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2013 ; 92
(6
): 324-330
Nephropedia Template TP
gab.com Text
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English Wikipedia
Central nervous system involvement in Whipple disease: clinical study of 18
patients and long-term follow-up
#MMPMID24145700
Compain C
; Sacre K
; Puéchal X
; Klein I
; Vital-Durand D
; Houeto JL
; De Broucker T
; Raoult D
; Papo T
Medicine (Baltimore)
2013[Nov]; 92
(6
): 324-330
PMID24145700
show ga
Whipple disease (WD) is a rare multisystemic infection with a protean clinical
presentation. The central nervous system (CNS) is involved in 3 situations: CNS
involvement in classic WD, CNS relapse in previously treated WD, and isolated CNS
infection. We retrospectively analyzed clinical features, diagnostic workup,
brain imaging, cerebrospinal fluid (CSF) study, treatment, and follow-up data in
18 patients with WD and CNS infection. Ten men and 8 women were included with a
median age at diagnosis of 47 years (range, 30-56 yr). The median follow-up
duration was 6 years (range, 1-19 yr). As categorized in the 3 subgroups, 11
patients had classic WD with CNS involvement, 4 had an isolated CNS infection,
and 3 had a neurologic relapse of previously treated WD. CNS involvement occurred
during prolonged trimethoprim-sulfamethoxazole (TMP-SMX) treatment in 1 patient
with classic WD. The neurologic symptoms were various and always intermingled, as
follows: confusion or coma (17%) related to meningo-encephalitis or status
epilepticus; delirium (17%); cognitive impairment (61%) including memory loss and
attention defects or typical frontal lobe syndrome; hypersomnia (17%); abnormal
movements (myoclonus, choreiform movements, oculomasticatory myorhythmia) (39%);
cerebellar ataxia (11%); upper motor neuron (44%) or extrapyramidal symptoms
(33%); and ophthalmoplegia (17%) in conjunction or not with progressive
supranuclear palsy. No specific pattern was correlated with any subgroup. Brain
magnetic resonance imaging (MRI) revealed a unique focal lesion (35%), mostly as
a tumorlike brain lesion, or multifocal lesions (23%) involving the medial
temporal lobe, midbrain, hypothalamus, and thalamus. Periventricular diffuse
leukopathy (6%), diffuse cortical atrophy (18%), and pachymeningitis (12%) were
observed. The spinal cord was involved in 2 cases. MRI showed ischemic sequelae
at diagnosis or during follow-up in 4 patients. Brain MRI was normal despite
neurologic symptoms in 3 cases. CSF cytology was normal in 62% of patients,
whereas Tropheryma whipplei polymerase chain reaction (PCR) analysis was positive
in 92% of cases with tested CSF. Periodic acid-Schiff (PAS)-positive cells were
identified in cerebral biopsies of 4 patients. All patients were treated with
antimicrobial therapy for a mean duration of 2 years (range, 1-7 yr) with either
oral monotherapy (TMP-SMX, doxycycline, third-generation cephalosporins) or a
combination of antibiotics that sometimes followed parenteral treatment with
beta-lactams and aminoglycosides. Eight patients also received
hydroxychloroquine. At the end of follow-up, the clinical outcome was favorable
in 14 patients (78%), with mild to moderate sequelae in 9. Thirteen patients
(72%) had stopped treatment for an average time of 4 years (range, 0.7-14 yr).
Four patients had clinical worsening despite antimicrobial therapy; 2 of those
died following diffuse encephalitis (n = 1) and lung infection (n = 1). In
conclusion, the neurologic manifestations of WD are diverse and may mimic almost
any neurologic condition. Brain involvement may occur during or after TMP-SMX
treatment. CSF T. whipplei PCR analysis is a major tool for diagnosis and may be
positive in the absence of meningitis. Immune reconstitution syndrome may occur
in the early months of treatment. Late prognosis may be better than previously
reported, as a consequence of earlier diagnosis and a better use of antimicrobial
therapy, including hydroxychloroquine and doxycycline combination.
|Adult
[MESH]
|Aged
[MESH]
|Anti-Infective Agents/therapeutic use
[MESH]
|Central Nervous System Infections/cerebrospinal fluid/drug therapy/*epidemiology
[MESH]
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