|LETTER TO THE EDITOR
|Year : 2019 | Volume
| Issue : 3 | Page : 176-177
“Wine Glass” sign in a case of juvenile amyotrophic lateral sclerosis
Arvind Vyas, Divya Goel
Department of Neurology, SMS Hospital, Jaipur, Rajasthan, India
|Date of Submission||20-May-2019|
|Date of Decision||20-May-2019|
|Date of Acceptance||21-May-2019|
|Date of Web Publication||26-Jun-2019|
Dr. Divya Goel
4Ba19, Jawahar Nagar, Jaipur - 302 004, Rajasthan
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Vyas A, Goel D. “Wine Glass” sign in a case of juvenile amyotrophic lateral sclerosis. Indian J Med Spec 2019;10:176-7
|How to cite this URL:|
Vyas A, Goel D. “Wine Glass” sign in a case of juvenile amyotrophic lateral sclerosis. Indian J Med Spec [serial online] 2019 [cited 2022 Dec 9];10:176-7. Available from: http://www.ijms.in/text.asp?2019/10/3/176/264534
Amyotrophic lateral sclerosis (ALS) is a form of motor neuron disease characterized by the degeneration of upper and lower motor neurons. The mean age of onset is 57 years. Juvenile ALS is reserved for the patients 25 years of age or less and is characterized by a prolonged survival. Here, we present a case of a 24-year-old male who presented with a history of subacute onset weakness of all four limbs for the past 1 year starting with left lower limb manifesting as difficulty in clearing off the foot from the ground. This was followed by a similar involvement of the right foot after 4–5 months. There was difficulty in gripping objects along with thinning of muscles and guttering noted between the thumb and the first dorsal interosseous. The patient started experiencing difficulty in swallowing, nasal regurgitation of fluids along with nasal twang to his voice. There was no associated sensory complaint, bowel, or bladder involvement. On examination, the vitals were normal. On neurological examination, jaw jerk was brisk. There was wasting of posterior fibers of deltoid, anterior compartments of forearms bilaterally, interossei, chiefly the first dorsal interosseous and calf muscles. Generalized spasticity was present. The muscle power was MRC grade 3/5 and 4/5 in the right and left upper limbs, respectively, 3/5 in lower limbs bilaterally. Deep tendon reflexes were exaggerated and bilateral Babinski sign present. Sensory and cerebellar examination was unremarkable. The patient was subjected to neurophysiological studies; electromyography revealing neurogenic affliction with multiple fasciculations. Magnetic resonance imaging (MRI) of the brain revealed linear, bilaterally symmetrical hyperintensities [Figure 1]a, [Figure 1]b, [Figure 1]c involving the corticospinal tracts in internal capsule, crus cerebri and pons on T2-weighted image (T2WI), giving a “wine glass” appearance, seen in the coronal plane [Figure 1]d. ALS is a neurodegenerative disease characterized by the involvement of both upper and LMN and is diagnosed using revised El Escorial criteria. It has conventionally been diagnosed on the basis of clinical and electromyographic data. Motor neurons undergo degeneration and result in axonal edema apparent on electron microscopy. About 90% of ALS cases are sporadic and 10% familial (majority being juvenile ALS). Juvenile ALS is inherited as autosomal recessive in majority mapped to chromosome regions 2q33 and 15q12-21. Some are autosomal dominant mapped to chromosome 9q34. Neuroimaging till date has been of limited use in diagnosing juvenile ALS. Kumar et al. in his case report, found the typical wine glass appearance in a 9-year-old male presenting with both upper motor neurons and LMN features. Midani et al. showed bilateral hyperintensities along the corticospinal tracts in T2WI MRI in a patient with juvenile ALS. There have been several studies conducted on adult patients of motor neuron disease showing their MRI changes., However, juvenile ALS being a rare entity does not have much literature on its MRI changes. In this case, we suggest that the involvement of corticospinal tracts results in a typical wine glass pattern in the coronal section of brain MRI, posing a diagnostic utility in these cases.
|Figure 1: (a) Magnetic resonance imaging brain axial section showing bilaterally symmetrical hyperintensities involving the corticospinal tracts in internal capsule. (b) Magnetic resonance imaging brain axial section showing bilaterally symmetrical hyperintensities involving the corticospinal tracts in crus cerebri. (c) Magnetic resonance imaging brain axial section showing bilaterally symmetrical hyperintensities involving the corticospinal tracts in pons. (d) Magnetic resonance imaging brain coronal section showing bilaterally symmetrical hyperintensities involving the corticospinal tracts giving a “wine glass” appearance|
Click here to view
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Brooks BR. El Escorial world federation of neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on motor neuron diseases/amyotrophic lateral sclerosis of the world federation of neurology research group on neuromuscular diseases and the el escorial “Clinical limits of amyotrophic lateral sclerosis” workshop contributors. J Neurol Sci 1994;124 Suppl 1:96-107.
Okamoto K, Hirai S, Shoji M, Senoh Y, Yamazaki T. Axonal swellings in the corticospinal tracts in amyotrophic lateral sclerosis. Acta Neuropathol 1990;80:222-6.
Hentati A, Bejaoui K, Pericak-Vance MA, Hentati F, Speer MC, Hung WY, et al.
Linkage of recessive familial amyotrophic lateral sclerosis to chromosome 2q33-q35. Nat Genet 1994;7:425-8.
Kumar S, Aga P, Gupta A, Kohli N. Juvenile amyotrophic lateral sclerosis: Classical wine glass sign on magnetic resonance imaging. J Pediatr Neurosci 2016;11:56-7.
] [Full text]
Midani H, Truwit CL, Parry GJ. MRI in juvenile ALS: A patient report. Neurology 1998;50:1879-81.
Goodin DS, Rowley HA, Olney RK. Magnetic resonance imaging in amyotrophic lateral sclerosis. Ann Neurol 1988;23:418-20.
Luís ML, Hormigo A, Maurício C, Alves MM, Serrão R. Magnetic resonance imaging in motor neuron disease. J Neurol 1990;237:471-4.