|Year : 2011 | Volume
| Issue : 2 | Page : 206-208
The magnetic resonance imaging "wine glass" sign of amyotrophic lateral sclerosis
Umesh C Parashari, Sachin Khanduri, Samarjit Bhadury, Divyanka Srivastava, Sugandha Saxena
Department of Radiodiagnosis, Era's Lucknow Medical College, Lucknow, Uttar Pradesh, India
|Date of Submission||02-May-2011|
|Date of Acceptance||25-May-2011|
|Date of Web Publication||23-Aug-2011|
Umesh C Parashari
Department of Radiodiagnosis, Era's Lucknow Medical College, Lucknow, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Amyotrophic lateral sclerosis is a disease of neurons characterized by progressive muscular paralysis due to degeneration of motor neurons in the primary motor cortex, corticospinal tracts, brainstem, and spinal cord. We present the case of a 40-year-old patient having progressive muscle weakness, limb and truncal atrophy, and bulbar signs and with classical finding of "wine glass" appearance on magnetic resonance imaging.
Keywords: Amyotrophic lateral sclerosis; brain MRI; progressive disease; t2 hyper intensities, wine glass appearance
|How to cite this article:|
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
|How to cite this URL:|
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 [serial online] 2011 [cited 2019 Jul 20];1:206-8. Available from: http://www.ijnpnd.com/text.asp?2011/1/2/206/84217
| Introduction|| |
Amyotrophic lateral sclerosis (ALS) is a fatal disease. It is a disease of an unknown cause characterized by slowly progressive degeneration of upper and lower motor neurons. Degeneration of these neurons causes muscle weakness and muscle atrophy, usually starting from the hands and arms and then spreading to other parts of the body. Thereafter, difficulty with speaking, swallowing, and breathing ensues. Mean age at diagnosis is 57 years. Disease progression is relentless, half the patients die within 3 years and 90% die by 6 years following symptoms onset.  As it is a progressive disease, so there is no known cure or way of reversing its effect. The other name of this disease is Lou Gehrig's disease and Charcoat's disease.
| Case Report|| |
We present the case of a 40-year-old patient who presented with progressive weakness in both arms and difficulty in walking. Subsequently, the patient developed difficulty in swallowing and speaking. On physical examination, stretch reflexes in upper extremities were normal but hyper in lower. There was associated hypertonia, diminished fine motor movement, and muscle wasting. Babinski sign was absent. Fasciculation were present.
Laboratory studies of serum and CSF (cerebrospinal fluid)were normal and rest of biochemical, hematological and serological investigations were also normal. Magnetic resonance imaging (MRI) of brain was performed on our 1.5 T GE scanner. T2-weighted sequences showed symmetric bilateral hyper intensities along large myelinated pyramidal tract fibers in the posterior limb of the internal capsule and cerebral peduncle, extending along the corticospinal tract from centrum semiovale to crus cerebri bilaterally [Figure 1]. On coronal T2WI hyperintensity with in pyramidal tracts resembled "wine glass" appearance [Figure 2]. Bansal et al described similar appearance simulating "garland." 
|Figure 1: Axial FLAIR image showing bilateral symmetrical hyperintensity (thin white arrow) (a) in corona radiata (more prominent on left side); (b) posterior limb of internal capsule; (c) along corticospinal tracts at the level of crus cerebri and (d) in pons.|
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|Figure 2: Coronal T2WI showing bilateral symmetrical hyperintensity along corticospinal tract (thin white arrows) forming a 'wine glass appearance'.|
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| Discussion|| |
In 1969, Dr. Jean Martin Charcoat was first to identify that motor neurons from the spinal cord are affected most in ALS. So it was named as Maladie de Charcoat. In 1941, Lou Gehrig a famous basketball player died of ALS. Hence, his name has also been associated with disease. ALS is a neurological disease that involves the degeneration of motor neurons. A-myo-trophic is derived from Greek language, where "A" means something negative, "myo" stands for muscle and "trophic" means nourishment. The term lateral mainly focused on the parts of spinal cord that are affected. Sclerosis implies the scarring that result from the degeneration of neurons in the spinal cord. It occurs in normally two major forms familial or sporadic. In familial, it is due to mutation of gene that produce copper/zinc superoxide dismutase (SOD1), an enzyme responsible for scavenging free radicals. The person will inherit the gene SOD-1 from one of their parents. In sporadic there is still no cause. It could be genetic or environmental.
ALS is derived from the combination of clinical examination findings with pathological finding of lateral sclerosis. Causes that result in degeneration of the motor system are primary lateral sclerosis, progressive bulbar palsy, and progressive muscular atrophy. Primary lateral sclerosis involves the lateral corticospinal tracts and manifests clinically as upper motor neuron signs. Progressive bulbar palsy is progressive dysphasia and dysarthria syndrome without other generalized findings. Progressive muscular atrophy is a disorder of the primary anterior horn cell destruction with isolated lower motor neuron findings on examination.
No ethnic or racial predisposition to ALS is seen worldwide. ALS is a fatal disease with median survival of 3-5 years. It affects all races equally and is much more prominent in men. Age of onset is between 40-60 years.
Neurological examination presents specific signs associated with upper and lower motor neuron degeneration. The clinical evaluation of a patient with suspected ALS or motor neuron disease (MND) should include neuroimaging to identify treatable conditions mimicking ALS. Neuroimaging study is an important tool. MRI can be used in revealing lesions in corticospinal tracts in ALS.  The earliest MR manifestation is hyperintensity on T2WI in the corticospinal tracts, seen earliest in the internal capsule, as the fibers are most concentrated here. Thereafter, the entire tract from motor strip to the spinal cord is affected with increased T2 signal and loss of volume.  T2WI, MRI of brain shows characteristic symmetrical hyperintensities of the corticospinal tracts bilaterally, extending from the internal capsule to the brain stem producing a "wine glass" appearance on coronal sections (as in our case). All these findings are subjected to areas of Wallerian degeneration More Details due to neuronal loss and hence consistent with the diagnosis of ALS. For early diagnosis there are MR spectroscopy, diffusion weighted imaging, diffusion tensor imaging that help in identifying disease changes earlier. Long TE proton spectroscopy may reveal significantly decreased NA/Cr values consistent with neuronal dysfunction and/or loss.
The treatment of patient depends completely on the symptomatic treatment.  There should be ventilator management, nutritional management, and drug-related treatment. Counseling and palliative care should be taken sideways. 
| References|| |
|1.||Brafman BH, Trojanowski JQ, Atlas W. The aging brain and neurodegenerative disorders. In Atlas SW, editor. Magnetic resonance imaging of the brain and spine. New York: Raven Press;1991. p.567-624. |
|2.||Bansal AR, Dash GK, Radhakrishnan A, Kesavadas C, Nair M. 'Garland sign' in amyotrophic lateral sclerosis. Neurol India 2009;57:354-5. |
|3.||Vance C, Rogelj B, Hortobágyi T, De Vos KJ, Nishimura AL, Sreedharan J, et al. "Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6". Science 2009;323:1208-11. |
|4.||Waragai M. MRI and clinical features in amyotrophic lateral sclerosis. Neuroradiology 1997;39:847-51. |
|5.||Turner MR, Kiernan MC, Leigh PN, Talbot K. Biomarker in amyotrophic lateral sclerosis. The Lancet Neurol 2009;8:94-109. |
|6.||Averill AJ, Kasarskis EJ, Segerstrom SC. Psychological health in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2007;8:243-54. |
|7.||Mitsumoto H, Rabkin JG. Palliative care for patients with amyotrophic lateral sclerosis" prepare for the worst and hope for the best". JAMA 2007;298:207-16. |
[Figure 1], [Figure 2]