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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

MRI at time of neurological onset 1 (2005).Notes: A small round lesion is situated in the right thalamus with tiny dot-like central post-gadolinium enhancement (arrow). (Native TSE T2 WI, transversal; post-gadolinium SE T1 WI, coronal).Abbreviations: MRI, magnetic resonance imaging; SE T1 WI, spin echo T1 weighted images; TSE T2 WI, turbo spin echo T2 weighted images.
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f3-ndt-11-2461: MRI at time of neurological onset 1 (2005).Notes: A small round lesion is situated in the right thalamus with tiny dot-like central post-gadolinium enhancement (arrow). (Native TSE T2 WI, transversal; post-gadolinium SE T1 WI, coronal).Abbreviations: MRI, magnetic resonance imaging; SE T1 WI, spin echo T1 weighted images; TSE T2 WI, turbo spin echo T2 weighted images.

Mentions: In 2005, the patient developed diplopia despite ongoing oral antibiotic treatment. Neurological investigation revealed vertical gaze paresis. Brain MRI was then performed, which showed partial regression of the temporal lesion, no significant change of the hypothalamic lesion, and two new lesions. One small lesion was situated in the right thalamus with discrete, central postgadolinium enhancement (Figure 3). The second new lesion had an infiltrative appearance and surrounded the cerebral aqueduct in the mesencephalon; discrete dot-like enhancement was also apparent (Figure 4). Antibiotic treatment remained unchanged and the patient’s symptoms improved after a few days.


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

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

MRI at time of neurological onset 1 (2005).Notes: A small round lesion is situated in the right thalamus with tiny dot-like central post-gadolinium enhancement (arrow). (Native TSE T2 WI, transversal; post-gadolinium SE T1 WI, coronal).Abbreviations: MRI, magnetic resonance imaging; SE T1 WI, spin echo T1 weighted images; TSE T2 WI, turbo spin echo T2 weighted images.
© Copyright Policy
Related In: Results  -  Collection

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

f3-ndt-11-2461: MRI at time of neurological onset 1 (2005).Notes: A small round lesion is situated in the right thalamus with tiny dot-like central post-gadolinium enhancement (arrow). (Native TSE T2 WI, transversal; post-gadolinium SE T1 WI, coronal).Abbreviations: MRI, magnetic resonance imaging; SE T1 WI, spin echo T1 weighted images; TSE T2 WI, turbo spin echo T2 weighted images.
Mentions: In 2005, the patient developed diplopia despite ongoing oral antibiotic treatment. Neurological investigation revealed vertical gaze paresis. Brain MRI was then performed, which showed partial regression of the temporal lesion, no significant change of the hypothalamic lesion, and two new lesions. One small lesion was situated in the right thalamus with discrete, central postgadolinium enhancement (Figure 3). The second new lesion had an infiltrative appearance and surrounded the cerebral aqueduct in the mesencephalon; discrete dot-like enhancement was also apparent (Figure 4). Antibiotic treatment remained unchanged and the patient’s symptoms improved after a few days.

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