Case presentation
A 49-year-old woman was admitted to the neurology department of a
teaching hospital with progressive complaints of gait disturbance,
urinary incontinence, apathy, bradyphrenia and short-term memory
impairment. Within 5-6 weeks she became unable to walk. Her medical
history revealed hypertension, dyslipidemia, transient ischemic attacks
(TIA’s) and a pontine stroke three years earlier. She never fully
recovered from her pontine stroke with persistent complaints of
cognitive impairment, fatigue and some difficulty walking. Computed
tomography (CT) was performed (fig 1 matter lesions and suspicion of
vasculitis related findings) showing quadriventricular hydrocephalus
compared to imaging one year earlier, as well as confluent white matter
changes, most notably in the deep white matter of both frontal lobes.
Repeated lumbar punctures with normal opening pressures (10-13 mmHg)
temporarily improved her clinical condition, which was objectified with
a gait analysis and the cognitive complaints and incontinence improved
subjectively as well. The cerebral spinal fluid (CSF) analysis showed a
mild lymphocytic pleocytosis (16*10E6/l), with an
erythrocyte count of 0, normal glucose (2,2 mmol/l), slightly elevated
proteins (0,94 g/l), albumin (655mg/l) and IgG (98 mg/l), intrathecal
igG was 0mg/ml, with 5 IgG oligoclonal bands in CSF and 2 in serum. Cell
count showed 0% polymorphonuclear cells and 100% mononuclear cells.
Infectious and immunologic screening in CSF was negative.
Magnetic resonance imaging (MRI) of the brain confirmed the
hydrocephalus and frontal white matter changes, but also showed
progressive white matter changes in the other lobes, basal nuclei and
cerebellum compared to earlier imaging. On sagittal imaging, CSF
pulsation artifacts could be appreciated in the Sylvian aqueduct, but
not in the foramen magnum or around the median or lateral apertures of
the fourth ventricle. Together with dilatation of the fourth ventricle
and ‘ballooning’ of both lateral apertures, this suggested an
obstruction of the fourth ventricle CSF-outflow (fig 2).
Contrast-enhanced MRI was performed later, showing linear and patchy
enhancement within the posterior cranial fossa and basal regions of the
brain (fig 3). The patient was referred to the neurosurgery department
for ventriculoperitoneal shunt (VPS) placement. Intracranial pressure
appeared to be slightly elevated during surgery, although not measured.
Analysis of peroperative obtained CSF showed no malignant cells; a
fluorescence-activated cell sorting (FACS) -analysis could not be
performed due to the low cell-count. Due to the differential diagnosis
of leptomeningeal metastases, basal leptomeningitis (including
granulomatous disease), vasculitis, lymphoma, auto-immune or
paraneoplastic pathologies, a PET-CT was performed. The PET-CT showed
supraclavicular, mediastinal and parailliacal lymphadenopathy. An
ultrasound-guided puncture was performed in the supraclavicular lymph
node. Histological examination showed a granulomatous disease with a
strong preference for sarcoidosis (fig 4). This was confirmed by a
second puncture of one of the PET-positive lymph nodes. Both a Mantoux
test and a PCR for Mycobacterium tuberculosis proved negative and
tuberculosis was thus excluded.