Autonomic Dysreflexia Associated with Transient Aphasia

Home , Autonomic dysreflexia, Intracerebral hemorrhage, Spinal shock

Case Report

Autonomic dysre¯exia associated with transient aphasia

SC Colachis*,1 and LP Fugate1 1The Department of Physical Medicine and Rehabilitation, The Ohio State University, College of Medicine, Columbus, Ohio, USA

Study design: Case report of autonomic dysre¯exia presenting with transient aphasia in a subject with C4 tetraplegia. Objectives: To report a rare case of autonomic dysre¯exia. Setting: Rehabilitation Service, The Ohio State University, USA. Case report: A 21-year-old man with a C4 spinal cord injury (ASIA B) developed aphasia associated with autonomic dysre¯exia. He was treated with an adrenergic blocking agent. Conclusion: Autonomic dysre¯exia manifested by a transient aphasia and seizures is uncommon. Spinal Cord (2002) 40, 142 ± 144. DOI: 10.1038/sj/sc/3101251

Keywords: spinal cord injury; tetraplegia, autonomic dysre¯exia; cerebral vascular insuciency

Introduction Autonomic dysre¯exia is a condition of massive on patients at risk for this re¯ex phenomenon. We are paroxysmal re¯ex sympathetic response to noxious unaware of a similar presentation in the literature. stimuli occurring in persons with tetraplegia and paraplegia with spinal cord injuries (SCI) above the Case report major splanchnic sympathetic out¯ow.1±3 Noxious stimuli with root entry zones more distal to the site A 21-year-old white male with no signi®cant past of neurologic injury (eg S2-S4) illicit the most profound medical history sustained an acute SCI with immediate sympathetic adrenergic and cholinergic responses4 C4 motor complete, sensory incomplete tetraplegia accounting for why the majority of episodes are (ASIA B) after `belly ¯opping' into a baby pool on 31 attributed to stimulation from the urinary and July 1999. Evaluation at that time revealed C5 and C6 alimentary tracts.3,5 Clinical features are varied, but vertebral body fractures, and C5 laminar fractures with depend in large part upon the resultant re¯ex canal compromise. There was no evidence of a sympathetic adrenergic and cholinergic activity. These traumatic brain injury. He underwent anterior decom- include cardiac chronotropic and inotropic eects, pression, fusion, and ®xation on 3 August 1999. His sudomotor and pilomotor responses, cephalgia, chest acute hospital course was complicated by recurrent pain, visual disturbances, and paroxysmal hyperten- mucous plugging requiring repeated bronchoscopy, sion.2±11 The resulting hypertension can be severe with aspiration pneumonia, and sepsis. He subsequently systolic and diastolic recordings two to three times underwent placement of a tracheotomy tube and a greater than the patient's baseline values.1,3,7,9 A feeding percutaneous endoscopic gastrostomy tube. hypertensive crisis, if untreated, can result in sei- He was admitted to our SCI rehabilitation service zures,5,6,8 ± 12 hemorrhages (cerebral, retinal, subarach- on 20 September 1999 for continued inpatient noid),6,12,13 stroke,12,14 coma,5,12 and death.5,12,13 rehabilitation. Over the next several days his medical Removal of the noxious stimuli results in abrupt condition improved considerably. His upper limb resolution of the autonomic response. motor function began to return and his vital capacity We present an unusual case of autonomic dysre- improved to over 2200 ml allowing the tracheotomy ¯exia presenting with transient aphasia in an indivi- tube to be downsized in anticipation of decannulation. dual with C4 tetraplegia which illustrates the profound He was out of the spinal shock phase of his injury and paroxysmal eects of the sympathetic nervous system was developing increased spasticity. During this time period, he developed severe central pain involving his lower limbs which was treated successfully with *Correspondence: SC Colachis, The Department of Physical Medicine and Rehabilitation, Dodd Hall, 480 West 9th Avenue, gabapentin, amitriptyline, and mild narcotic analge- Columbus, OH 43210 1245, USA sics. He was also noted to have a mildly elevated Autonomic dysreflexia and aphasia SC Colachis and LP Fugate 143 blood pressure for brief periods associated with Thompson and Withan10 induced, with bladder ¯ushing. His neuropathic bladder dysfunction was distention in a patient with spinal cord injury managed with a program of re¯ex voiding and symptoms of autonomic dysre¯exia followed by a condom catheter drainage at his request. seizure which resolved with bladder emptying. Seven days after admission, while receiving a range In the present case the seizure onset was near of motion exercises in bed, he exclaimed that he didn't immediate raising the possibility of another serious feel well, and abruptly became unresponsive with etiology such as intracranial hemorrhage or severe seizure-like activity and spontaneous raising of his hypoxia. No intracranial abnormalities were identi®ed upper limbs and drooling for approximately 1 min. and oxygen saturation was normal. The transient When he awoke he appeared to be in a post-ictal state aphasia observed later was associated with an elevated with confusion and emotional liability which shortly blood pressure 50% above his baseline but not with thereafter resolved. During the event his blood alterations in his level of consciousness or clinical pressure was 180/110 mmHg with a pulse of 86 beats seizure activity. This was initially felt to represent per min and respirations of 20 per min. Pulse cerebral vascular insuciency and a stroke in evolu- oxymetry was 98% saturation. His heart and lung tion but resolved shortly thereafter. We currently examinations were unremarkable, profuse ¯ushing of believe that the seizure was a result of heightened his face was noted. sympathetic activity with hypertension, and the He was transferred urgently to the acute care aphasia a possible manifestation of cerebral vascular hospital for further evaluation and management. insuciency. During that initial evaluation it was noted that he The present case illustrates several features of this was developing word-®nding diculties and aphasia in condition. Our patient sustained a SCI with C4 the absence of clinical seizure activity and the incomplete tetraplegia, above the level of the major possibility of a stroke in evolution was entertained. splanchnic sympathetic out¯ow (ie T4-T6), placing The aphasia resolved subsequently. Extensive evalua- him at risk for autonomic dysre¯exia.1±3 It is only tion over the next 48 h including CT and MRI scans rarely that reports have appeared in the literature of his head, an electroencephalogram, electrocardio- describing this phenomenon in patients with spinal gram, carotid dopplers, cardiac echo, laboratory cord injuries below T4-T6. and in our experience, studies, and repeat lower limb duplex ultrasonography those patients with lower injury levels experiencing failed to identify an etiology for the episodes. These these episodes give a history of neurologic improve- episodes were associated with elevated blood pressure ment from an original injury level above T4-T6. and ultimately believed to be of autonomic origin. Autonomic dysre¯exia occurs in both complete and Upon readmission to our facility 2 days later, he was incomplete injuries after resolution of the spinal shock placed initially on clonidine, which was later changed phase of their injury1 as in our patient. to doxazosin with improved bladder emptying, and Although the onset of this unusual presentation of resolution of hypertension associated with autonomic autonomic dysre¯exia was associated with a range of dysre¯exia. He has had no recurrence of mental status motion exercises, he experienced more episodes with changes, aphasia, or seizures since. re¯ex voiding and condom catheter drainage. The range of motion exercises have been associated with 15 Discussion autonomic dysre¯exia, but stimulation from the urinary tract is much more common.3,5 The range of The incidence of seizures associated with autonomic motion exercises in this instance have resulted in dysre¯exia appear to be uncommon, and transient increased intravesicular bladder pressure providing the aphasia in the absence of stroke or hemorrhage is rare. needed noxious stimulation. Resolution of the episodes The clinical features of autonomic dysre¯exia in our with the use of an adrenergic blocking agent (eg patient were quite unusual and we are unaware of a doxazosin) with improved bladder drainage and similar presentation. Prior authors have reported blockage of re¯ex sympathetic activity peripherally aphasia in the presence of hemorrhage,12 in one other points to this etiology for this phenomenon. His description, clari®cation was not given.5 We have neuropathic bladder dysfunction is currently managed treated three other patients who experienced seizures with a program of clean intermittent catheterization. associated with autonomic dysre¯exia over the past The medical literature is limited in regard to long- several years. None were associated with aphasia. In all term outcomes in patients experiencing seizures cases, there was a period of time ranging from a few attributed to autonomic dysre¯exia. One of three minutes to over an hour where classic symptoms (eg patients reported by Kursh et al12 died shortly after ¯ushing, sweating, cephalgia, etc.) were observed a seizure associated with intracerebral hemorrhage. before the onset of seizures, unlike this patient's Other reports reviewed do not detail long term presentation. Although prior observations indicate the outcomes.5,6,8 ± 12 We believe that patients who have clinical symptoms appear to precede seizures, descrip- experienced a seizure associated with autonomic tions of duration of those symptoms are not well dysre¯exia are at increased risk for other serious characterized.10 ± 12 In one of the earliest descriptions of sequelae and close monitoring and treatment of hypertension associated with autonomic dysre¯exia, autonomic dysre¯exia is very important. The sympa-

Spinal Cord Autonomic dysreflexia and aphasia SC Colachis and LP Fugate 144

thetic stimulation sucient to produce such a response 2 Guttman L, Whitteridge D. Eects of bladder distension in these individuals is profound and may well place on autonomic mechanisms after spinal cord injuries. them at increased risk for subsequent serious compli- Brain 1947; 70: 361 ± 404. cations. 3 Kewalramani LS. Autonomic dysre¯exia in traumatic Two of our four patients who have experienced myelopathy. Am J Phys Med 1980; 59: 1 ± 21. 4 Bors E, French JD. Management of paroxysmal seizures have since died. The ®rst patient sustained a hypertension following injuries to cervical and upper massive intracerebral hemorrhage and was reported thoracic segments of the spinal cord. Arch Surg 1952; 64: 1 previously. The second patient was 17 at the time of 803 ± 812. his SCI and after discharge from rehabilitation had a 5 Lindan R, Joiner B, Freehafer AA, Hazel C. Incidence seizure during an autonomic episode with hyperten- and clinical features of autonomic dysre¯exia in patients sion. During his subsequent care, he was noted to have with spinal cord injury. Paraplegia 1980; 18: 285 ± 292. a small cerebral infarct without clinical symptoms or 6 Brown BT, Carrion HM, Politano VA. Guanethidine signs. His death occurred approximately 16 months sulfate in the prevention of autonomic hyperre¯exia. J later and was not believed to be related to autonomic Urol 1979; 122: 55 ± 57. dysre¯exia. Our patient in the present case is doing 7 Frankel HL, Mathias CJ. Severe hypertension in patients with high spinal cord lesions undergoing electroejacula- well without signi®cant recurrences. The fourth patient tion ± management with prostaglandin E2. Paraplegia hashadnofurtherseizuresbutcontinuestohave 1980; 18: 293 ± 299. signi®cant diculty with vasomotor instability with 8McGuireEJ,WagnerFM,WeissRM.Treatmentof hypotension and intermittent autonomic dysre¯exia Autonomic dysre¯exia with phenoxybenzamine. JUrol for the past several years. 1976; 115: 53 ± 55. 9 Scott MB, Morrow JW. Phenoxybenzamine in neuro- genic bladder dysfunction after spinal cord injury. II. Conclusion Autonomic dysre¯exia. JUrol1978; 119: 483 ± 484. Autonomic dysre¯exia manifested by transient aphasia 10 Thompson CE, Witham AC. Paroxysmal hypertension in and seizures is uncommon. This small population of spinal cord injuries. NEJM 1948; 239: 291 ± 294. 11 Yarkony GM, Katz RT, Wu YC. Seizures secondary to spinal injured patients may be at increased risk of autonomic dysre¯exia. Arch Phys Med Rehabil 1986; 67: serious sequelae and should be monitored closely. We 834 ± 835. believe that long-term follow-up studies are clearly 12 Kursh ED, Freehafer A, Persky L. Complications of warranted to further assess the risk. The current autonomic dysre¯exia. JUrol1977; 118: 70 ± 72. availability of shared data bases (eg Model Systems) 13 Eltorai I et al. Fatal cerebral hemorrhage due to make such evaluation possible. autonomic dysre¯exia in a tetraplegic patient: case report and review. Paraplegia 1992; 30: 355 ± 360. 14 Guttman L, Frankel HL, Paeslack V. Cardiac irregula- rities during labour in paraplegic women. Paraplegia References 1965; 3: 144 ± 151. 15 McGarry J, Woolsey RM, Thompson CW. Autonomic 1 Colachis SC. Autonomic hyperre¯exia with spinal cord hyperre¯exia following passive stretching to the hip joint. injury. Top Spinal Cord Inj Rehabil 1997; 3: 71 ± 81. Phys Ther 1982; 62: 30 ± 31.

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