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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 progression 2 (2006).Notes: Small ring-like post-gadolinium enhancement in the brain stem lesion also depicted in Figure 5. (Post-gadolinium SE T1 WI, transversal and sagittal).Abbreviations: MRI, magnetic resonance imaging; SE T1 WI, spin echo T1 weighted images.
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f6-ndt-11-2461: MRI at time of neurological progression 2 (2006).Notes: Small ring-like post-gadolinium enhancement in the brain stem lesion also depicted in Figure 5. (Post-gadolinium SE T1 WI, transversal and sagittal).Abbreviations: MRI, magnetic resonance imaging; SE T1 WI, spin echo T1 weighted images.

Mentions: In 2006, the patient suffered from recurrent diplopia and paresthesia of the right arm. Vertical gaze palsy again was observed, and additionally, paresis of the left abducens nerve was also detected. Brain MRI showed a new mass-like lesion in the brain stem with small ring-like, central, postgadolinium enhancement (Figures 5 and 6). The lesion previously observed in the right thalamus as well as hypothalamic infiltration had completely regressed. Remarkable regression of the initial mass-like lesion in the right temporal lobe was also noted. Spinal tap was again performed, and although the cerebrospinal fluid (CSF) was PCR-negative for WD, no other cause was found. Antibiotic treatment therefore remained unchanged (oral trimethoprim–sulfamethoxazole 160/800 mg bid).


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 progression 2 (2006).Notes: Small ring-like post-gadolinium enhancement in the brain stem lesion also depicted in Figure 5. (Post-gadolinium SE T1 WI, transversal and sagittal).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

f6-ndt-11-2461: MRI at time of neurological progression 2 (2006).Notes: Small ring-like post-gadolinium enhancement in the brain stem lesion also depicted in Figure 5. (Post-gadolinium SE T1 WI, transversal and sagittal).Abbreviations: MRI, magnetic resonance imaging; SE T1 WI, spin echo T1 weighted images.
Mentions: In 2006, the patient suffered from recurrent diplopia and paresthesia of the right arm. Vertical gaze palsy again was observed, and additionally, paresis of the left abducens nerve was also detected. Brain MRI showed a new mass-like lesion in the brain stem with small ring-like, central, postgadolinium enhancement (Figures 5 and 6). The lesion previously observed in the right thalamus as well as hypothalamic infiltration had completely regressed. Remarkable regression of the initial mass-like lesion in the right temporal lobe was also noted. Spinal tap was again performed, and although the cerebrospinal fluid (CSF) was PCR-negative for WD, no other cause was found. Antibiotic treatment therefore remained unchanged (oral trimethoprim–sulfamethoxazole 160/800 mg bid).

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