhanced following intravenous (IV) gadolinium administration. Additionally, a smaller lesion was detected in the hypothalamus (Figures 1 and 2). Glioma was suspected based on the imaging results, and stereotactic biopsy of the lesion was performed. Histological analyses revealed granular, foamy, periodic acid–Schiff (PAS)-positive macrophages with no signs of tumor. WD was suspected and then confirmed at a referential histopathological laboratory (Bonn, Germany) using PAS staining technique. For WD confirmation, the patient underwent a thorough gastroenterological evaluation including repeated duodenal biopsy; all biopsies were deep; histology was done with correct PAS staining and the specimens examined by experienced pathologists were found to be negative. The diagnosis of primary CNS WD was postulated and was supported by spinal tap, which showed polymerase chain reaction (PCR)-positive results for WD. Antibiotic treatment was initiated, first with IV ceftriaxone 2 g bid for 2 weeks followed by oral trimethoprim–sulfamethoxazole 160/800 mg bid. The fever subsided with treatment; however, the patient still suffered from lower back pain.


Primary Whipple disease of the brain: case report with long-term clinical and MRI follow-up.

Peregrin J, Malikova H - Neuropsychiatr Dis Treat (2015)

Initial post-gadolinium MRI (2003).Notes: There is nonhomogenous irregular post-gadolinium enhancement in the mass-like lesion. Also, enhancing infiltration in the hypothalamus is clearly depicted. (SE T1 WI after gadolinium enhancement, transversal).Abbreviations: MRI, magnetic resonance imaging; SE T1 WI, spin echo T1 weighted images.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4590553&req=5

f2-ndt-11-2461: Initial post-gadolinium MRI (2003).Notes: There is nonhomogenous irregular post-gadolinium enhancement in the mass-like lesion. Also, enhancing infiltration in the hypothalamus is clearly depicted. (SE T1 WI after gadolinium enhancement, transversal).Abbreviations: MRI, magnetic resonance imaging; SE T1 WI, spin echo T1 weighted images.
Mentions: The 33-year-old Caucasian female was first admitted to our hospital in 2003. At that time, the patient had a history of two spontaneous abortions followed by amenorrhea and galactorrhea, and also a history of migratory arthralgia of the large joints. She suffered from nonspecific symptoms including lower back pain accompanied by mild fever. Despite a lack of neurological manifestations, the patient underwent several investigations including brain computerized tomography (CT) and MRI, which surprisingly showed a mass-like lesion in the right temporal lobe with cystic and infiltrative portions that partially enhanced following intravenous (IV) gadolinium administration. Additionally, a smaller lesion was detected in the hypothalamus (Figures 1 and 2). Glioma was suspected based on the imaging results, and stereotactic biopsy of the lesion was performed. Histological analyses revealed granular, foamy, periodic acid–Schiff (PAS)-positive macrophages with no signs of tumor. WD was suspected and then confirmed at a referential histopathological laboratory (Bonn, Germany) using PAS staining technique. For WD confirmation, the patient underwent a thorough gastroenterological evaluation including repeated duodenal biopsy; all biopsies were deep; histology was done with correct PAS staining and the specimens examined by experienced pathologists were found to be negative. The diagnosis of primary CNS WD was postulated and was supported by spinal tap, which showed polymerase chain reaction (PCR)-positive results for WD. Antibiotic treatment was initiated, first with IV ceftriaxone 2 g bid for 2 weeks followed by oral trimethoprim–sulfamethoxazole 160/800 mg bid. The fever subsided with treatment; however, the patient still suffered from lower back pain.

Bottom Line: The first neurological manifestations developed 2 years later despite recommended antibiotic treatment, with cognitive impairment developing more than 10 years later.According to the MRI findings and clinical course, the disease was active for several years when multiple lesions on MRI appeared despite antibiotic therapy.In the discussion, we compare the present case with similar cases previously reported and we elaborate on the similarities and discrepancies in clinical features, diagnostic procedures, results, and treatment options.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Charles University, Prague, Czech Republic.

ABSTRACT
Whipple disease (WD) is a rare systemic disorder caused by the bacteria Tropheryma whipplei. In its classic form, it manifests with gastrointestinal problems including diarrhea, abdominal pain, and weight loss. However, various other systems can be affected, including the central nervous system (CNS). Even more rarely, the CNS is primarily affected without gastrointestinal symptoms and with a negative small bowel biopsy. The incidence of primary CNS WD is unknown. We report the case of a young female with the primary CNS form of WD. In this report, we highlight the main clinical features and diagnostic procedures that lead to the diagnosis and comment on the treatment and clinical response. We stress the importance of neuroimaging and brain biopsy. A unique feature of this case is that the patient has been followed up for 12 years. At the time of diagnosis, no neurological manifestations were detected, although a tumor-like lesion in the right temporal lobe and hypothalamic infiltration were present on magnetic resonance imaging (MRI). The first neurological manifestations developed 2 years later despite recommended antibiotic treatment, with cognitive impairment developing more than 10 years later. According to the MRI findings and clinical course, the disease was active for several years when multiple lesions on MRI appeared despite antibiotic therapy. In the discussion, we compare the present case with similar cases previously reported and we elaborate on the similarities and discrepancies in clinical features, diagnostic procedures, results, and treatment options.

No MeSH data available.


Related in: MedlinePlus