Wine Glass Sign in Bulbar Onset Amyotrophic Lateral Sclerosis

A 46-year-old man presented with progressive changes in voice, swallowing difficulty with nasal regurgitation of fluids, and spells of excessive crying/laughing for 8 months. There was no associated sensory complaint or bowel or bladder involvement. He denied muscle loss, fasciculation, cognitive impairment, or a family history of similar illness. On examination, the patient had spastic dysarthria and pseudobulbar palsy. The motor system assessment revealed normal power with grade 3 spasticity (modified Ashworth scale) in the lower extremities and grade 2 spasticity in the upper extremities. Deep tendon reflexes were exaggerated (grade 3+). The jaw jerk was brisk, with a bilateral extensor plantar response. Electromyography (EMG) revealed a neurogenic pattern with grade 3 fasciculations in the right flexor digitorum indices, genioglossus, and vastus lateralis muscle. The autoimmune and viral markers were negative. The repetitive nerve stimulation test was normal. Acetylcholinesterase receptor antibodies and anti-muscle-specific kinase antibodies were negative. The cerebrospinal fluid examination was unremarkable. Magnetic resonance imaging (MRI) of the brain revealed linear, bilaterally symmetrical hyperintensities (Figs 1-3) involving the corticospinal tracts (CST) in the internal capsule, crus cerebri and pons on T2-weighted imaging, revealing a “wine glass” appearance in the coronal plane (Fig 4). CST hyperintensity was graded as hyperintensity positive.1 MRI of the spine was normal. The patient met the Gold Coast criteria for diagnosing amyotrophic lateral sclerosis (ALS), involving both upper (UMN), and lower motor neurons. Therefore, the diagnosis of bulbar-onset ALS was made based on clinical, neuroimaging, and EMG findings. The MRI results in our case were unique, indicating symmetrical degeneration of the CST in a linear pattern, resulting in a classic “wine glass” appearance. The CST typically has a uniform width throughout its course. In ALS, the CST typically constricts at its junction with the pyramids in the medulla oblongata, forming a wine glass shape. This narrowing is the outcome of motor neuron degeneration, demyelination, and axonal injury in the CST.2 This imaging finding can also be observed in primary CNS lymphoma, osmotic myelinosis, prior hemorrhage, ischemic white matter disease, vitamin B12 deficiency, and demyelinating diseases, including neuromyelitis optica spectrum disorder. CST hyperintensity can be used to evaluate UMN degradation in ALS regardless of age, disease duration, and region of onset, particularly in clinical situations where UMN signs can be masked by remarkable muscle atrophy.3, 4 This case report highlights the unique radiological signs that can be used to evaluate UMN degeneration in ALS patients. These radiological abnormalities may assist in delineating clinical and genetic variability within this category of illnesses. Assessment of UMN degeneration for ALS is often limited in clinical examinations. Combining CST hyperintensity and clinical examination can effectively improve the sensitivity of diagnostic performance for UMN degeneration in ALS patients. The extent of narrowing may correspond with the severity of ALS and can be used to track disease progression over time. As a result, although the appearance of wine glass is an essential signal in screening patients with suspected ALS, it must be considered alongside other clinical and diagnostic data. We thank the patients and their families for their participation in this study. The author expresses gratitude to Dr Shivangi Tiwari for assisting him. P.B. and A.R.P. contributed to the conception and design of the study; P.B. contributed to the acquisition and analysis of data; P.B. and A.R.P. contributed to drafting the text or preparing the figures. Nothing to report. Deidentified patient data used for this study are available on request.