Objective: Primary lateral sclerosis is a very uncommon progressive disease and represents 2-4% of motor neuron diseases group. It is characterized by spasticity because corticospinal and corticobulbar tracts impairment in midbrain and medulla. Magnetic resonance shows white matter hyperintensities in T2 weight sequence. When the impairment is symmetrical the image takes the appearance of a “wine glass”.

Background: Only one case was reported in 2013 with the “wine glass” sign where bilateral impairment of corticospinal pathway was related with another different diagnosis that primary lateral sclerosis or amyotrophic lateral sclerosis, like osmotic demyelination syndrome. Then, “wine glass” sign is not exclusive of the motor neuron diseases but these have very different clinical course.

Methods: A 56-year-old-male patient who two years ago presented behavior change, progressive tiredness, and weakness in lower and upper extremities until inability to walking and brushing that progressed to spastic quadriparesis and pseudobulbar syndrome without neither clinical and electrophysiological second motor neuron nor peripheral nerve impairment. The case meets the clinical diagnostic criteria of primary lateral sclerosis and frontotemporal dementia.

Results: Magnetic resonance images in coronal T2 weighted sequences showed symmetrical impairment of corticospinal pathway from centrum semiovale to medullary pyramids producing the “wine glass” appearance.

Conclusions: Our patient gets together the diagnostic criteria of Primary lateral sclerosis propose by Pringle because a slow and progressive clinical course characterized by spactic quadriplegic and pseudobulbar syndrome without clinical or neurophysiological impairment of second motor neuron nor peripheral nerve. Finally, motor neuron diseases and frontotemporal dementia would be part of the same entity because both share neuropathological and genetic aspects.

References: Parashari UC, Khanduri S, Bhadury S, Srivastava D, Saxena S. The magnetic resonance imaging “wine glass” sign of amyotrophic lateral sclerosis. Int J Nutr Pharmacol Neurol Dis 2011;1:206-8. Saroja AO, Naik KR, Mali RV, Kunam SR. ‘Wine Glass’ sign in recurrent postpartum hypernatremic osmotic cerebral demyelination. Ann Indian Acad Neurol 2013;16:106-10.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/wine-glass-appearance-in-a-primary-lateral-sclerosis-and-frontotemporal-dementia-case/