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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

Last MRI (2015).Notes: On the last MRI, diffuse brain atrophy and severe hippocampal atrophy/sclerosis were found. (TSE T2 WI, coronal).Abbreviations: MRI, magnetic resonance imaging; TSE T2 WI, turbo spin echo T2 weighted images.
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f7-ndt-11-2461: Last MRI (2015).Notes: On the last MRI, diffuse brain atrophy and severe hippocampal atrophy/sclerosis were found. (TSE T2 WI, coronal).Abbreviations: MRI, magnetic resonance imaging; TSE T2 WI, turbo spin echo T2 weighted images.

Mentions: In December 2014, the patient was found unconscious by her family. Upon admission to the hospital, she was severely dehydrated with hyperosmolarity, metabolic acidosis, and hypotension. After rehydration and correction of the acid–base dysbalance, she regained consciousness. Right-sided abducens paresis in addition to her previous ocular palsies and severe cognitive impairment was found. She suffered from short- and long-term memory loss and had retrograde amnesia spanning several months prior to the new episode. MRI showed diffuse brain atrophy and severe atrophy of the right hippocampus with hippocampal sclerosis (Figure 7). All enhancing lesions on MRI had regressed, only postinflammatory gliosis in the right temporal lobe and a small periaqueductal pseudocyst were found. Oral antibiotic treatment with trimethoprim–sulfamethoxazole 160/800 mg bid was reestablished. Unfortunately, her neurological status thus far (April 2015) remains the same. The patient signed the informed consent and agreed with publishing medical data in scientific literature in anonymous form. This case report was approved by the ethics committee of Na Homolce Hospital, Prague, Czech Republic.


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

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

Last MRI (2015).Notes: On the last MRI, diffuse brain atrophy and severe hippocampal atrophy/sclerosis were found. (TSE T2 WI, coronal).Abbreviations: MRI, magnetic resonance imaging; 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

f7-ndt-11-2461: Last MRI (2015).Notes: On the last MRI, diffuse brain atrophy and severe hippocampal atrophy/sclerosis were found. (TSE T2 WI, coronal).Abbreviations: MRI, magnetic resonance imaging; TSE T2 WI, turbo spin echo T2 weighted images.
Mentions: In December 2014, the patient was found unconscious by her family. Upon admission to the hospital, she was severely dehydrated with hyperosmolarity, metabolic acidosis, and hypotension. After rehydration and correction of the acid–base dysbalance, she regained consciousness. Right-sided abducens paresis in addition to her previous ocular palsies and severe cognitive impairment was found. She suffered from short- and long-term memory loss and had retrograde amnesia spanning several months prior to the new episode. MRI showed diffuse brain atrophy and severe atrophy of the right hippocampus with hippocampal sclerosis (Figure 7). All enhancing lesions on MRI had regressed, only postinflammatory gliosis in the right temporal lobe and a small periaqueductal pseudocyst were found. Oral antibiotic treatment with trimethoprim–sulfamethoxazole 160/800 mg bid was reestablished. Unfortunately, her neurological status thus far (April 2015) remains the same. The patient signed the informed consent and agreed with publishing medical data in scientific literature in anonymous form. This case report was approved by the ethics committee of Na Homolce Hospital, Prague, Czech Republic.

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