LETTER TO THE EDITOR
|Year : 2016 | Volume
| Issue : 4 | Page : 535-536
A case of "main en succulente" in high cervical myelopathy
Sheetal Sajan, Madhusudanan Mohan, Reji Thomas, Finu Mathew Baby
Department of Neurology, Pushpagiri Medical College and Research Institute, Tiruvalla, Kerala, India
|Date of Submission||11-Feb-2016|
|Date of Decision||01-Mar-2016|
|Date of Acceptance||04-Mar-2016|
|Date of Web Publication||18-Oct-2016|
Department of Neurology, Pushpagiri Medical College and Research Institute, Tiruvalla, Kerala
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sajan S, Mohan M, Thomas R, Baby FM. A case of "main en succulente" in high cervical myelopathy. Ann Indian Acad Neurol 2016;19:535-6
A 50-year-old man presented to us with history of burning pain of his entire left upper limb for the past 10 years. About 7 years, after the onset of symptoms, he noticed similar burning pain in the right lower extremity. He also developed difficulty in walking with a tendency to trip over and fall. On examination, his left hand was soft, smooth, swollen, and cold with shiny skin ("main en succulente") [Figure 1]a-c. His right hand appeared normal. There was spasticity of both lower limbs. In the left upper limb, there was marked weakness of all small muscles with Grade IV power of left triceps and finger extensors. There was no wasting of the small muscles of left hand. Rest of the muscles was normal in the left upper limb. The power in the other limbs was also normal. Examination of deep tendon reflexes showed brisk triceps with normal biceps and supinator jerks. Lower limb reflexes were brisk with bilateral extensor plantars. Abdominal reflex was not elicitable. On sensory examination, he had impaired pain and temperature perception over both upper limbs below mid forearm as well as over the entire right lower limb. Severe impairment of joint and position sense was noticed in the left hand as well as both lower limbs. Left hand showed pseudoathetosis. Romberg's test was positive, and gait was spastic. Nerve conduction study as well as electromyography was done in both upper limbs and was found to be normal.
|Figure 1: (a-c) Shiny and edematous left hand compared with the normal right hand as shown in different positions of hand|
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Magnetic resonance imaging of the cervical spine revealed a central disc protrusion at C4-C5 level with myelomalacic changes [Figure 2]a and b. There was no evidence of a central cord lesion in the spinal segments corresponding to the swollen left hand. X-ray examination of the cervicothoracic region did not reveal any cervical ribs.
|Figure 2: (a and b) Magnetic resonance imaging of the cervical spine showing central disc protrusion at C4-C5 level with myelomalacic changes|
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Clinical picture in our case suggested a high cervical myelopathy. Presence of "main en succulente" pointed to a possible intramedullary lesion as this phenomenon has been described exclusively with intramedullary lesions such as syringomyelia. However, neuroimaging revealed a central disc protrusion at C4-C5 level with cord compression and myelomalacic changes at the same level, with no evidence of any central cord lesion in the spinal segments corresponding to the swollen left hand. In the literature that we reviewed, there was no mention about "main en succulente" with high cervical compressive myelopathy.
"Main en succulente" was first described by Marinesco to refer to the swollen hand seen in syringomyelia.  It was thought to be due to trophic changes in the hand and is postulated to be due to the involvement of the central autonomic pathways. The mechanism underlying the manifestation of "main en succulente," in our case with high cervical cord compression is not clear. A plausible explanation in our case may be affection of the central part of the spinal cord at C8-T1 level implicating the crossing pain fibers and the central autonomic pathways. This is corroborated by the fact that patient had associated loss of pain and temperature on the left hand, the site of "main en succulente." How the central cord is affected on the left side due to high cervical lesion is a matter of debate; probably it may be related to stagnant hypoxia in the central cord region due to the compression of the draining veins from the lower cervical region, akin to the pathogenetic mechanism offered to the anterior horn cell seen loss in high cervical extramedullary compression. 
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| References|| |
Marinesco G. Main Succulente et Atrophic Musculaire Dans Ia Syringomyelia. M.D. Thesis. University of Paris; 1897. p. 241.
Larner AJ. False localising signs. J Neurol Neurosurg Psychiatry 2003;74:415-8.
[Figure 1], [Figure 2]