Spastic paraparesis associated with advanced liver cirrhosis: a condition obscure in terms of treatment and prognosis.

Hepatic myelopathy or spastic paraparesis of liver disease is an insidious onset condition with pure motor spastic paraparesis without sensory, bladder or bowel involvement in patients with chronic liver disease, in which the neurological dysfunction cannot be explained by other causes. It is a rare, relentlessly progressive and mostly irreversible neurological complication resulting from portosystemic shunts occurring spontaneously, created surgically or due to 'functional shunting'. In some cases, no evidence of shunting is elicitable due to difficulty in locating the hidden collaterals. We report this rare case of a 33-year-old man with chronic liver disease presenting with spastic paraparesis after 11 months of resolution of an episode of hepatic encephalopathy.

Caso Clínico: Paraparesia Espástica Tropical Asociada a Infección por HTLV-1 / Case Report: Spastic Tropical Paraparesis Associated with HTLV-1

INTRODUCCIÓN: La mielopatía asociada al virus linfotrópico de células T humano tipo 1 o llamada comúnmente paraparesia espástica tropical, constituye un síndrome espástico caracterizado por una evolución insidiosa, crónica, lentamente progresiva y muy variable; es considerada por algunos autores como una patología desmielinizante que afecta la médula espinal. CASO CLÍNICO: Paciente de sexo femenino de 53 años de edad, procedente de Manabí y residente en Santo Domingo de los Tsáchilas, que acudió con un cuadro de 10 meses de evolución caracterizado por parestesias en miembro inferior derecho que progresó a temblores, parestesias, hiperalgesia y pérdida de fuerza muscular de las cuatro extremidades más afectación esfinteriana. Se realizaron los estudios necesarios para estudiar las causas más comunes de paraparesia; finalmente, se detectó infección por virus linfotrópico de células T humano tipo I. EVOLUCIÓN: ELa paciente permaneció hospitalizada durante 28 días y fue dada de alta en condiciones estables con los déficits neurológicos previamente establecidos. Se mantuvo en seguimiento durante 4 años posterior al diagnóstico con deterioro progresivo de la fuerza muscular en sus cuatro extremidades hasta la monoplejía con espasticidad de miembro inferior derecho. Se han reportado episodios de disartria y disnea que han remitido espontáneamente. CONCLUSIÓN: La mielopatía asociada a infección por HTLV-1 o paraparesia espástica tropical es una enfermedad de baja prevalencia, debe ser sospechada y estudiada en pacientes con un cuadro crónico de paraparesia, alteraciones sensitivas y disfunción vesical.(au)

BACKGROUND: The human T lymphotropic virus type-I associated myelopathy or also called spastic tropical paraparesis, is a spastic syndrome characterized by a slow progressive, insidious and chronic evolution. It is considered by some authors as a demyelinating spinal cord disease. CASE REPORT: 53-year-old female patient, born in Manabi and residing in Santo Domingo de los Tsáchilas. With 10 months history of right lower limb paresthesias that progressed to tremor, paresthesias, hyperalgesia and four limb muscle weakness plus sphincter disorders. An appropriate workup were performed in order to study the most common causes of paraparesis, infection with HTLV-1 was detected finally. EVOLUTION: The patient remained hospitalized for 28 days; she was discharged in stable conditions with all the prior established neurological deficits. A 4-year follow up was registered after diagnosis; muscle weakness progressed in four limbs causing right lower limb monoplegia. Dysarthria and dyspnea have been also reported, with spontaneous recovery. CONCLUSION: Human T lymphotropic virus type-I associated myelopathy or spastic tropical paraparesis is a low frequency disease, must be suspected and studied in patients with chronic paraparesis with sensory loss and sphincter disorders.(au)

Effects of a 6-month self-rehabilitation programme in addition to botulinum toxin injections and conventional physiotherapy on limitations of patients with spastic hemiparesis following stroke (ADJU-TOX): protocol study for a randomised controlled, investigator blinded study.

INTRODUCTION: Home-based self-rehabilitation programmes combined with botulinum toxin injections (BTIs) appear to be a relevant approach to increase the recommended intensive rehabilitation of patients with spasticity following a stroke. The literature highlights a lack of evidence of beneficial effects of this adjuvant therapy to reduce limitations of patients with stroke. The aim of this study is to assess the effects of a 6-month self-rehabilitation programme in adjunction to BTI, in comparison with BTI alone, to reduce limitations of patients with spasticity following a stroke. METHODS AND ANALYSIS: 220 chronic patients will participate to this multicentre, prospective, randomised, controlled, assessor blinded study. All patients will benefit from two successive BTI (3 months apart), and patients randomised in the self-rehabilitation group will perform in adjunction 6 months of self-rehabilitation at home. All patients continue their conventional physiotherapy. The main outcome is the primary treatment goal (PTG), which will be determined jointly by the patient and the medical doctor using Goal Attainment Scaling. Impairments and functions, quality of life, mood and fatigue will be assessed. Botulinum toxin will be injected into the relevant muscles according to the PTG. Patients in the self-rehab group will be taught the self-rehabilitation programme involving respectively 10 min of stretching, 10 min of strengthening and 10 min of task-oriented exercises, corresponding to their PTG. Compliance to the self-rehabilitation programme will be monitored. ETHICS AND DISSEMINATION: Patients will sign written informed consent. Ethical approval was obtained from ethics committee. The results will be disseminated in a peer-reviewed journal and presented at international congresses. The results will also be disseminated to patients. TRIAL REGISTRATION NUMBER: NCT02944929.

Partial splenic artery embolization for severe hepatic myelopathy in cirrhosis.

Hepatic myelopathy (HM) is a devastating but rare complication of cirrhosis and portal hypertension that profoundly affects quality of life and improves only with liver transplantation. We present a case where progressive severe spastic paraparesis due to HM was substantially reversed with partial splenic artery emobilization (PSAE). (Hepatology 2018;67:1169-1171).

Serial Casting as an Adjunct to Botulinum Toxin Type A Treatment in Children With Cerebral Palsy and Spastic Paraparesis With Scissoring of the Lower Extremities.

The purpose of this study was to examine whether combination therapy of serial casting and botulinum toxin type A injection can further enhance the effects of botulinum toxin type A in children with cerebral palsy with scissoring of both legs. This study was a prospective and randomized trial. The children were divided into 2 groups, one of which received serial casting after botulinum toxin type A (n = 40), and the other which only received botulinum toxin type A (n = 40). Serial casting started 3 weeks after the botulinum toxin type A. Both groups received physiotherapy. Groups were assessed at baseline then compared at 6 and 12 weeks following the intervention. Significant improvements in Gross Motor Function Measure-66 and Caregiver Health Questionnaire were recorded in both groups ( P < .001). The modified Ashworth scale improved significantly following botulinum toxin type A in the serial casting group ( P < .05), but not in botulinum toxin type A only group. These results suggest that serial casting after botulinum toxin type A can enhance the benefits of botulinum toxin type A in children with cerebral palsy.

Effect of Dual Therapy with Botulinum Toxin A Injection and Electromyography-controlled Functional Electrical Stimulation on Active Function in the Spastic Paretic Hand.

BACKGROUND: Many previous studies have demonstrated that botulinum toxin A (BTX-A) injections satisfactorily reduce spasticity. Nevertheless, BTX-A, with or without an adjuvant therapy, effectively improves the direct functional movement in few patients with spastic upper extremity paralysis. Therefore the present study aimed to determine the effectiveness of task-orientated therapy on spasticity and functional movement by using electromyography-triggered functional electrical stimulation (EMG-FES) after BTX-A injections. DESIGN: Open-label, prospective clinical trial Method: The subjects were 15 patients with spastic paresis (12 male, 3 female; age range, 17-74 years; 14 due to stroke, 1 due to spinal cord injury) who received BTX-A injections. Before the study was started, all subjects had undergone task-orientated therapy sessions with EMG-FES for 4 months. Despite all patients showing a various extent of improved upper extremity function, upper extremity function reached a plateau because of upper extremity spasticity. After BTX-A injection, all patients underwent task-orientated therapy sessions with EMG-FES for 4 months. The outcomes were assessed with the modified Ashworth scale, the simple test for evaluating hand function, box and block test, grip and release test, finger individual movement test, and grip strength. Assessments were performed at baseline and 10 days and 4 months after BTX-A injection. RESULTS: The median modified Ashworth scale score decreased from 2 at baseline to 1 at 10 days and 4 months after BTX-A injection. The finger individual movement test score increased slightly at 10 days (p=0.29) and further increased at 4 months (p<0.05). The simple test for evaluating hand function, grip and release test, box and block test, and grip strength decreased after 10 days (p<0.05, p=0.26, p<0.01, and p<0.01, respectively) but increased after 4 months (p<0.01, p<0.05, p<0.01, and p=0.18, respectively). CONCLUSION: Task-orientated therapy with EMG-FES after BTX-A injection effectively reduced spasticity and improved upper limb motor function. Our results also suggest that spasticity occurs as a compensation for the force of the affected muscles and leads to misuse movements and ostensible dexterity in many patients. In addition, we hypothesize that BTX-A injection initializes the abnormal adapted movement pattern and that more active hand movements with facilitation of the paretic muscles when using EMG-FES induce an efficient muscle reeducation of the inherent physiological movement pattern that ultimately could prove useful in the activities of daily living.

Episodic spastic paraparesis successfully treated with unaided blood transfusions: a case report.

BACKGROUND: Extramedullary haemopoiesis is a common compensatory phenomenon in most haemolytic anaemias. However, spinal cord compression due to extramedullary spinal epidural haemopoiesis is an extremely rare complication of thalassemia. In such situation patients present with paraplegia with a sensory level. Usual treatment options are surgery and/or radiotherapy. CASE PRESENTATION: Here we report a 27 year old Sri Lankan Muslim male with haemoglobin E-Beta thalassaemia presented with episodic spastic paraparesis when he was anaemic which was dramatically responded to blood transfusion therapy. CONCLUSION: Most of the reported cases with paraplegia have been treated with surgery with or without radiation therapy or radiation therapy alone. Our patient makes dramatic recovery after blood transfusion in each presentation.

Long Term Outcome of Conservative Management or Surgical Treatment of Bovine Spastic Paresis: 79 Cases.

OBJECTIVE: To report the long term clinical outcome of calves treated surgically or managed conservatively for bovine spastic paresis of the gastrocnemius (BSP-G), quadriceps femoris muscle (BSP-Q), or mixed muscle involvement (BSP-M). STUDY DESIGN: Retrospective case study. ANIMALS: Calves (n = 79) with bovine spastic paresis. METHODS: Medical records of calves treated by partial tibial neurectomy or managed conservatively for bovine spastic paresis were analyzed for sex, breed, lineage history, and the onset, duration, and severity of clinical signs. Cases were classified as unilateral or bilateral BSP-G, BSP-Q, or BSP-M. Long term follow-up information was obtained by telephone questionnaire. RESULTS: The study group included 26 BSP-G (33%), 16 BSP-Q (20%), and 37 BSP-M (47%) calves. BSP-M and BSP-Q calves were significantly more bilaterally affected compared to BSP-G calves. Twenty-five of 26 BSP-G calves were treated surgically; 86% had complete resolution of clinical signs. Twenty-nine of 37 BSP-M calves were treated surgically; 81.5% improved, but none completely recovered. In all of the conservatively managed BSP-M calves, clinical signs gradually worsened. None of the BSP-Q calves were treated surgically; in 66.7%, clinical signs gradually worsened and 33.3% of calves spontaneously improved. CONCLUSION: Partial tibial neurectomy is advocated for the treatment of BSP-G and in selected cases of BSP-M. However, only partial resolution of clinical signs should be expected for BSP-M. No surgical treatment exists for BSP-Q calves, although spontaneous improvement is possible.

The effects of functional electrical stimulation on walking in hereditary and spontaneous spastic paraparesis.

OBJECTIVES: To investigate in people with spastic paraparesis (SP): 1) the factors contributing to foot drop and reduced toe clearance while walking; 2) short-term effects of bilateral functional electrical stimulation (FES) of the common peroneal nerve. MATERIALS AND METHODS: Long term (>0.5 years) users of FES with SP were compared to matched controls (N = 11 per group). Ankle strength and plantarflexor stiffness and walking kinematics were objectively recorded. The effects of FES on: 1) perceived efficacy; 2) muscle torque and ankle motion; 3) clinical outcome measures and walking kinematics were assessed. Results were compared using an analysis of covariance. RESULTS: Ankle weakness and stiffness is higher among people with SP. Higher plantarflexor stiffness is associated with reduced swing phase dorsiflexion; higher toe clearance while walking is associated with increased hip flexion. FES increases dorsiflexor torque, improves toe clearance and dorsiflexion in swing phase, and significantly improves walking speed (p < 0.05). CONCLUSIONS: There are multiple causes of tripping in people with SP; FES reduces foot drop and improves walking speed.

Adrenomyeloneuropathy.

A 29-year-old male patient presented with progressive spastic paraparesis of three years duration. He also had gait ataxia which led to recurrent falls. In addition, there was pigmentation of the skin creases, tongue and buccal mucosa. His clinical course was remarkable by recurrent episodes of diarrhea, pulmonary tuberculosis. The investigatory work up showed a normal MRI scan of the brain, spinal cord and normal abdominal structures. The basal serum cortisol levels were low. Adrenomyeloneuropathy was diagnosed and he was started on corticosteroid supplementation. Mineralocorticoid supplementation also is planned in the follow up. The case is being presented for its rarity.

Transition from pediatric to adult health services: the perioperative care perspective.

Increasing numbers of children with congenital and chronic diseases are surviving beyond adolescence. Consequently, arrangements must be put in place to ensure that these groups of patients continue to receive high-quality care into adulthood. Although some medical disciplines have reacted positively to this growing challenge, perioperative care has lagged behind. This review explores some of the difficulties in delivering optimal perioperative care in the transitional period. Key issues, including the paucity of experience in the management of pediatric diseases among adult-trained clinicians and unfamiliarity with child/family focused care, are discussed. We provide some suggestions for the development of transitional healthcare policies.

Botulinum toxin assessment, intervention and after-care for lower limb spasticity in children with cerebral palsy: international consensus statement.

Botulinum neurotoxin type-A (BoNT-A) has been used in association with other interventions in the management of spasticity in children with cerebral palsy (CP) for almost two decades. This consensus statement is based on an extensive review of the literature by an invited international committee. The use of BoNT-A in the lower limbs of children with spasticity caused by CP is reported using the American Academy of Neurology Classification of Evidence for therapeutic intervention. Randomized clinical trials have been grouped into five areas of management, and the outcomes are presented as treatment recommendations. The assessment of children with CP and evaluation of outcomes following injection of BoNT-A are complex, and therefore, a range of measures and the involvement of a multidisciplinary team is recommended. The committee concludes that injection of BoNT-A in children with CP is generally safe although systemic adverse events may occur, especially in children with more physical limitations (GMFCS V). The recommended dose levels are intermediate between previous consensus statements. The committee further concludes that injection of BoNT-A is effective in the management of lower limb spasticity in children with CP, and when combined with physiotherapy and the use of orthoses, these interventions may improve gait and goal attainment.

Dolor neuropático y espasticidad abdomino-crural secundario a lesion medular torácica: manejo con neuromodulación / Neuropathic pain and abdominal-crural spasms following a thoracic bone marrow lesion: management includes the use of neuromodulation

Las funciones o disfunciones de las vías del dolor periféricas y centrales pueden originar un cuadro álgico distinto al nociceptivo, mucho menos frecuente pero igualmente importante, que se describe como dolor neuropático. Este dolor neuropático no es una enfermedad propiamente dicha, es una manifestación de múltiples y variados trastornos que afectan al sistema nervioso central y a sus componentes somatosensitivos. El dolor neuropático puede aparecer en trastornos del sistema nervioso central y, especialmente, en lesiones medulares, esclerosis múltiple y lesiones cerebrovasculares del tronco del encéfalo y el tálamo. El dolor neuropático, que se origina en lesiones del sistema nervioso central, no suele responder a la estimulación medular. Se presenta el caso de un paciente que padece una lesión tumoral dorsal (hemangioma) a nivel torácico con paraparecia espástica secundaria y dolor neuropático no controlado de 7 meses de evolución, al cual se le instala un sistema de neuromodulación, que logra controlar el dolor y la recuperación del paciente.

Lesions or malfunctions of peripheric and central pain pathways may cause algid clinical manifestations, and despite being much less frequent, are equally important. These are described as neuropathics pain. This neuropathic pain is not in itself a disease, but a manifestationof multiple and varied disorders that affect the central nervous system and its somatosensitive components. Neuropathic pain may be associated to disorders of the central nervous system and specially in bone marrow lesions, multiplesclerosis, and brain vascular lesions of the brain trunk and thalamus. Neuropathic pain that results from damages of the central nervous system does not usually respond to bone marrow stimulation. This is the case of a patient with a dorsal tumour lesion (hemangioma) at thoracic level with secondary spastic parapareis and uncontrolled neuropathic pain present for a period of 7 months to which a neuromodulation system is installed in order to control pain and recover the patient, finally accomplishing the two objectives.

Pot paresis: marijuana and a case of hypokalemic periodic paralysis.

A Polynesian (Maori) man presented with muscle pain and weakness 36 h after smoking marijuana and then going on an eating binge, consuming a large amount of salt and carbohydrates. The electrocardiogram showed hypokalemic changes. Serum potassium was 2.0 mmoles/L. His symptoms and the hypokalemia resolved within 12 h of presentation without any treatment.

[Patient-reported outcomes: advances in research and practical application].

Clinical evaluation proceedings from patient-reported outcomes (PRO) are being concerned increasingly. Based on the concept of PRO, the report discusses the findings in the research recently made at home and abroad. PRO from apoplexy patients with spastic paralysis was taken as an example in the exploration of how to evaluate the curative effects in clinical practice on the basis of PRO.

Adrenomyeloneuropathy as a cause of primary adrenal insufficiency and spastic paraparesis.

Adrenomyeloneuropathy is a varient of adrenoleukodystrophy, both of which are rare inherited disorders of peroxisomes characterized by the accumulation of very-long-chain fatty acids in plasma, the central and peripheral nervous systems, adrenal glands and testes, which leads to dysfunction of these organs and systems. In this article, we describe an illustrative case of adrenomyeloneuropathy and discuss the clinical presentation, diagnosis and management of the 2 disorders.

[The genetics of movement disorders--spinocerebellar degenerations]. / Genetikk ved bevegelsesforstyrrelser--spinocerebellaere lidelser.

BACKGROUND: Hereditary movement disorders include spinocerebellar disorders, a large and heterogeneous group of syndromes with ataxia or spasticity as the prominent symptom. In spite of the vast clinical and genetic heterogeneity, patterns of pathogenesis slowly emerge and help us understand these disorders. MATERIAL AND METHODS: This review is based on personal experience and recent literature. RESULTS: More than 20 types of hereditary spastic paraparesis have been reported. Dominant SPG4 and SPG3 with mutations in the spastin or the atlastin gene have been identified in many countries. The most prevalent type of recessive ataxia in Europe, Friedreich's ataxia, has become a model of integrated clinical-molecular-therapeutic research. More recessive ataxias (AOA1-2) have been described recently. More than 20 autosomal dominant ataxias have been reported, with 12 identified genes including the episodic ataxias, and 9 mapped. SCA7 appears to be the most frequent type in some Nordic countries. INTERPRETATION: A striking feature of many of these diseases is the involvement of very different genes for similar phenotypes. Conversely, very heterogeneous phenotypes are due to single-gene defects. Recently there has been considerable progress in the clinical description of movement disorders and the understanding of their genetic basis. Possible therapies are emerging.