No abstract available.
Chorea ; Creutzfeldt-Jakob Syndrome

Background & Objectives: Arachnoiditis which involves the optic chiasm and optic nervecan rarely occurs in the patients with tuberculous meningitis (TBM). The primary objective of this study was to determine the incidence, assess the clinical and neuroimaging findings, and associations, understand its pathogenesis of these patients, and determine its prognosis. Methods: The patients admitted with TBM in the neurology wards of two tertiary care hospitals from 2009 to 2017 in Kuala Lumpur, Malaysia were screened. The patients with OCA and optochiasmatic tuberculoma were included in this study. We assessed the clinical, cerebrospinal fluid (CSF), imaging findings of the study subjects and compared with other patients without OCA or optochiasmatic tuberculoma. Results: Eighty-eight patients with TBM were seen during the study period. Seven (8.0%) had OCA and one (1.1%) had optochiasmatic tuberculoma. Five out of seven (71.4%) patients with OCA were newly diagnosed cases of TBM. The other two (28.6%) had involvement while on treatment with antituberculous treatment (paradoxical manifestation). The mean age of the patients with OCA was 27.3 ± 11.7. All the OCA patients had leptomeningeal enhancement at other sites. All had hydrocephalus and cerebral infarcts on brain neuroimaging. Three (42.9%) patients had cerebral tuberculoma at sites other than suprasellar and optic chiasm areas. On univariate analysis, the presence of OCA and optochiasmatic tuberculoma was associated with raised CSF opening pressure (p=0.014), younger age (p=0.024), cerebral infarcts (p=0.018) and hydrocephalus (p= 0.046). There was no statistically significant association on logistic regression. Only one (14.3%) patient had visual impairment. Conclusion: OCA and optochiasmatic tuberculoma were seen in 9% of a cohort of Malaysian TBM patients. They were more likely to be younger, have raised CSF opening pressure, cerebral infarcts and hydrocephalus, suggesting the association with a more severe exudative disease.

Moyamoya disease (MMD) was first described in 1957 as “bilateral hypoplasia of internal carotid arteries (ICAs)”.1 Aside from involving the intracranial arteries, MMD can also affect extracranial ICAs and external carotid arteries (ECAs).2-4 High resolution magnetic resonance (MR) vessel wall imaging (VWI) is increasingly being used to help with the diagnosis and characterization of the condition focusing mainly on intracranial vessels and extracranial ICAs.5-9 We present a case of a young woman with MMD, demonstrating vessel wall enhancement of nonstenotic maxillary branches of bilateral ECAs.

Childhood chronic inflammatory demyelinating polyneuropathy (CIDP) can be misdiagnosed for the more common genetic neuropathies such as Charcot-Marie-Tooth (CMT) disease. We present a case of childhood-onset demyelinating polyneuropathy who was initially diagnosed as CMT before a revised diagnosis of CIDP was made. A 14-year-old boy with bilateral pes cavus presented with progressive history of ataxic gait, generalized areflexia and proprioceptive sensory loss. Nerve conduction studies showed demyelinating features including markedly slow motor conduction velocities and prolonged distal motor latencies resembling CMT1. Despite the absence of a family history of genetic neuropathies, a diagnosis of CMT1 was considered most likely. The patient presented two years later with an acute onset of worsening instability and muscle weakness. A detailed history revealed functional improvement following the last presentation along with two separate episodes of exacerbations suggesting a relapsingremitting form of neuropathy. Cerebrospinal fluid analysis showed cytoalbuminergic dissociation. Nerve ultrasound demonstrated enlarged peripheral nerves, particularly in the proximal and non-entrapment sites. Genetic testing was negative for known mutations in common CMT genes. A course of intravenous immunoglobulin resulted in clinically significant improvement. In conclusion, our patient highlights the diagnostic challenges in childhood-onset demyelinating neuropathies and the importance of not missing a potentially treatable immune-mediated neuropathy.