Aggravation of an injured dentato-rubro-thalamic tract in a patient with mild traumatic brain injury: A case report.

RATIONALE: We report on a patient with mild traumatic brain injury (TBI) by follow-up diffusion tensor tractography (DTT), and observed for approximately nine monthsby serial diffusion tensor tractography (DTT). PATIENT CONCERNS: A 66-year-old male patient was injured in a car crash. Approximately four weeks after the crash, he developed a tremor in the right hand and leg. His symptoms worsened over time. DIAGNOSES: Approximately six months after the crash, he developed a mild tremor in the left hand. Nine months after the crash, he manifested severe tremor in his right hand, mild resting and intentional tremor in his left hand and both legs, and mild trunkal ataxia. INTERVENTIONS: N/A. OUTCOMES: On 3-week DTT, well reconstructed DRTTs were observed in both hemispheres, except for the thinned lower portion of the right DRTT. On 9-month DTT, the right lower DRTT had thinned compared with the 3-week DTT and showed a disruption at the upper portion. The left DRTT showed thinning in the lower portion and tearing in the upper portion compared with 3-week DTT. LESSONS: Aggravation of an injured DRTT was demonstrated in a patient with mild TBI, using serial DTT examination.

Rat motor neurons caudal to a rubrospinal tract (RST) transection remain viable.

In the rat, the rubrospinal tract (RST) is a descending motor pathway involved in the production of skilled reaching movement. The RST originates in the red nucleus in the midbrain and runs down the spinal cord in the lateral most aspect of the dorsolateral funiculus (DLF). The RST makes monosynaptic contact with interneurons within the intermediate laminae of the cord, however a contingent of RST axons constitutes direct supraspinal input for spinal cord motor neurons. The current study investigated the effects of unilateral RST transection at cervical levels C3-4 on the population of motor neurons in both spinal segments C5-6 and L2-3. The total number of large, medium and small motor neurons in these segments was estimated with stereological techniques in both ventral horns at 1, 3, 7 and 14days post-injury. In both spinal cord segments under investigation, no change was detected in mean number of motor neurons over time, in either ventral horn. That the loss of direct supraspinal input resulting from the RST transection does not affect the viability of motor neurons caudal to the injury indicates that these neurons have the potential to be re-innervated, should the RST injury be repaired.

Reorganization of Intact Descending Motor Circuits to Replace Lost Connections After Injury.

Neurons have a limited capacity to regenerate in the adult central nervous system (CNS). The inability of damaged axons to re-establish original circuits results in permanent functional impairment after spinal cord injury (SCI). Despite abortive regeneration of axotomized CNS neurons, limited spontaneous recovery of motor function emerges after partial SCI in humans and experimental rodent models of SCI. It is hypothesized that this spontaneous functional recovery is the result of the reorganization of descending motor pathways spared by the injury, suggesting that plasticity of intact circuits is a potent alternative conduit to enhance functional recovery after SCI. In support of this hypothesis, several studies have shown that after unilateral corticospinal tract (CST) lesion (unilateral pyramidotomy), the intact CST functionally sprouts into the denervated side of the spinal cord. Furthermore, pharmacologic and genetic methods that enhance the intrinsic growth capacity of adult neurons or block extracellular growth inhibitors are effective at significantly enhancing intact CST reorganization and recovery of motor function. Owing to its importance in controlling fine motor behavior in primates, the CST is the most widely studied descending motor pathway; however, additional studies in rodents have shown that plasticity within other spared descending motor pathways, including the rubrospinal tract, raphespinal tract, and reticulospinal tract, can also result in restoration of function after incomplete SCI. Identifying the molecular mechanisms that drive plasticity within intact circuits is crucial in developing novel, potent, and specific therapeutics to restore function after SCI. In this review we discuss the evidence supporting a focus on exploring the capacity of intact motor circuits to functionally repair the damaged CNS after SCI.

Diffusion tensor imaging-based assessment of white matter tracts and visual-motor outcomes in very preterm neonates.

INTRODUCTION: The purpose of this study was to assess the impact of brain injury on white matter development and long-term outcomes in very preterm (VPT) neonates. METHODS: Eighty-five VPT neonates (born <32/40 weeks gestational age (GA)) scanned within 2 weeks of birth were divided into three groups based on the presence of perinatal cerebral injury: (i) no injury, (ii) mild/moderate injury and (iii) severe injury. Diffusion tensor imaging (DTI) was acquired for each neonate and fractional anisotropy (FA), and diffusivity measures were calculated in the posterior limb of the internal capsule (PLIC) and optic radiation (OR). At 2 and 4 years of age, 41 and 44 children were assessed for motor and visual-motor abilities. Analyses determined the relation between GA and DTI measures, injury groups and DTI measures as well as developmental assessments. RESULTS: GA was related to all DTI measures within the PLIC bilaterally, FA in the OR bilaterally and AD in the left OR. The severely injured group had significantly different DTI measures in the left PLIC compared to the other two groups, independent of lateralization of lesions. Group differences in the left OR were also found, due to higher incidence of the white matter injury in the left hemisphere. No differences were found between groups and outcome measures at 2 and 4 years, with the exception of destructive periventricular venous haemorrhagic infarction (PVHI). CONCLUSIONS: DTI measures of the PLIC and OR were affected by injury in VPT neonates. These findings seen shortly after birth did not always translate into long-term motor and visual-motor impairments suggesting compensatory mechanisms.

Detection of hand and leg motor tract injury using novel diffusion tensor MRI tractography in children with central motor dysfunction.

PURPOSE: To examine whether an objective segmenation of corticospinal tract (CST) associated with hand and leg movements can be used to detect central motor weakness in the corresponding extremities in a pediatric population. MATERIAL AND METHODS: This retrospective study included diffusion tensor imaging (DTI) of 25 children with central paresis affecting at least one limb (age: 9.0±4.2years, 15 boys, 5/13/7 children with left/right/both hemispheric lesions including ischemia, cyst, and gliosis), as well as 42 pediatric control subjects with no motor dysfunction (age: 9.0±5.5years, 21 boys, 31 healthy/11 non-lesional epilepsy children). Leg- and hand-related CST pathways were segmented using DTI-maximum a posteriori (DTI-MAP) classification. The resulting CST volumes were then divided by total supratentorial white matter volume, resulting in a marker called "normalized streamline volume ratio (NSVR)" to quantify the degree of axonal loss in separate CST pathways associated with leg and hand motor functions. A receiver operating characteristic curve was applied to measure the accuracy of this marker to identify extremities with motor weakness. RESULTS: NSVR values of hand/leg CST selectively achieved the following values of accuracy/sensitivity/specificity: 0.84/0.84/0.57, 0.82/0.81/0.55, 0.78/0.75/0.55, 0.79/0.81/0.54 at a cut-off of 0.03/0.03/0.03/0.02 for right hand CST, left hand CST, right leg CST, and left leg CST, respectively. Motor weakness of hand and leg was most likely present at the cut-off values of hand and leg NSVR (i.e., 0.029/0.028/0.025/0.020 for left-hand/right-hand/left-leg/right-leg). The control group showed a moderate age-related increase in absolute CST volumes and a biphasic age-related variation of the normalized CST volumes, which were lacking in the paretic children. CONCLUSIONS: This study demonstrates that DTI-MAP classification may provide a new imaging tool to quantify axonal loss in children with central motor dysfunction. Using this technique, we found that early-life brain lesions affect the maturational trajectory of the primary motor pathway which may be used as an effective marker to facilitate evidence-based treatment of paretic children.

Accurate segmental motor innervation of human lower-extremity skeletal muscles.

BACKGROUND: There is limited knowledge about accurate segmental motor innervation of the human lower extremity skeletal muscles. The aim of the present study was to explore the truth of segmental motor innervation of the lower extremity. METHODS: Included in this study were 20 patients with unilateral fracture of the sacrum and sacral nerve injury, who underwent internal fixation and decompression of the sacral nerve in our hospital between June 2009 and January 2014. L4-S4 nerve roots of the uninjured side were stimulated during operation. Motor innervation was determined by stimulating the spinal nerves with supramaximal intensity. RESULTS: We found the gluteus medius and the gluteus maximus were both mainly innervated by L5 and S1. In addition, the nerve fibres that innervated the extensor digitorum brevis, the abductor hallucis and the flexor digitorum brevis were mainly from S2 to S3. CONCLUSIONS: Our study provides the electrophysiological mapping of the segmental anatomy of the human lower extremity muscles, which should be clinically useful in helping the diagnosis and treatment of nerve injury and neuropathies.

Permanent anosmia and ageusia after resection of a left temporoinsular low-grade glioma: anatomofunctional considerations.

Five percent of the general population has olfactory or gustatory disorders, although most do not complain about it. However, in some cases, these symptoms can be disabling and may affect quality of life. Anosmia was reported as a possible complication following head injury and neurosurgical procedures, particularly after the resection of tumors located in the anterior fossa and the treatment of aneurysms in the anterior circulation. Nonetheless, in all of these situations, olfactory dysfunction could be explained by damage to the peripheral olfactory system. Here, the authors report a case of complete anosmia associated with ageusia following awake resection of a low-grade glioma involving the left temporoinsular region, with no recovery during a follow-up of 3 years. The frontal lobe was not retracted, and the olfactory tract was not visualized during surgery; therefore, postoperative anosmia and ageusia are likely explained by damage to the cortex and central pathways responsible for these senses. The authors suggest that the patient might have had a subclinical right hemianosmia before surgery, which is a common condition. After resection of the central structures critical for smell and taste processing in the left hemisphere, the patient could have finally had bilateral and complete olfactory and gustatory loss. This is the first known report of permanent anosmia and ageusia following glioma surgery. Because these symptoms might have been underestimated, more attention should be devoted to olfaction and taste, especially with regard to possible subclinical preoperative deficit.

Return of spinal reflex after spinal cord surgery for brachial plexus avulsion injury.

Motor but not sensory function has been described after spinal cord surgery in patients with brachial plexus avulsion injury. In the featured case, motor-related nerve roots as well as sensory spinal nerves distal to the dorsal root ganglion were reconnected to neurons in the ventral and dorsal horns of the spinal cord by implanting nerve grafts. Peripheral and sensory functions were assessed 10 years after an accident and subsequent spinal cord surgery. The biceps stretch reflex could be elicited, and electrophysiological testing demonstrated a Hoffman reflex, or Hreflex, in the biceps muscle when the musculocutaneous nerve was stimulated. Functional MR imaging demonstrated sensory motor cortex activities on active as well as passive elbow flexion. Quantitative sensory testing and contact heat evoked potential stimulation did not detect any cutaneous sensory function, however. To the best of the authors' knowledge, this case represents the first time that spinal cord surgery could restore not only motor function but also proprioception completing a spinal reflex arch.

The time course of serotonin 2A receptor expression after spinal transection of rats: an immunohistochemical study.

Hyperexcitability of motoneurons is one of the key mechanism that has been demonstrated to underlie the pathogenesis of spasticity after spinal injury. Serotonin (5-HT) denervation supersensitivity is one of the mechanisms underlying this increased motoneuron excitability. In this study, to examine whether the supersensitivity is caused by 5-HT receptor upregulation we investigated changes in levels of 5-HT2A receptor immunoreactivity (5-HT2AR-IR) following a spinal transection in the sacral spinal cord of rats at seven different time points post injury: 2, 8, 16 h, and 1, 2, 7 and 28 days, respectively. 5-HT2AR-IR density was analyzed in motoneurons (regions containing their somata and dendrites) in the spinal segments below the lesion. The results showed no significant changes in 5-HT2AR-IR in the motoneurons up to 16 h following the transection. After 1-day, however the levels of 5-HT2AR-IR increased in the motoneurons and their dendrites, with the density level being 3.4-fold higher in spinalized rats than in sham-operated rats. The upregulation increased progressively until a maximal level was reached at 28 days post-injury. We also investigated 5-HT and 5-HT transporter expressions at five different post injury time points: 1, 2, 7, 21 and 60 days and they showed concurrent down-regulation changes after 2 days. These results suggest that the upregulation of 5-HT2ARs may at least partly underlie the development of 5-HT denervation supersensitivity in spinal motoneurons following spinal injury and thereby implicates their involvement in the pathogenesis of the subsequent development of spasticity.

Secondary damage in the spinal cord after motor cortex injury in rats.

When neurons within the motor cortex are fatally injured, their axons, many of which project into the spinal cord, undergo wallerian degeneration. Pathological processes occurring downstream of the cortical damage have not been extensively studied. We created a focal forelimb motor cortex injury in rats and found that axons from cell bodies located in the hindlimb motor cortex (spared by the cortical injury) become secondarily damaged in the spinal cord. To assess axonal degeneration in the spinal cord, we quantified silver staining in the corticospinal tract (CST) at 1 week and 4 weeks after the injury. We found a significant increase in silver deposition at the thoracic spinal cord level at 4 weeks compared to 1 week post-injury. At both time points, no degenerating neurons could be found in the hindlimb motor cortex. In a separate experiment, we showed that direct injury of neurons within the hindlimb motor cortex caused marked silver deposition in the thoracic CST at 1 week post-injury, and declined thereafter. Therefore, delayed axonal degeneration in the thoracic spinal cord after a focal forelimb motor cortex injury is indicative of secondary damage at the spinal cord level. Furthermore, immunolabeling of spinal cord sections showed that a local inflammatory response dominated by partially activated Iba-1-positive microglia is mounted in the CST, a viable mechanism to cause the observed secondary degeneration of fibers. In conclusion, we demonstrate that following motor cortex injury, wallerian degeneration of axons in the spinal cord leads to secondary damage, which is likely mediated by inflammatory processes.

Differential plasticity of the GABAergic and glycinergic synaptic transmission to rat lumbar motoneurons after spinal cord injury.

Maturation of inhibitory postsynaptic transmission onto motoneurons in the rat occurs during the perinatal period, a time window during which pathways arising from the brainstem reach the lumbar enlargement of the spinal cord. There is a developmental switch in miniature IPSCs (mIPSCs) from predominantly long-duration GABAergic to short-duration glycinergic events. We investigated the effects of a complete neonatal [postnatal day 0 (P0)] spinal cord transection (SCT) on the expression of Glycine and GABA(A) receptor subunits (GlyR and GABA(A)R subunits) in lumbar motoneurons. In control rats, the density of GlyR increased from P1 to P7 to reach a plateau, whereas that of GABA(A)R subunits dropped during the same period. In P7 animals with neonatal SCT (SCT-P7), the GlyR densities were unchanged compared with controls of the same age, while the developmental downregulation of GABA(A)R was prevented. Whole-cell patch-clamp recordings of mIPSCs performed in lumbar motoneurons at P7 revealed that the decay time constant of miniature IPSCs and the proportion of GABAergic events significantly increased after SCT. After daily injections of the 5-HT(2)R agonist DOI, GABA(A)R immunolabeling on SCT-P7 motoneurons dropped down to values reported in control-P7, while GlyR labeling remained stable. A SCT made at P5 significantly upregulated the expression of GABA(A)R 1 week later with little, if any, influence on GlyR. We conclude that the plasticity of GlyR is independent of supraspinal influences whereas that of GABA(A)R is markedly influenced by descending pathways, in particular serotoninergic projections.

Phrenic nerve stimulation: the Australian experience.

Phrenic nerve stimulation is a technique whereby a nerve stimulator provides electrical stimulation of the phrenic nerve to cause diaphragmatic contraction. The most common indications for this procedure are central alveolar hypoventilation and high quadriplegia. This paper reviews the available data on the 19 patients treated with phrenic nerve stimulation in Australia to date. Of the 19 patients, 14 required pacing due to quadriplegia, one had congenital central hypoventilation syndrome and one had brainstem encephalitis. Information was unavailable for the remaining three patients. Currently, 11 of the pacers are known to be actively implanted, with the total pacing duration ranging from 1 to 21 years (mean 13 years). Eight of the 19 patients had revision surgeries. Four of these were to replace the original I-107 system (which had a 3-5-year life expectancy) with the current I-110 system, which is expected to perform electrically for the patient's lifetime. Three patients had revisions due to mechanical failure. The remaining patients' notes were incomplete. These data suggest that phrenic nerve stimulation can be used instead of mechanical ventilators for long-term ongoing respiratory support.

Functional recovery of stepping in rats after a complete neonatal spinal cord transection is not due to regrowth across the lesion site.

Rats receiving a complete spinal cord transection (ST) at a neonatal stage spontaneously can recover significant stepping ability, whereas minimal recovery is attained in rats transected as adults. In addition, neonatally spinal cord transected rats trained to step more readily improve their locomotor ability. We hypothesized that recovery of stepping in rats receiving a complete spinal cord transection at postnatal day 5 (P5) is attributable to changes in the lumbosacral neural circuitry and not to regeneration of axons across the lesion. As expected, stepping performance measured by several kinematics parameters was significantly better in ST (at P5) trained (treadmill stepping for 8 weeks) than age-matched non-trained spinal rats. Anterograde tracing with biotinylated dextran amine showed an absence of labeling of corticospinal or rubrospinal tract axons below the transection. Retrograde tracing with Fast Blue from the spinal cord below the transection showed no labeled neurons in the somatosensory motor cortex of the hindlimb area, red nucleus, spinal vestibular nucleus, and medullary reticular nucleus. Retrograde labeling transsynaptically via injection of pseudorabies virus (Bartha) into the soleus and tibialis anterior muscles showed no labeling in the same brain nuclei. Furthermore, re-transection of the spinal cord at or rostral to the original transection did not affect stepping ability. Combined, these results clearly indicate that there was no regeneration across the lesion after a complete spinal cord transection in neonatal rats and suggest that this is an important model to understand the higher level of locomotor recovery in rats attributable to lumbosacral mechanisms after receiving a complete ST at a neonatal compared to an adult stage.

Postnatal conversion of cross phrenic activity from an active to latent state.

Spinal cord hemisection rostral to the phrenic nucleus leads to paralysis of the ipsilateral hemidiaphragm and respiratory insufficiency. Recovery of the paralyzed hemidiaphragm may be induced by activating a latent respiratory motor pathway in adult rats. Although the pathway is latent in adults, it may not be latent in neonatal rats as shown by the spontaneous expression of activity over this pathway in an earlier in vitro study. Activity mediated over the latent pathway is known as "crossed phrenic activity". Whether crossed phrenic activity following C2 spinal cord hemisection occurs spontaneously in the neonatal rat in vivo is still unknown. We hypothesized that crossed phrenic activity may be spontaneously expressed in neonates in vivo and may be converted from a spontaneously active state to a latent and nonfunctional state during postnatal development. Thus, a time course study was designed to analyze this activity in rat pups at different ages. The functional status of the ipsilateral and contralateral hemidiaphragms was tested by EMG analysis following hemisection. Crossed phrenic activity was expressed in ventral, lateral, and dorsal parts of the ipsilateral hemidiaphragm in P2 and some P3 and P4 neonatal rats. During postnatal development, the activity was observed only in the ventral area of the ipsilateral hemidiaphragm in P7, P14, P21 and P28 animals. Significant decreases in the extent of ventral crossed phrenic activity were observed from P2 to P28. The pathway generating this activity becomes latent by postnatal day 35. The present results suggest that spontaneous crossed phrenic activity occurs in vivo following C2 hemisection and the activity gradually decreases during the first four postnatal weeks.

Sonographic measurement of diaphragm movement in patients with tetraplegia.

STUDY DESIGN: This was an observational, descriptive case-series study. The magnitude and direction of diaphragm movement during tidal and maximal inspiratory breaths in tetraplegic subjects were measured using B-mode sonography on a single occasion. Data were tabulated for descriptive analysis. OBJECTIVE: There is a paucity of literature reporting dynamic movements of the paralyzed diaphragm. The aim of this pilot study was to investigate and record diaphragm movement in subjects with a cervical spinal cord injury (C1-C5), which had resulted in tetraplegia with partial or complete diaphragm paralysis. Subjects were patients of the Royal Adelaide Hospital in South Australia. RESULTS: Three subjects participated in the study. The magnitude of diaphragm movement was small in two subjects and approached normal in one subject. During tidal inspiratory and maximal inspiratory breaths, the diaphragm moved in a caudal direction in two subjects. In the other subject, the diaphragm moved in a cephalad direction during a maximal inspiratory breath. CONCLUSION: Imaging of diaphragm movement was well tolerated by three subjects with cervical spinal cord injury. The difference in magnitude of diaphragm movement was not fully explained by the level of injury and the American Spinal Injury Association classification.

Anatomical and functional outcomes following a precise, graded, dorsal laceration spinal cord injury in C57BL/6 mice.

To study the pathophysiology of spinal cord injury (SCI), we used the LISA-Vibraknife to generate a precise and reproducible dorsal laceration SCI in the mouse. The surgical procedure involved a T9 laminectomy, dural resection, and a spinal cord laceration to a precisely controlled depth. Four dorsal hemisection injuries with lesion depths of 0.5, 0.8, 1.1, and 1.4 mm, as well as normal, sham (laminectomy and dural removal only), and transection controls were examined. Assessments including the Basso Mouse Scale (BMS), footprint analysis, beam walk, toe spread reflex, Hargreaves' test, and transcranial magnetic motor-evoked potential (tcMMEP) analysis were performed to assess motor, sensorimotor, and sensory function. These outcome measures demonstrated significant increases in functional deficits as the depth of the lesion increased, and significant behavioral recovery was observed in the groups over time. Quantitative histological examination showed significant differences between the injury groups and insignificant lesion depth variance within each of the groups. Statistically significant differences were additionally found in the amount of ventral spared tissue at the lesion site between the injury groups. This novel, graded, reproducible laceration SCI model can be used in future studies to look more closely at underlying mechanisms that lead to functional deficits following SCI, as well as to determine the efficacy of therapeutic intervention strategies in the injury and recovery processes following SCI.

Ibuprofen enhances recovery from spinal cord injury by limiting tissue loss and stimulating axonal growth.

The GTP-binding protein RhoA regulates microfilament dynamics in many cell types and mediates the inhibition of axonal regeneration by myelin and chondroitin sulfate proteoglycans. Unlike most other nonsteroidal anti-inflammatory drugs, ibuprofen suppresses basal RhoA activity (Zhou et al., 2003). A recent report suggested that ibuprofen promotes corticospinal axon regeneration after spinal cord injury (Fu et al., 2007). Here, we confirm that ibuprofen reduces ligand-induced Rho signaling and myelin-induced inhibition of neurite outgrowth in vitro. Following 4 weeks of subcutaneous administration of ibuprofen, beginning 3 days after spinal cord contusion, animals recovered walking function to a greater degree, with twice as many rats achieving a hind limb weight-bearing status. We examined the relative role of tissue sparing, axonal sprouting, and axonal regeneration in the action of ibuprofen. Histologically, ibuprofen-treated animals display an increase in spared tissue without an alteration in astrocytic or microglial reaction. Ibuprofen increases axonal sprouting from serotonergic raphespinal axons, and from rostral corticospinal fibers in the injured spinal cord, but does not permit caudal corticospinal regeneration after spinal contusion. Treatment of mice with complete spinal cord transection demonstrates long-distance raphespinal axon regeneration in the presence of ibuprofen. Thus, administration of ibuprofen improves the recovery of rats from a clinically relevant spinal cord trauma by protecting tissue, stimulating axonal sprouting, and allowing a minor degree of raphespinal regeneration.

Task-dependent compensation after pyramidal tract and dorsolateral spinal lesions in rats.

The purpose of this research was to investigate whether pathways in the dorsal part of the lateral spinal funiculus (DLF) can compensate for loss of corticospinal input (CST) to the spinal cord. The CST is known to control skilled limb movements in rats. The DLF contains several different pathways, including the rubrospinal tract (RST) which is also thought to influence limb movements. After lesions of either the corticospinal or the rubrospinal system, it is unclear how much of the remaining forelimb function is due to the presence of the alternate pathway. To begin to address this issue, the present study investigates the compensatory role of pathways in the DLF, including the rubrospinal tract, after bilateral lesions of the pyramidal tract (PT). We initially performed bilateral PT lesions in rats, which effectively removed the CST input to the spinal cord. We tested these rats during overground locomotion, skilled locomotion and skilled forelimb usage. After a 6 week recovery period, we then performed bilateral DLF lesions and compared the behavioural abilities of these rats to those of animals which underwent simultaneous PT and DLF lesions. If DLF pathways do compensate for PT lesions, then animals with PT lesions would rely more on DLF pathways than animals without PT lesions. Thus we hypothesized that animals with DLF lesions which were performed 6 weeks after PT lesions would exhibit more deficits on several behavioural tasks compared to animals which received PT and DLF lesions simultaneously. Our hypothesis was supported only for skilled pellet retrieval. Hence some DLF pathways, including the RST, were able to compensate for loss of CST input during skilled reaching but not during overground or skilled locomotion in PT-lesioned rats. These differential responses suggest that behavioural tasks vary in their reliance on specific pathways after injury, and, furthermore, that compensation for loss of specific connections can arise from numerous sources.

Favorable response to intrathecal, but not oral, baclofen of priapism in a patient with spinal cord injury.

STUDY DESIGN: Case report. OBJECTIVE: To retrieve data about the utility of intrathecal baclofen for the treatment of otherwise refractory priapism that may occur in patients with spinal spasticity. SUMMARY OF BACKGROUND: Baclofen exerts well-known inhibitory effects on sexual function both in animals and humans. This observation led to the introduction of oral baclofen in the treatment of refractory and recurrent idiopathic priapism. METHODS: We report a 41-year-old male patient who sustained a severe traumatic spinal cord injury in a motor vehicle accident. On clinical examination he presented with tetraplegia (motor level C3). Initial cervical magnetic resonance imaging showed a C3 to C4 lesion with a herniated disc and compression of the dural sac. One month later the patient developed priapism episodes. RESULTS: He received oral baclofen with only minimal beneficial effect on priapism. Eight months postinjury the patient underwent a test-trial with intrathecal baclofen bolus, which caused absence of priapism for 10 hours. One month later, an intrathecal pump system was implanted, leading to absence of priapism episodes. CONCLUSION: In the present case report, reduction of supraspinal control on the spinal cord may have induced an up-regulation of GABAB receptors, which are involved in penile tumescence. The trauma induced also liberation of penile reflexes with episodes of priapism. Normal full blood count and color duplex ultrasonography of the penis excluded a vascular genesis of priapism. This is the first report about the utility of intrathecal baclofen for the successful control of otherwise untreatable priapism in a patient with severe spinal spasticity. Hence, evaluation of intrathecal baclofen should be considered in patients suffering from severe and/or frequent priapism when oral baclofen and/or hormonal therapy are ineffective. The beneficial effect of intrathecal, but not oral baclofen, in our patient suggests a dose-dependent effect.