Lhermitte-Duclos disease presenting with atypical positional nystagmus.
We describe a patient with dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) who presented with an acute onset of positional disequilibrium. Video-oculography in the right Hallpike position revealed rightward torsional down-beat nystagmus, initially thought to be right anterior canal benign positional vertigo. However, the presence of spontaneous nystagmus, the persistent character of the positional nystagmus and the absence of fatigability indicated central positional nystagmus, attributable to his right-sided Lhermitte-Duclos disease. These findings emphasise the need for clinicians to reconsider a central cause before diagnosing the rare anterior canal benign positioning vertigo variant.
Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Central Clinical School, University of Sydney, Missenden Road, Camperdown, Sydney, NSW 2050, Australia.
Lucy G
LG
Williams
Miranda Neurology, Miranda, NSW, Australia.
Peter
P
Brimage
Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Central Clinical School, University of Sydney, Missenden Road, Camperdown, Sydney, NSW 2050, Australia.
Corinna
C
Lechner
Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Central Clinical School, University of Sydney, Missenden Road, Camperdown, Sydney, NSW 2050, Australia.
Rachael L
RL
Taylor
Brain & Mind Research Institute, Camperdown, NSW, Australia.
Lynette
L
Masters
Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Central Clinical School, University of Sydney, Missenden Road, Camperdown, Sydney, NSW 2050, Australia. Electronic address: miriam@icn.usyd.edu.au.
Miriam S
MS
Welgampola
D001921:Brain / Q000473:pathology; D003937:Diagnosis, Differential; D053483:Eye Movement Measurements; D019788:Fluorodeoxyglucose F18; D006223:Hamartoma Syndrome, Multiple / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D064847:Multimodal Imaging; D009759:Nystagmus, Pathologic / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology*; D009760:Nystagmus, Physiologic; D049268:Positron-Emission Tomography; D019275:Radiopharmaceuticals; D014057:Tomography, X-Ray Computed; D014741:Video Recording
D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18
Anterior canal benign positioning vertigo; Eye movements; Lhermitte–Duclos disease; Nystagmus