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Transverse myelitis is an autoimmune neurological disorder of the spinal cord that affects individuals of all age groups, including infants.
To report priapism as a unique clinical manifestation of infantile transverse myelitis.
Transverse Myelitis Center at Johns Hopkins, Johns Hopkins Hospital, Baltimore, Maryland.
Three infants younger than 12 months.
Intravenous corticosteroids, intravenous immunoglobulin, physical therapy, and rehabilitation.
Main Outcome Measure
Clinical outcomes in infants with priapism and transverse myelitis.
All 3 infants demonstrated rapidly progressive quadriplegia that resulted in substantial disability and proved fatal for 1 infant.
Priapism in infants with transverse myelitis may be an indication of severe inflammation and neural injury that necessitates immediate and aggressive treatment.
Priapism is a painful sustained erection that is not associated with sexual stimulation and is not relieved by ejaculation. Although rare in children, priapism occurs in all age groups of life.1 It has varying causes and can be classified as low-flow ischemic priapism2 or high-flow nonischemic priapism.3 Sickle cell disease and other hemoglobinopathies, hematologic dyscrasias, vasoactive drugs, traumatic injury, and some neurologic conditions4 are known causes of priapism. Priapism in the context of neurologic diseases has been attributed to disturbance in neurotransmissions that mediate erection5 or abnormal activation of reflexes involved in erection after genital stimulation.6 Intracorporeal blood gas analysis or color Doppler ultrasonographic examination of the penis can enable differentiation between low- and high-flow priapism. Prompt intervention in low-flow priapism is necessary to prevent long-term damage to erectile tissue. Aspiration at the base of the corpora cavernosa with a nonheparinized syringe is the first-line treatment in low-flow priapism, with a success rate of approximately 30%.7 Any identified underlying cause of priapism must also be treated. In high-flow priapism, lack of evidence of substantial pathologic damage and maintenance of erectile function make observation and noninterventional management a good treatment option.8 In both low- and high-flow priapism, surgical treatment options can be used if medical management fails.
Transverse myelitis (TM) is a rare autoimmune neurologic disorder of the spinal cord9 with 20% of cases occurring in children younger than 18 years.10 Transverse myelitis in children seems to have a bimodal distribution with 2 peaks of incidence: (1) a narrow range involving children younger than 3 years and accounting for about 38% of cases and (2) a broader range in children aged 5 to 17 years.11 Pediatric TM occurs equally among both sexes. Early-onset TM, that is, before age 3 years, is associated with more severe long-term disability than that occurring between ages 5 and 17 years.11 Acute clinical symptoms of pediatric TM include pain, weakness, sensory loss, and urinary dysfunction. To our knowledge, no cases of TM with priapism have been reported as the initial finding. We report 3 cases of priapism in infantile TM at the Transverse Myelitis Center at Johns Hopkins.
A 7-month-old boy was healthy until he developed fever and lethargy with rapid progression to quadriplegia. These signs and symptoms were preceded 4 weeks earlier by low-grade fever, cough, runny nose, restlessness, and impaired sleep. A diagnosis of upper respiratory tract infection was made, and antibiotic therapy was initiated. However, the symptoms did not resolve, and otitis media developed that persisted for 2 weeks. When seen, the patient had fever (temperature, 100°C), and he appeared weak and made few movements. He was seen in the emergency department, at which time he had decreased respiration, was lethargic, and had encephalopathy. His condition progressed rapidly to complete quadriplegia with no sensation or strength below the neck, and tracheotomy and mechanical ventilatory assistance were necessary. He had a sustained penile erection at the time of evaluation. All serologic and infectious samples when cultured yielded no organisms. Magnetic resonance (MR) imaging with gadolinium enhancement revealed increased T2-weighted signal intensity and substantial expansion of the cervical spinal cord from C3 to T1. A diagnosis of idiopathic TM was made, and treatment was instituted with intravenous corticosteroids and plasma exchange. The penile erection resolved by the third day of treatment with intravenous corticosteroids. The patient required a G-tube for feeding. Magnetic resonance imaging repeated after 6 months showed marked atrophy of the spinal cord from C4 to T4. The patient underwent a series of rehabilitation and inpatient hospital stays over the next 12 months. Thirteen months after initial onset, the tracheotomy and G-tube were still in place. Mechanical ventilation was turned off for 6 hours per day, with suctioning every half hour to 3 hours. The patient exhibited some shoulder shrugging; however, no active range of motion was observed in the upper limbs. There was volitional hip flexion on the right side and to a lesser degree on the left side. Deep-tendon reflexes were moderate (3+) at the biceps and knees with sustained clonus at both ankles. We recommended treatment with intravenous immunoglobulin, 2 g/kg, and a repeated dose after 3 months. At 5 years, the patient's condition continues to improve. He has improvement in head and truncal control but cannot bear weight or ambulate. Urinary function has returned to normal. There is no evidence to suggest permanent damage to erectile function. Bowel function is normal with a regimen of daily mild laxatives.
A 5-month old boy who was previously well developed a runny nose with clear exudate on the day before presentation and then became restless. Six hours later, he was found to have a penile erection. He was unable to urinate or pass stool. He was evaluated in the emergency department and determined to be diffusely hypotonic and quadriplegic. The penile erection persisted. Magnetic resonance imaging of the spinal cord with diffuse heterogeneous gadolinium enhancement showed a minimally expanded spinal cord with increased T2-weighted signal intensity from the cervicomedullary junction to T7. Examination of the cerebrospinal fluid (CSF) indicated no inflammation. A diagnosis of idiopathic TM was made, and treatment was initiated with intravenous corticosteroids. By day 3 of treatment, the priapism resolved, and bladder function returned and bowel function improved. Magnetic resonance imaging repeated 2 months later showed improvement over the previous MR image at the time of onset but still indicated subtle enhancement with increased T2-weighted signal intensity within the spinal cord.
Over the next 3 to 6 months, the patient's condition continued to improve with some strength returning to his arms. Fourteen months after onset, he was still undergoing water therapy and physical and occupational therapy with transcutaneous electrical nerve stimulation. Physical examination revealed truncal weakness, and the patient needed support of at least 1 arm to prop himself up. Both hands required splinting. He had good finger flexion in the left hand but absent flexion in the right hand. Muscle tone in the lower limbs was mildly increased, and the patient was unable to walk without bilateral assistance.
A 12-month-old male twin with normal growth and development developed sudden-onset quadriplegia. Neurologic examination showed complete loss of sensory and motor function caudal to C5 and a sustained penile erection. Examination of the CSF revealed 4 white blood cells and 7.8 g/dL of protein (to convert protein to grams per liter, multiply by 10.0). Polymerase chain reaction of the CSF and cultures yielded normal findings, and the CSF was positive for protein 14-3-3, a marker of neuronal injury in TM.10 Evoked potentials showed no conduction rostral to C5. Magnetic resonance imaging revealed a swollen cervical and upper thoracic cord with fusiform T2-weighted hyperintensity that showed enhancement from C3 to C7 after contrast administration (Figure 1A). A diagnosis of TM was made, and treatment with intravenous corticosteroids was initiated. The priapism resolved after 2 days of treatment. No neurologic function returned caudal to C5. Magnetic resonance imaging repeated after 1 month revealed severe myelomalacia of the spinal cord from C3 to T1 with focal loss of 78% of spinal cord cross-sectional area (Figure 1B). The patient died 6 weeks later of respiratory failure with no neurologic return of function caudal to C5. Postmortem examination revealed mononuclear infiltrates in the meninges (Figure 2A) and severe necrosis of the spinal cord evidenced by mononuclear cells and macrophages (Figure 2B) and tissue vacuolation or necrosis (Figure 2C). These findings are evidence of the fulminant aggressive inflammation in the spinal cord noted in TM.
Magnetic resonance images show progressive myelomalacia in transverse myelitis. Patient 3, sagittal (top) and axial magnetic resonance images were obtained at symptom onset (A; axial C5) and after 4 weeks (B; axial C5). At 4 weeks after injury, 78% of the cross-sectional area of the spinal cord is eliminated.
Spinal cord necrosis after transverse myelitis. Patient 3, autopsy findings and histochemistry revealed meningeal infiltrate (A), and the spinal cord showed mononuclear cells (B) and tissue cavitation (C) evidence of the fulminant and aggressive inflammation, which are sequelae of transverse myelitis (all parts: hematoxylin-eosin–Luxol fast blue, original magnification ×20).
We report 3 cases of rapidly progressing acute TM with priapism and quadriplegia in infants who had poor outcomes. Priapism, to our knowledge, has not been previously reported in patients with nontraumatic spinal cord disease of any kind. Priapism, sometimes referred to as a “death erection” or “terminal erection,” is typically observed in men who have been executed by hanging and results from complete functional spinal cord severing or traumatic injury to the spinal cord. The cervical dislocation after hanging is thought to result in removal of the central inhibition of erection or in sudden influx of autonomic outflow that results in an erection.
The presence of priapism in our patients suggests intense and fulminant immune-mediated injury of the spinal cord resulting in the nearly complete and rapid functional severing of the spinal cord by inflammation. All 3 of our patients had severe neural injury and were substantially disabled or died as a result of TM. Longitudinal MR imaging in patient 3 revealed a 78% loss of spinal cord cross-sectional area 4 weeks after symptom onset. Pathologic examination 6 weeks after the onset of TM with priapism revealed complete necrosis of the cervical spinal cord (Figure 2), evidence of the fulminant aggressive inflammation in this patient. We checked for CSF protein 14-3-3 only in patient 3 because we saw him in the acute phase of the illness. The presence of protein 14-3-3 in the CSF indicates neuronal injury in the spinal cord and could be helpful in prognosticating following TM.12
Thus, priapism may be a unique sign of TM in infants and may suggest severe inflammation and neural injury. To our knowledge, no cases of priapism in adults with TM have been reported. Priapism as an initial symptom in infants may reflect either (1) more severe injury in these patients because infantile TM often leads to higher residual disability than TM in older populations or (2) the immaturity of the developing nervous system. Priapism in infantile TM may be related to cervical spine pathologic disorders, as observed in all 3 of our patients. In a center-based analysis of 38 pediatric TM cases at our center, 19 (50%) revealed T2-weighted signal intensity abnormalities in the cervical spinal cord.11 We report priapism in 3 of the 19 patients (16%) with cervical cord involvement. There is a need to better understand the factors that precipitate priapism in infants with severe TM affecting the cervical spinal cord. Research into clinical outcomes in these patients is also needed.
Early recognition of priapism as an initial sign of TM may help clinicians avert unnecessary studies performed to disclose other disorders and may suggest a need for consideration of aggressive therapies such as plasma exchange or intravenous immunoglobulin to treat TM. We, however, have no data to suggest that aggressive treatment with plasma exchange or intravenous immunoglobulin in this patient population will change clinical outcomes because none of our patients received either treatment in the acute phase. Our patient in case 1 received 2 doses of intravenous immunoglobulin 13 months after onset because we observed an early plateau in his recovery. In our experience, we have observed delayed recovery after intravenous immunoglobulin therapy, which could facilitate myelin and inhibitory factor removal, thereby enhancing regeneration. Neither of our patients in cases 1 and 2 have had a recurrence of TM or priapism.
Correspondence: Douglas A. Kerr, MD, PhD, Department of Neurology, The Johns Hopkins University School of Medicine, 600 N Wolfe St, Baltimore, MD 21287-5371 (email@example.com).
Accepted for Publication: October 8, 2008.
Author Contributions: Dr Kerr had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: Hammond and Kerr. Acquisition of data: Hammond and Kerr. Analysis and interpretation of data: Hammond and Kerr. Drafting of the manuscript: Hammond and Kerr. Critical revision of the manuscript for important intellectual content: Hammond and Kerr. Administrative, technical, and material support: Hammond and Kerr. Study supervision: Kerr.
Financial Disclosure: None reported.
Hammond ER, Kerr DA. Priapism in Infantile Transverse Myelitis. Arch Neurol. 2009;66(7):894-897. doi:10.1001/archneurol.2009.66