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|LETTERS TO EDITOR
|Year : 2019 | Volume
| Issue : 4 | Page : 1140-1141
Chiari Type I Malformation with Syringomyelia Presenting with Isolated Hemi-anhidrosis: Report of a Rare Case
Gautam Dutta, Daljit Singh, Anita Jagetia, Manish Garg, Mohd Iqbal, AK Srivastava
Department of Neuro-Surgery, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India
|Date of Web Publication||10-Sep-2019|
Dr. Gautam Dutta
Department of Neuro-Surgery, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi - 110 002
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Dutta G, Singh D, Jagetia A, Garg M, Iqbal M, Srivastava A K. Chiari Type I Malformation with Syringomyelia Presenting with Isolated Hemi-anhidrosis: Report of a Rare Case. Neurol India 2019;67:1140-1
|How to cite this URL:|
Dutta G, Singh D, Jagetia A, Garg M, Iqbal M, Srivastava A K. Chiari Type I Malformation with Syringomyelia Presenting with Isolated Hemi-anhidrosis: Report of a Rare Case. Neurol India [serial online] 2019 [cited 2020 Nov 24];67:1140-1. Available from: https://www.neurologyindia.com/text.asp?2019/67/4/1140/266256
Chiari type I malformation, classically described as inferior displacement of the cerebellar tonsils and sometimes, the inferior vermis through the foramen magnum into the rostral cervical canal is associated with syrinx formation in 25%-65% of the cases. Formation of syrinx results from obstruction of cerebrospinal fluid circulation in the cisterna magna and it has been noted that the presence of syrinx is more commonly associated with symptoms and sometimes an unusual association with holocord syrinx may be seen. These patients commonly present with weakness and atrophy of the limbs. Although Horner's syndrome has been seen in 4%-6% of patients undergoing surgical decompression for the disease, isolated Horner's syndrome as a presenting feature of Chiari type I malformation with syringomyelia is uncommon [Table 1]. We report on a 25-year-old male patient with an isolated hemi-anhidrosis who was found to have a Chiari I malformation and syringomyelia. This is by far the second case reported to have such association.
|Table 1: Cases of syringomyelia with isolated hemi.anhidrosis (1 case) and Hornerfs syndrome (5 cases) reported in the literature|
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A 25-year-old non-diabetic non-hypertensive male patient was referred to our department for evaluation and management of anhidrosis of left side of the body. The patient gave a history of mild to moderate neck pain along with an absence of sweating on the left side of the body. There was no history of trauma. There were no associated symptoms of dizziness or difficulty in swallowing. On examination, he was a healthy appearing male noted to have dryness of the left side of the face, neck and trunk and the left upper and lower limbs without any evidence of sweating. Minor test (starch-iodine test) was performed after a bout of physical exercise which confirmed anhidrosis on left side of the body. Neurological examination was all unremarkable with no limb weakness, wasting, numbness, temperature impairment or ptosis of the involved side or any evidence of facial or pupillary asymmetry. Routine lab tests and chest X-ray were within normal limits. Magnetic resonance imaging (MRI) spine revealed herniation of cerebellar tonsils with long segment T2/STIR hyperintense signal with dilatation of central canal from lower border of C2 up to mid-dorsal spine compatible with Chiari type I malformation with syringomyelia [Figure 1]a and [Figure 1]b. Patient underwent midline sub-occipital craniectomy and foramen magnum decompression. At operation, the cerebellar tonsils were found to be descended up to C2 and were elongated and compressed. Free flow of CSF was seen after decompression.
|Figure 1: (a): Cerebellar tonsillar herniation (red arrow) with holocord syrinx (green arrows) starting from the level of C2. (b): Syrinx extending up to mid-dorsal spine (green arrows)|
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The postoperative course was uneventful and patient was discharged on eighth postoperative day. At the time of this report (1-month follow-up), a minor test was still indicative of hemi-anhidrosis although the dryness over the involved area had improved significantly. He is currently under follow-up on a regular basis.
More than half of all cases of Chiari type I malformation are associated with syringomyelia, which is a development fluid-filled cavity or syrinx within the spinal cord. The causation of syringomyelia has been a focus of considerable controversy. The putative factors include craniovertebral junction malformation or secondary to trauma or tumour or it may be idiopathic.
The primary thermoregulatory center first reported in the late 1800s, is located within the pre-optic hypothalamic regions of the brain, and based on human anatomical data, the neural pathway to the sweat glands is thought to be as follows: efferent signals from the pre-optic hypothalamus travel via the tegmentum of the pons and the medullary raphe, crossing over to the regions of intermediolateral cell column of the spinal cord. In the spinal cord, neurons emerge from the ventral horn, pass through the white ramus communicans and then synapse in the sympathetic ganglia. Postganglionic non-myelinated C-fibres pass through the gray ramus communicans, combine with peripheral nerves and travel to sweat glands, with these nerve fibres entwined around the periglandular tissue of the eccrine sweat gland. Thus, a lesion in the medulla could result in contralateral or ipsilateral anhidrosis, and a lesion distal to the medulla will cause ipsilateral anhidrosis. It is thought that in the clinical course of syringomyelia, when slowly progressive tissue damage around the syrinx reaches the lateral horn, it segmentally affects the sympathetic preganglionic neurons (SPGNs) causing disinhibition of the inhibitory local interneurons (ILINs), which are located in the vicinity of SPGNs, resulting in hyperhidrosis. Sudo et al. proposed that initially hyperactivity of the SPGNs occurs as long as the disability is mild; however, as the disability progresses, the hyperactivity gradually decreases and is replaced by hypoactivity, which results in anhidrosis. Although this hypothesis looks appealing, in our case, however, there was no evidence of preceding hyperhidrosis suggesting alternate mechanism might co-exist behind isolated anhidrosis. Extensive Medline search for publications dealing with anhidrosis in syringomyelia returned scanty literature. Although Horner's syndrome with segmental anhidrosis in association with syringomyelia has been described, there is report of only one case  of hemi-anhidrosis till now, making the current case the second one. The probable hypothesis of anhidrosis in our case may be due to enlarging syrinx causing compression and ischemia to the preganglionic neurons causing hypoactivity and autonomic suppression, however, more studies are required to explain the mechanism in such patients.
There is no study in literature to outline the plan of management of such patients primarily because of scarcity of cases. Based on our experience of the current case, we feel that surgical decompression might be helpful to provide the patient with a quality life. However, long-term follow-up is needed to assess the success of a surgical intervention.
Treating physicians need to be aware of the various ways a patient of syringomyelia may present and we propose that in diagnosing hemi-anhidrosis, attention should be given to the possibility of syringomyelia. The current case demonstrates that thorough evaluation followed by surgical decompression may improve symptoms but long-term follow-up is warranted to evaluate the outcome.
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.
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