Intravenous immunoglobulins as first-line therapy for IgLON5 encephalopathy
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A 56-year-old man presented with a 6 month history of dysarthrophonia, orofacial dyskinesia and chorea of both hands. His wife reported snoring, day sleepiness, insomnia and limb movements during sleep. The patient and his wife negated a cognitive impairment at the time of presentation. Mood was unimpaired.
Neurological examination showed oculomotor dysfunction (saccadic pursuit, absent vertical optokinetic nystagmus and delayed horizontal optokinetic nystagmus in routine examination), dysarthrophonia, orofacial dyskinesia and chorea.
The patient scored 28 of 30 points on the Montreal Cognitive Assessment Score (MoCA). Additional neuropsychological assessment showed abnormalities in alertness, information processing speed, selective attention and verbal memory. Unified Huntington’s Disease Rating Scale motor score (UHDRS-MS), used as a measure for hyperkinesia, was 17 of 124 points.
Cerebral MRI showed a macroadenoma of the pituitary gland but was otherwise unremarkable....
The authors thank J Enczmann for testing the HLA types of the patient and for giving the permission for publishing these results.
Compliance with ethical standards
Conflicts of interest
The authors have no conflicts of interests to report.
The study was performed in accordance with the Declaration of Helsinki.
The patient gave his consent to publish the case report.