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| LETTER TO EDITOR |
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| Year : 2012 | Volume
: 60
| Issue : 1 | Page : 110-111 |
"Wine-glass appearance" of pyramidal tracts in a patient with primary lateral sclerosis
Vimal Kumar Paliwal1, Sushil Kumar Rahi1, Prabhat Singh1, Rakesh Kumar Gupta2
1 Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, Uttar Pradesh, India
2 Department of Radiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, Uttar Pradesh, India
| Date of Submission | 17-Jan-2012 |
| Date of Decision | 18-Jan-2012 |
| Date of Acceptance | 22-Jan-2012 |
| Date of Web Publication | 7-Mar-2012 |
Correspondence Address:
Vimal Kumar Paliwal
Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, Uttar Pradesh
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.93606
How to cite this article:
Paliwal VK, Rahi SK, Singh P, Gupta RK. "Wine-glass appearance" of pyramidal tracts in a patient with primary lateral sclerosis. Neurol India 2012;60:110-1 |
Sir,
A 45-year-old gentleman presented with progressive weakness of all four limbs, change in voice, swallowing difficulty and spells of excessive crying/laughing since 15 years. He denied any muscle loss, fasciculation, memory impairment, bladder symptoms or family history of similar illness. Patient never consumed 'khesri dal' (Lathyrus sativus). On examination, he had spastic dysarthria and pseudobulbar palsy. Motor system revealed spastic quadriparesis, (Grade 3/5 power) and Grade 4 spasticity (modified Ashworth scale) in all four limbs. All deep-tendon jerks were brisk and sustained ankle/patellar clonus was present. Palmar grasp, Meyerson's sign, palmomental and snout reflexes were present. There was no cognitive decline, tongue/limb muscle atrophy, fasciculation, sensory loss or skin changes. Electromyography failed to reveal signs of lower motor neuron involvement. Cerebrospinal fluid examination including venereal disease research laboratory test was normal. Enzyme-linked immunosorbent assay for human immunodeficiency virus, serum vitamin B12 levels, serological tests for human T-lymphotropic virus-1, arylsulphatase-A levels and slit-lamp examination for Kaysar-Fleischer ring did not reveal any abnormality. Magnetic resonance imaging (MRI) showed confluent, symmetrical white matter hyperintensity extending from pons up to internal capsule producing a "wine-glass" appearance [Figure 1]a. Symmetrical involvement of the posterior limb of the internal capsule and pontine tegmentum was also noted [Figure 1]b and c. MRI cervical spine was normal. | Figure 1: (a) MRI brain T2-weighted coronal section showing hyperintense pyramidal tracts extending from the pons up to the periventricular region. (b) Axial T2-FLAIR sections showing hyperintense posterior limb of the internal capsule and (c) pontine tegmentum
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Our patient fulfilled the diagnostic criteria for primary lateral sclerosis (PLS). [1] PLS is a rare disease and accounts for 2-4% of all motor neuron diseases. Radiology may help to differentiate amyotrophic lateral sclerosis (ALS) from PLS. [2],[3] Focal hyperintensities of the internal capsule and brainstem are seen in ALS but confluent sclerosis of the pyramidal tract is not observed as seen in PLS using MRI or advanced imaging techniques like diffusion tensor imaging. [2],[3],[4],[5] Severe longstanding pathology only confined to the pyramidal tract possibly results in pyramidal tract sclerosis in PLS thereby giving a "wine-glass" appearance. [3] In the absence of a definite image-biomarker to differentiate ALS from PLS, this MRI appearance may add to the diagnosis of PLS.
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| 1. | Pringle CE, Hudson AJ, Munoz DG, Kiernan JA, Brown WF, Ebers GC. Primary lateral sclerosis. Clinical features, neuropathology and diagnostic criteria. Brain 1992;115:495-520. 
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| 2. | Wang S, Melhem ER. Amyotrophic lateral sclerosis and primary lateral sclerosis: The role of diffusion tensor imagin g and other advanced MR-based techniques as objective upper motor neuron markers. Ann N Y Acad Sci 2005;1064:61-77.
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| 3. | Chan S, Kaufmann P, Shungu DC, Mitsumoto H. Amyotrophic lateral sclerosis and primary lateral sclerosis: Evidence-based diagnostic evaluation of the upper motor neuron. Neuroimaging Clin N Am 2003;13:307-26.
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| 4. | Kuruvilla A, Joseph S. 'Wine glass' appearance: A unique MRI observation in a case of primary lateral sclerosis. Neurol India 2002;50:306-9.
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| 5. | Qiu JT, Shang XL. Magnetic resonance imaging and diffusion tensor imaging in primary lateral sclerosis. Neurol India 2011;59:767-8.
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