Hyperhidrosis As the Presenting Symptom in Post-Traumatic Syringomyelia*

Hyperhidrosis as the presenting symptom in post-traumatic syringomyelia*

FE Glasauer MDlt J J Czyrny MD2

1 Department of Neurosurgery, 2 Department of Rehabilitation Medicine, State University of New York at Buffalo School of Medicine and Biophysical Sciences, Buffalo, New York, USA.

Post-traumatic syringomyelia is now a well known entity and occurs months or years after a spinal cord injury. The presenting symptoms are usually pain, progressive motor weakness, sensory changes, and increased spasticity. Profuse sweating or hyperhidrosis can be a symptom of the post-traumatic syrinx or can occur in autonomic dysreflexia provoked by peripheral stimuli. We present two patients with cervical spine fractures whose presenting symptom of post traumatic syringomyelia was hyperhidrosis affected by posture. The pathophysio logy involved and the management of th ese patients is discussed.

Keywords: hyperhidrosis; post-traumatic syringomyelia; autonomic dysreflexia.

Introduction autonomic hyperreflexia due to bladder sphincter dyssynergia, which was ultimately Profuse sweating or hyperhidrosis (HH ) is successfully treated with a suprapubic catheter. one of the many complications th at can In 1987 he developed severe HH which al occur after spinal cord injury. It may occur ways occurred when sitting upright and was as an isolated symptom without any associ relieved in the recumbent position. The sweat ated et iology, as part of the autonomic ing was more pronounced in the face and upper hyperreflexia syndrome, or as a manifesta extremities than in the trunk or the lower extremities. The HH was accompanied by a tion of post-traumatic syringomyelia (PTS).I right-sided Horner's syndrome but no other Two cases are presented in which the first manifestations of autonomic hyperreflexia. and predominant symptom of PT S was Gastrointestinal and genitourinary investiga postural induced HH . tions were negative and there was no skin breakdown. An MRI on 11/30/87 revealed a post Case reports traumatic syrinx extending from C4-CS to T7 -T8. (Figs la, b) Subsequently, he under Case 1 went CS-C6 and TS-T6 laminectomies with This patient was a 17 year old male who placement of a syringo-subarachnoid shunt. sustained a CS vertebral burst fracture while Intraoperative ultrasound at the cervical level playing ice hockey on 12/7/83. At his initial showed collapse of the cyst. Postoperatively, presentation he was a CS complete, Frankel's A there was significant improvement in the pa quadriplegic. He underwent a posterior cervical tient's symptom and the right Horner's syn fusion with iliac bone graft, followed by halo drome disappeared. No MRI was performed. immobilization. Over time he gained one motor The patient did well until 1989 when he again level to become a C6 level quadriplegic. The developed HH in the upright position associ patient's subsequent course was complicated by ated this time with a mild increase in spasticity and recurrence of the right Horner's syndrome. An MRI was repeated on 3/7/89 and showea a persistent large syrinx from CS to T6. The 'This paper was presented at the 39th Annual Con syrinx appeared to be the same size as was ference of the American Paraplegia Society, Las demonstrated in his Vegas, Nevada. September 7-9, 1993 preoperative ,MRI, imply t Correspondcnce: Department of Neurosurgery. Erie ing malfunction of the drainage tube. On 7/6/89 County Medical Center, 462 Grider Street, Buffalo, the TS-T6 laminectomy was reopened and the New York 14215, USA. syringo-subarachnoid shunt was revised. 424 Glasauer and Czyrny Paraplegia 32 (1994) 423-429

Figure 1 Case 1. MRI scan of (a) cervical and (b) upper thoracic spine 11/30/87 demonstrating an extensive syrinx cavity from C4 to T8.

Again, postoperatively there was significant dominal spasms as well as spasms of the lower improvement in his positional HH, although extremities which, however, were not interfer not complete resolution. A repeat MRI showed ing with her activities of daily living. significant decrease in the size of the syrinx and In September 1991, she developed HH com subsequent MRI studies have shown a stable ing on either immediately or within one-half syrinx size with minimal changes. (Fig 2) hour after being in the upright position. It would also happen when she was lying on hcr right side. The HH occurred in the area of her Case 2 normal sweating: namely her head and should A 27 year old female was involved in a motor ers and would be so profuse that her clothes vehicle accident on 6/26/78 resulting in a com and towels were soaked. Again, no other man pression fracture of the C6 vertebral body. On ifestations of autonomic dysreflexia were pre neurological examination she was a C6 com sent. Changing to the supine position or lying plete Frankel's A quadriplegic. She was treated down would instantly stop the HH. In addition, initially with skeletal traction for 6 weeks and she noted some tingling in both hands, more then had an external orthosis. In the course of marked in the fingertips and a vibratory sensi her intensive rehabilitation she developed tivity of her bones, especially around the waist. severe cystitis, and a sacral decubitus sore re There may have been a very mild increase in quiring a rotation flap operation. Spasticity was her spasticity. However, sensation and the initially treated with dantrium. strength in the upper extremities remained un Her normal sweat pattern involved both up changed. per extremities, face and the neck anteriorly An MRI in February of 1992 demonstrated a down to the clavicle and posteriorly to the post-traumatic syrinx extending from C2 to lower portion of the scapulae. She experienced about the midthoracic region T8. In the area of the typical sympathetic sweating (autonomic her vertebral injury the spinal canal was some dysreflexia) with the occurrence of bowel or what encroached upon by the deformed bone bladder problems. She also developed mild ab- (Fig 3). Paraplegia 32 (1994) 423�429 Post-traumatic syringomyelia and hyperhidrosis 425

Figure 2 Case 1. MRI of thoracic spine in June 1990, demonstrating considerable collapse of the syrinx after successful shunt revision.

A syringo-subarachnoid shunt at the upper Figure 3 Case 2. Preoperative MRI of cervical thoracic spinal level was carried out 6/29/92. and upper thoracic spine confirms extensive She had immediate short term relief of her HH syrinx from C2 to T8 and encroachment of for about 1 month, followed by recurrence of spinal canal by bony deformity at C6. her symptom. A repeat MRI in September of 1992 again subarachnoid space was found to be tethered showed the presence of the syrinx but the and enveloped by scar tissue. After this pro cavity was slightly narrower. cedure there was considerable improvement in In November 1992 she reported complete her symptoms. Presently there is mild sweating relief of her HH for about 3 months following a only after being upright for a couple of hours bout of bronchitis. This occurrence was at compared to the immediate onset prior to sur tributed to a clearing of a possible obstructed gery and no HH when she is in the right lateral shunt tube from debris by the increased intra position. spinal pressure during coughing. Subsequently, her HH returned and an MRI in January of 1993 showed the syrinx to be enlarged, extend Discussion ing from C2 to no. In March 1993 the previous syringo PTS occurs in a small percentage of spinal subarachnoid shunt was converted to a syringo cord injured patients. Herz et al2 reported peritoneal shunt. At the time of surgery the that among 850 individuals referred to a distal portion of the shunt catheter in the rehabilitation center, five patients devel- 426 Glasauer and Czyrny Paraplegia 32 (1994) 423-429

oped new symptoms due to PTS, an incid series of 30 patients with PT S included four ence of 0.6%. Oakley et ae in 1981 re patients with symptoms of increased sw eat viewed the literature and recovered 93 cases ing and only in one patient was HH the of PTS. It occurred in about 1.8% of initial symptom.5 Anderson et all in their paraplegics and less than 0.5% of quadriple report on HH emphasized that current HH gics. A similar incidence was quoted by problems were reported by 56 patients Barnett et al. 4 In Rossier et al 's5 series among 192 spinal cord injured patients. Six of 951 patients with spinal cord injury, 30 of the patients reported the onset of very (3.9%) developed cervical syringomyelia. annoying HH 2-8 years after their spinal This condition was found in 22 (4.5%) cord lesion. Barnett et al 4 observed seg post-traumatic quadriplegic and 8 (1.7%) mental HH in eight of their 17 patients with post-traumatic paraplegic patients. The in PTS. Sw eating was severe enough that cidence of cervical syringomyelia was about patients commented on it spontaneously 8% in patients with complete quadriplegia. and three patients required frequent daily However, greater aw areness of this entity changes of clothing. However, in only one and the increased use of MRI will result in patient was HH the initial symptom of PT S an earlier diagnosis and in diagnosing a whereas in the other patients increased greater number of patients. sw eating appeared to be a presentation of The interval between trauma and the autonomic dysreflexia. Only StanworthS re presentation of a syrinx varies widely, from ports on the significance of HH in eight several months to many years.2 ,6,7 In the patients with PT S. In tw o of his patients HH patients reported here the interval of spinal was the first presenting symptom. The injury to the onset of symptoms was 4 years excessive sw eating occurred below the le and 13 years respectively. The interval for sion in five and above the lesion in three appearance of the syrinx is somewhat patients. Unilateral sw eating occurring in shorter in those with complete compared to three was provoked by lying in the lateral those with partial spinal cord lesions. Most decubitus position. Other reports rarely of the responsible injuries for PT S occurred refer to HH as a presenting symptom. in the lower cervical or high thoracic area If HH is present it occurs in such excess but may also be associated with low thoracic ive quantities that, for example, pools of or even upper lumbar lesions. sw eat accumulate in the bed, or sw eat can The syrinx cavity can either ascend or be wrung out of clothes and towels. It may descend within the cord from the site of occur above or below the level of transec damage and not infrequently is found above tion, unilaterally, and may involve derma and below the spinal cord lesion.s However, tomes whose sensation is later altered by the in almost all cases the upper thoracic cord is syrinx.s In our tw o patients HH occurred involved. primarily above the lesion and bilaterally. Pain, various sensory changes, progress Sw eat glands derive their innervation ive motor weakness, and increase in spasti from the sympathetic nervous system. The city are the most frequent symptoms of hypothalamus and other suprasegmental PT S.4,7 Excessive sw eating or HH is not an structures contribute to the regulation of a early feature of PTS. Piatt7 does not men variety of visceral reflex activities through tion HH at all in his description of PT S. the descending fiber system. Within the Batzdorf6 reported on several patients spinal cord the supranuclear sudomotor whose symptoms of syringomyelia were fibers pass through the posterior aspect of triggered by recent trauma and mentions the anterolateral tract very close to the abnormalities of sw eating occuring in the lateral pyramidal tract and the border zone upper limbs and upper face or the occa of the intermediolateral horns where the sional lack of sw eating on one side or the segmental synapse with the ganglion cells of other in syringobulbia. If Horner's syn the gray matter takes place. The inter drome was present it occurred usually uni mediolateral nucleus consists of several ad laterally. He referred to one patient with jacent cell columns, the most apical cell persistent and distressing HH . Rossier's group constituting the lateral horn (Fig 4). It Paraplegia 32 (1994) 423-429 Post-traumatic syringomyelia and hyperhidrosis 427

Nuc. dorsalis lof Clarke) Those terminating in the paravertebral gan Nuc. proprius dorsalis glia either end there or terminate in ganglia Substantia gelatinosa above or below the level of their entrance. While the white rami communicantes are limited to the thoracic and upper lumbar nerves, each spinal nerve receives a gray ramus communicans from the sympathetic trunk. The gray ramus consists of unmye :ff>--'-'-"'+f-- Nuc. intermediolateralis linated, sympathetic postganglionic fibers which supply the dilator muscles of blood vessels, sweat glands, the hairs of the head Nuc. motorius medialis and face, and the lacrimal and salivary Figure 4 Cross-section of spinal cord at upper glands.9 thoracic (TS) level. The distribution of the postganglionic sympathetic fibers compares closely to that of the sensory dermatomes.1 3,14 However, since sympathetic innervation does not fol contains preganglionic sympathetic neurons low a clear segmental distribution, normal and is limited to thoracic and lumbar levels 10 sweating may be preserved at a higher or TI-L2.9, Laminae 7 and 8, which include 0 lower level than skin sensation.1 the dorsal nucleus of Clarke, extend Although the thoracolumbar outflow throughout the zona intermedia and ventral arises from a cell column in a restricted portions of the gray matter. The intermedial region of the spinal cord, it represents the cell column is associated with visceral motor total sympathetic output for the entire body. reflexes. 11,12 Lesions of the descending central tracts can The peripheral processes of these cells result in complete loss or altered control of give rise to preganglionic sympathetic fibers visceral activities at lower segmental levels. which emerge from the spinal cord via the Complete interruption of the sudomotor ventral roots (Fig 5). Peripherally these pathways abolishes the thermoregulatory fibers leave the mixed spinal nerve as the function of the sweat glands resulting in white rami communicantes, enter the anhidrosis.13 In the cervical cord there is a �ympathetic trunk and terminate upon cells somatotopic arrangement within the supra m the paravertebral or prevertebral ganglia. nuclear sudomotor tract which is similar to other tracts. Visceral afferent fi ber Most viscera receive a double autonomic / Arrector innervation with the notable exception of pili muscle " Hair the glands of the skin and peripheral blood Lateral Spinal �.." , horn ganglion �A vessels which receive only a sympathetic Peripheral , innervation. The sweat glands of the head ,1; <:::.' and neck are supplied by the intermedio �� _____ Sweat gland . 0 - anglionic 3 -_ .- � 1 -'::';' : ...... -:: �� lateral nucleus in cord segments Tl-T . 9

-White -;� The neurohumoral transmitter of all ,\, e;J Blood vessel amus preganglionic autonomic fibers, of all post Gray ramus ganglionic parasympathetic fibers, and of Vertebral ganglion postganglionic sympathetic nerve fibers in " Sympathetic trunk nervating sweat glands is acetylcholine. Overactivity of both the adrenergic and

Digestive tu be cholinergic fibers results in autonomic im balance. Figure 5 Diagram of sympathetic reflex arcs (reproduced with permission of the author from In patients with spinal cord injury the Carpenter MD & Sutin J (1983) Human afferent impulses from below the level of Neuroanatomy, figure 8-2, p. 212, Williams & the lesion enter the spinal cord through the Wilkins, Baltimore) . dorsal nerve root and ascend along the 428 Glasauer and Czyrny Paraplegia 32 (1994) 423-429 spinothalamic tracts or the dorsal columns. hyperactive after section of the corticospinal At segmental levels up to the level of the tracts.1 5 lesion the impulses may cause a spinal mass The HH occurred in our two patients reflex, referred to as autonomic hyper primarily when in an upright position and in reflexia or autonomic dysreflexia. Auto the second patient also when she was on her nomic hyperreflexia has been suggested to right side and was promptly abolished by be the result of sprouting of ascending fibers change or in the recumbent position. This forming abnormal synapses in the inter change of position is indicative of pressure mediolateral gray columns and resulting in changes within the syrinx especially in the mass discharge of sympathetic neurons from upright or other position when the hydro peripheral stimuli.I It is usually the result of static pressure in the 'syrinx tube' may result metabolic or mechanical irritation below the in irritation or pressure on the adjacent level of the lesion such as bladder or bowel intermediolateral tract or nucleus. Septation distention, pressure sores, or infection. This in the syrinx and adjacent scarring of the can result in hypertension, alterations in spinal cord can contribute to this pheno gastrointestinal motility, bradycardia, and menon rather than the postural influence on vasodilatation proximal to the level of the epidural veins. Percutaneous puncture lesion. Sweating is one of the manifestations of the syrinx has been carried out for - of autonomic hyperreflexia. For unknown the diagnosis and treatment. 16 18 Direct reasons there seems to be a head and neck measurements of the syrinx pressure have predominance of sweating during auto been found to be elevated especially in cases nomic discharge. The lowest level most of septation which may represent loculation often mentioned in which autonomic hyper or an evolving picture in the progression of reflexia has been seen is T6 but cases even at the syrinx and is frequently found to be levels down to Tll have been reported.uo communicating.17.l8 Increase in ventricular A change of posture especially in cervical pressure has been observed to increase the lesions may evoke reflex sweating. This may pressure within the syrinx or cyst. The occur when a patient is in the prone position transmission of the thoracic pressure to the or lying on the side. It is also observed when spinal subarachnoid space with compression a quadriplegic patient is raised from a of the syrinx is thought to be the principal horizontal to upright position, in particular force that enlarges the syrinx.19 when standing in the parallel bars. Different methods of treatment for HH Frequently, only part of the sympathetic have been used with variable results. ally innervated functions are activated. Among them is the use of acupuncture and a Thus, in the autonomic crisis, drenching short course of epidural morphine injection. sweat may occur without vasospasm. This Methantheline bromide diminished excess can be explained by the presence of distinct ive sweating but never abolished it.1.15 In fiber tracts subserving sudomotor and vaso one study topical scopolamine successfully 0 motor functions.1 controlled reflex sweating without anti 0 Anterior horn lesions are not infrequently cholinergic side effects.2 combined with vasomotor disturbances of . Direct drainage of the syrinx or various sweating. The classical representation of shunting procedures, preferably a syringo central damage or destruction of the spinal peritoneal shunt are the most successful cord is syringomyelia. surgical procedures in the treatment of PT S. HH develops above the level of complete The use of intraoperative ultrasound for the cord lesions. The exaggerated activity of exact placement of the shunt catheter has sweat glands is particularly conspicuous in been recommended by Wilberger et al.21 the segments nearest the lesion and reveals Drainage of the syrinx via a shunting pro itself by early onset of increased sweating in cedure either abolished or markedly im a bandlike form. The sympathetic mechan proved the HH in our two patients. Mal ism can be considered to be disinhibited function of the drainage system resulted in but locally intact comparable to the intact re-expansion of the syrinx and recurrent muscle stretch reflex mechanism which is HH . Subsequent improvement after Paraplegia 32 (1994) 423-429 Post-traumatic syringomyelia and hyperhidrosis 429

revision of the syrinx shunt supports the cur several years after the injury. The syrinx anatomical and pathophysiological events extends either above and/or below the level and explains the presenting symptom of of injury and almost always involves the HH . upper thoracic spinal cord. HH is not a HH can be an early and significant symp frequent symptom and is especially rare as tom in the development of PTS. Spinal cord a presenting symptom of PTS. If HH is injured patients frequently have a precari present it occurs in excessive quantities and ous independence and, therefore, are easily is affected by posture. The symptoms are threatened by additional loss of function. promptly relieved by surgical drainage of Early detection of the development of PTS the syrinx. is very important in the diagnosis and prompt surgical treatment. Acknowledgements

Summary We wish to thank Paul H Dressel from the Department of Neurosurgery for the prepara PTS usually occurs in those with a low tion of the illustrations for this article, and Judy cervical spinal cord injury especially with a Berst from the Department of Rehabilitation complete neurological lesion. Symptoms oc- Medicine for preparation of the manuscript.

References

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