Enric Portell

Incidence of skeletal fractures after traumatic spinal cord injury: a 10-year follow-up study:

Objective: To analyse the incidence and factors related to the development and clinical evolution of fractures in patients with traumatic spinal cord injury. Design: A retrospective 10-year follow-up study. Setting: Neurorehabilitation centre. Subjects: Sixty-three patients (50M/13F) with a mean age of 36 ± 20 years with recent traumatic spinal cord injury attended over a one-year period (January to December 2000). Main measures: Medical reports were reviewed, evaluating risk factors for osteoporosis, fracture incidence during the 10 years following spinal cord injury, severity (ASIA score) and level of spinal cord injury (paraplegia/tetraplegia), type of lesion (spastic/flaccid), weight-bearing standing activity, and the cause, location and evolution of the fracture. Results: Of the 129 patients attending during the study period, 75 had traumatic spinal cord injury (7 died and 5 had no follow-up). Finally, 63 patients were included. Fifty-four per cent had complete motor injury (ASIA A). Twenty-five per cent of these patients developed fractures, with 2.9 fractures per 100 patient-years. The femur was the most frequent location of the fractures. Fractures were observed 6.4 ± 2.4 years after spinal cord injury (range 2–10 years), all in males. Most fractures (70%) were related to low-impact injuries. Fifty per cent presented with associated clinical complications and only 20% of the patients had received anti-osteoporotic treatment. Spinal cord injury severity was the only risk factor for the development of fractures (complete spinal cord injury (ASIA A)) (RR 4.043; 95% confidence interval (CI) 1.081–23.846, P = 0.037). Conclusion: The incidence of fractures after spinal cord injury is high, with severity and time since spinal cord injury being the main determinants for their development. Fractures were frequently associated with clinical complications. However, the use of anti-osteoporotic treatment was uncommon.

Alterations in Excitatory and Inhibitory Brainstem Interneuronal Circuits after Severe Spinal Cord Injury

Reorganization of the central nervous system following spinal cord injury (SCI) involves changes not only at the cortical level, but also at the level of the brainstem. To further understand changes in excitatory and inhibitory brainstem interneuronal circuits following SCI, we studied recovery curves of the blink reflex (BR) to paired suprathreshold stimuli at various intervals (160, 300, 500, and 1000 msec), and prepulse inhibition of the BR following right index finger stimulation 100 msec prior to supraorbital nerve stimulation. Both parameters were studied in patients with SCI without baclofen treatment (n = 19), in patients with continuous intrathecal baclofen (CITB) (n = 9), and in healthy controls (n = 13). R2 recovery, expressed as the ratio of R2 area following the second stimulus divided by the R2 area following the first stimulus, was significantly greater in SCI patients without baclofen compared to controls and patients with CITB at all intervals, while there was no difference between patients with CITB and controls. Prepulse inhibition of R2 was significantly less in patients without baclofen compared to patients with CITB and healthy controls. Our findings indicate enhanced excitability and reduced inhibition of brainstem interneuronal circuits in patients with SCI, that are restored in the presence of baclofen to levels comparable to controls. In conclusion, SCI patients show more extended alterations in brainstem circuitry than previously thought. Decreased GABAergic mechanisms seem to be related to both excitatory and inhibitory brainstem circuit alterations. Baclofen appears to effectively restore this decreased GABAeregic activity.

Brainstem reflexes are enhanced following severe spinal cord injury and reduced by continuous intrathecal baclofen.

Objective. Plastic changes in the human central nervous system can occur at multiple levels, including circuits rostral to the lesion level in spinal cord injury (SCI). GABA is the most important inhibitory neurotransmitter in the brain. The authors hypothesized that one of the consequences of plasticity in SCI patients could be enhancement of brainstem reflexes, and they investigated the effect of continuous intrathecal baclofen (CITB) on such enhancement. Methods. The authors studied the early ipsilateral component R1 and the late component R2 of the blink reflex (BR), jaw jerk, masseter silent period (MSP), and auditory startle response (ASR) in 9 SCI patients without baclofen and in 8 with CITB. Nine healthy volunteers served as controls. Results. The amplitude of R1 of BR was significantly smaller in patients with CITB than in the other groups. The area of R2 of BR and of the ASR recorded in the orbicularis oculi, sternocleidomastoid, and biceps brachii muscles were significantly larger in SCI patients without baclofen than in controls, whereas there was no difference between patients with CITB and controls. The MSP magnitude was significantly larger in patients with CITB as compared with those without baclofen. Conclusion. The enhancement of brainstem reflexes in SCI patients may be due to plastic changes at the brainstem level after SCI. The significant reduction in response size in patients with CITB in comparison with patients without baclofen suggests that the enhancement of brainstem reflexes may be due to decreased GABAergic activity and that CITB is effective in reducing abnormal brainstem hyperexcitability.

Effect of Recent Spinal Cord Injury on Wnt Signaling Antagonists (Sclerostin and Dkk‐1) and Their Relationship With Bone Loss. A 12‐Month Prospective Study

Spinal cord injury (SCI) has been associated with a marked increase in bone loss and bone remodeling, especially short‐term after injury. The absence of mechanical load, mediated by osteocyte mechanosensory function, seems to be a causative factor related to bone loss in this condition. However, the pathogenesis and clinical management of this process remain unclear. Therefore, the aim of the study was to analyze the effect of recent SCI on the Wnt pathway antagonists, sclerostin and Dickkopf (Dkk‐1), and their relationship with bone turnover and bone mineral density (BMD) evolution. Forty‐two patients (aged 35 ± 14yrs) with a recent (<6months) complete SCI were prospectively included. Sclerostin and Dkk‐1, bone turnover markers (bone formation: PINP, bone ALP; resorption: sCTx) and BMD (lumbar spine, proximal femur, total body and lower extremities [DXA]) were assessed at baseline and at 6 and 12 months. The results were compared with a healthy control group. 22/42 patients completed the 12‐month follow‐up. At baseline, SCI patients showed a marked increase in bone markers (PINP and sCTx), remaining significantly increased at up to 6 months of follow‐up. Additionally, they presented significantly increased Dkk‐1 values throughout the study, whereas sclerostin values did not significantly change. BMD markedly decreased at the proximal femur (‐20.2 ± 5.4%, p < 0.01), total body (‐5.7 ± 2.2%, p = 0.02) and lower extremities (‐13.1 ± 4.5%, p = 0.01) at 12 months. Consequently, 59% of patients developed densitometric osteoporosis at 12 months. Patients with higher Dkk‐1 values (>58 pmol/L) at baseline showed higher sublesional BMD loss. In conclusion, this study shows that short‐term after SCI there is a marked increase in bone turnover and bone loss, the latter associated with an increase in Dkk‐1 serum levels. The persistence of increased levels of this Wnt antagonist throughout the study and their relationship with the magnitude of bone loss suggests a contributory role of this mediator in this process.

Restless legs syndrome in patients with sequelae of poliomyelitis

BACKGROUND No studies have examined the association between RLS and the sequelae of poliomyelitis (PM). We studied the frequency and severity of RLS in a group of consecutive patients with the sequelae of poliomyelitis (PM) and the effect of treatment with dopaminergic drugs. METHODS A diagnosis of RLS was made according to the criteria of the International RLS Study Group, and severity was assessed by the RLS rating scale. Information on sex, age, age at onset, site affected by PM, disease duration of PM, and history of post-polio syndrome (pPS) was obtained in a cohort of 52 PM patients. RESULT The mean age was 55.9 ± 6.5 years; 39 patients had post-polio syndrome (75%). RLS was diagnosed in 21 (40.4%) patients. Sixteen of the 21 patients (76.2%) with RLS had pPS, which was similar to the non-RLS group (74.2% patients with pPS). RLS symptoms were very severe in 5 patients, severe in 13, moderate in 2 and mild in 1. Nineteen of the 21 patients with RLS had symptoms predominantly in the more affected lower limb (90% of patients). Sixteen patients received dopaminergic agonist treatment with a significant reduction in their scores on the RLS severity scale from 28.3 ± 4.7 to 6.9 ± 7.3 (p < 0.001). DISCUSSION RLS occurs frequently in patients with PM, both in those with and without pPS, and responds well to treatment with dopaminergic drugs.

Alteraciones de la función intestinal en pacientes con lesión medular: relación con las características neurológicas de la lesión

Fundamento y objetivo Evaluar clinicamente la funcion intestinal en pacientes con lesion medular y relacionarla con sus caracteristicas neurologicas. Pacientes y metodo Se evaluo a 109 pacientes, de los que el 30% presentaba tetraplejia y el 70%, paraplejia. En cuanto a la gravedad de la lesion segun la American Spinal Cord Injury Association (ASIA) Impairment Scale, el 65% se clasifico como A (completa), el 12% como B (incompleta sensitiva), el 11% como C (incompleta motora con balance muscular Resultados El 77% de los pacientes necesitaba laxantes y el 68%, tacto rectal; un 10% evacuaba menos de 3 veces por semana y un 18% precisaba mas de 1 h para la defecacion; el 27% presentaba estrenimiento, el 31%, incontinencia, el 31%, enfermedad anorrectal y el 18%, disreflexia autonoma. Los clasificados como ASIA A, B y C con RES usaban mas supositorios, evacuaban con menos frecuencia y necesitaban mas tiempo para la evacuacion que los pacientes sin RES. Los pacientes con tetraplejia y gravedad ASIA A, B y C presentaban mas estrenimiento. Solo las localizaciones altas de la lesion, con gravedad ASIA A, B, C y RES presentaban disreflexia autonoma. Los pacientes con gravedad ASIA D tambien precisaban laxantes, tacto rectal y presentaban sintomas colorrectales. Conclusiones Los sintomas colorrectales son muy prevalentes y las caracteristicas del intestino neurogeno estan relacionadas con el tipo de lesion medular.

Restless leg syndrome in patients with spinal cord injury

BACKGROUND The presence of restless leg syndrome (RLS) in patients with spinal cord injury (SCI) is not well established. We studied the frequency and severity of RLS in a population of patients with SCI and the effect of treatment with dopaminergic drugs when clinically indicated. METHODS Consecutive patients with SCI admitted to an out-patient clinic of a neurorehabilitation hospital (n:195) were evaluated for the presence of RLS between February 2013 and May 2014. A diagnosis of RLS was made according to the criteria of the International RLS Study Group, and severity was assessed by the RLS rating scale. Information on gender, age, time since SCI, level and severity of SCI, was obtained. RESULT The mean age was 54.7 ± 15.6 years (range: 22-81 year); with time since SCI: 16.9 ± 11.4 years (range: 1-50 years). Thirty-five of 195 patients (17.9%) presented RLS. Twenty-two out of 154 (14.3%) patients with cervicothoracic SCI and 13 out of 41 (31.7%) patients with lumbosacral SCI presented RLS. Restless leg symptoms were mild in 2 patients, moderate in 10 patients, severe in 12 patients and very severe in 11. Ten patients received dopaminergic agonist treatment with a significant reduction in RLS severity scale from 29.1 ± 5.9 to 10.2 ± 7.9. DISCUSSION RLS occurs frequently in SCI patients and responds to dopaminergic treatment. Physicians have to be aware of this diagnosis to avoid unnecessary suffering in this patient population.

Mechanical and thermal hyperalgesia in patients with poliomyelitis

OBJECTIVE Paralytic poliomyelitis (pPM) is clinically suspected in individuals experiencing a non-progressive syndrome of flaccid paralysis and atrophy as a sequel of an acute infection. Despite normal sensory perception, patients with pPM complain of pain more than matched siblings. Here, we studied the characteristics of evoked pain in a cohort of pPM patients using contact heat evoked potentials and psychophysical tests. METHODS Fifteen patients with pPM and 15 controls were studied. Inclusion criteria were unilateral or asymmetric involvement of lower extremities. Mechanical, warm and heat pain perception thresholds and evoked pain were measured in both thighs. Contact heat evoked potentials were recorded from the vertex. RESULTS Mechanical and heat pain thresholds were significantly lower in the affected than in the less-affected leg or in the legs of controls. Evoked pain ratings were significantly higher in the affected leg than in either the less-affected leg or in controls. Evoked potentials were significantly higher in the affected than in the less-affected leg. CONCLUSION Patients with pPM have mechanical and thermal hyperalgesia, which suggests abnormalities in processing of somatosensory inputs in these patients. SIGNIFICANCE This phenomenon should be taken into account in the routine clinical evaluation and management of pPM patients.

Efecto de la lesión medular motora completa reciente en el recambio óseo y en la evolución de la masa ósea: resultados preliminares

Summary Background and aim: Spinal cord injury (SCI) has been associated with a marked increase in bone loss and a higher incidence of skeletal fractures, however the pathogenesis and clinical management of this condition remains unclear. The aim of this study was to analyze the bone mineral density (BMD) evolution in patients with complete SCI and its relationship with parameters of bone metabolism and bone turnover markers. Methods: Patients with a recent complete motor SCI (ASIA A)(<6 months) were prospectively included. Bone metabolism parameters (calcium, phosphate, PTH and 25-OHD), bone turnover markers (bone formation: procollagen type 1 aminoterminal propeptide -P1NP-, bone alkaline phosphatase -bone AP-, osteocalcin -OC-; bone resorption: C-telopeptides of type I collagen -CTx-) and BMD were assessed in all patients at baseline and at 6 months. The results were compared with a control group. Results: 23 men with complete SCI (ASIA A) and a mean age of 38±15 years were included at 102±33 days of SCI onset. 52% had paraplegia. 12 patients were assessed at 6 months of follow-up. Patients with SCI showed a significant increase in bone turnover markers, especially P1NP and CTx, compared to controls (P1NP: 191±90 vs 51±19 ng/ml, p<0.001; CTx: 1.37±0.49 vs 0.51±0.23 ng/ml, p<0.001). At 6 months, bone turnover markers decreased (P1NP: -34%, p=0.005 and CTx: -26%, p=0.002) and BMD had a mean decrease of 12% at total femur (p=0.002) compared to baseline, with osteoporosis development in 50% of patients. Bone markers (bone AP, P1NP and OC) were negatively correlated with total femur BMD values. Conclusions: Patients with complete SCI show a marked increase in bone turnover and bone loss, especially at the proximal femur, with the development of osteoporosis being observed in 50% of these patients at 6 months of follow-up. These findings indicate the need to implement preventive measures within the therapeutic approach in these patients.

S16.2 Mechanical and thermal hyperalgesia in the affected leg in patients with poliomyelitis

Introduction: POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, M protein, skin changes) syndrome is a rare multisystemic disorder associated with plasma cell dyscrasia, whose main neurological feature is a demyelinating polyneuropathy. Objective: The aim of our study was to assess the pattern of ultrasound (US) nerve alterations in patients with POEMS syndrome. Methods: Seven patients (4 men and 3 women, mean onset age 53.1±14 yrs, range 32 67) with POEMS syndrome, all presenting with demyelinating polyneuropathy with secondary axonal degeneration, underwent neurological examination with quantification of muscular strength and sensory evaluation, and US examination of median, ulnar, peroneal, tibial and sural nerves. Nerve and single fascicle cross sectional area (CSA) alterations, structure and echogenicity were analyzed. Results: US alterations were mostly circumscribed to entrapment sites. Five of 7 patients had increased median nerve CSA at wrist, 3 of whom bilaterally, 2 of 7 patients presented ulnar nerve hypertrophy at elbow. Three patients had mild peroneal nerve hypertrophy at fibular head. Enlargements outside the entrapment sites were uncommon. No correlation was found between muscular strength deficit and US focal findings. Conclusions: No homogeneous pattern of US alterations along the nerve course and among different nerves was found. The lack of correlation between US and clinical findings may be secondary to the chronic nerve damage that is common in patients with POEMS syndrome, where diagnosis is often delayed, and that would be consistent with the absence of US signs of acute damage such as nerve swelling.