Klüver-Bucy syndrome was first diagnosed in humans in 1955, after a group
of people who had experienced temporal lobectomy. It is a rare
neuropsychiatry disease and of which little is understood about its
pathophysiological processes. Here we present a 60-year-old man seen in the
outpatient psychiatric department in a tertiary hospital in Kuala Lumpur
who presented to us with hyper sexuality, impulsivity, docility, amnesia and
hyperphagia for the past 10 months. He was diagnosed with Herpes Simplex
Virus with encephalitis 18 months ago and was shown to have bilateral
meningoencephalitis of his temporal lobes. Thus, a diagnosis of Klüver-Bucy
was made. We have taken a multi-disciplinary team approach to treat his
illness and specific goals has been laid out in each discipline. A written
consent has been taken by the patient and his family for the publication of
this report.
Adult-onset metachromatic leukodystrophy is often a diagnostic challenge to many clinicians. It may be presented with psychiatry symptom before other evidences of leukodystrophy are uncovered. We report a 53-year-old patient who presented with 7-year history of manic-like presentation in addition to progressive neurocognitive deterioration. Diagnosis was made eventually with neuroimaging. Mutational analysis showed compound heterozygous of ARSA gene. This case demonstrated the challenge in diagnosing this condition due to its complex neuropsychiatric presentation.
Background: Tuberculous disease of spine (spinal TB) is under-recognized in tuberculous (TB) meningitis.
The objective of the study was to evaluate the frequency, clinical and neuroimaging changes, and
outcome in the patients with spinal TB.
Methods: All the patients with spinal TB admitted in the two
largest tertiary hospitals in Kuala Lumpur from 2009 to 2017 were recruited, the clinical features were
documented, the magnetic resonance imaging (MRI) of the spine was performed. Clinical outcome was
assessed with Modified Rankin scale (MRS).
Results: Twenty two patients were recruited. This was
out of 70 TB meningitis patients (31.4%) seen over the same period. Eighteen (81.8%) patients had
concomitant TB meningitis. The clinical features consisted of systemic symptoms with fever (63.6%),
meningitis symptoms with altered sensorium (45.5%), myelopathy with paraparesis (36.4%). The
findings on spinal MRI were discitis (36.4%), spinal meningeal enhancement (31.8%), spinal cord
compression (31.8%), psoas abscess (27.3%), osteomyelitis (22.7%), and cord oedema (22.7%). All
except two patients (90.9%) had involvement in psoas muscle, bone or leptomeningeal enhancement,
features that can be used to differentiate from myelopathy that affect the parenchyma only, such as
demyelination. Unusual manifestations were syringomyelia and paradoxical manifestations seen in 3
patients each. The outcome were overall poor, with 68% having MRS 3 or more.
Conclusion: Spinal TB is common in TB meningitis. The outcome is overall poor. A heightened
awareness is crucial to enable early diagnosis and treatment.
Objective: The primary objective of this study was to describe the neuroimaging changes of tuberculous meningitis (TBM), and to determine the role of neuroimaging in the diagnosis of TBM.
Methods: Between January 2009 and July 2015, we prospectively recruited TBM patients in two hospitals in Malaysia. Neuroimaging was performed and findings were recorded. The control consists of other types of meningo-encephalitis seen over the same period.
Results: Fifty four TBM patients were recruited. Leptomeningeal enhancement was seen in 39 (72.2%) patients, commonly at prepontine cistern and interpeduncular fossa. Hydrocephalus was observed in 38 (70.4%) patients, 25 (46.3%) patients had moderate and severe hydrocephalus. Thirty four patients (63.0%) had cerebral infarction. Tuberculoma were seen in 29 (53.7%) patients; 27 (50.0%) patients had classical tuberculoma, 2 (3.7%) patients
had “other” type of tuberculoma, 18 (33.3%) patients had ≥5 tuberculoma, and 11 (20.4%) patients had < 5 tuberculoma. Fifteen (37.2%) patients had vasculitis, 6 (11.1%) patients had vasospasm. Close to nine tenth (88.9%) of the patients had ≥1 classical neuroimaging features, 77.8% had ≥ 2 classical imaging features of TBM (basal enhancement, hydrocephalus, basal ganglia / thalamic infarct, classical tuberculoma, and vasculitis/vasospasm). Only 4% with other types of meningitis/encephalitis had ≥1 feature, and 1% had two or more classical TBM neuroimaging features. The sensitivity of the imaging features of the imaging features for diagnosis of TBM was 88.9% and the specificity was 95.6%.
Conclusion: The classic imaging features of basal enhancement, hydrocephalus, basal ganglia/thalamic infarct, classic tuberculoma, and vasculitis are sensitive and specific to diagnosis of TBM.