Les soins aux enfants et aux adolescents ayant la paralysie cérébrale (aux niveaux III à V du GMFCS).

La paralysie cérébrale (PC) est l'incapacité physique la plus fréquente chez les enfants canadiens. Les soins complets des enfants ayant la PC, qui sont capables de marcher et qui fonctionnent aux niveaux I et II du système de classification de la fonction motrice globale (GMFCS) sont déjà exposés dans un point de pratique. Le présent document complémentaire traite des soins aux enfants ayant la PC qui fonctionnent aux niveaux III à V du GMFCS. Ceux qui fonctionnent aux niveaux III et IV du GMFCS utilisent des dispositifs d'assistance comme des déambulateurs, des cannes ou des aides à la mobilité motorisées, tandis que ceux qui fonctionnent au niveau V du GMFCS ont besoin d'assistance à la mobilité comme un fauteuil roulant manuel. Le présent document contient un aperçu des principaux concepts relatifs à la détection précoce, aux services de réadaptation et aux possibilités de traitement pour les enfants présentant ces niveaux de PC, conjointement avec des ressources pratiques pour contribuer à la surveillance de la santé assurée par les pédiatres qui s'occupent de cette population.

[Neurodevelopment and neuroprotection in young children]. / Le neurodéveloppement et la neuroprotection du jeune enfant.

In France, the most pessimistic estimates put the prevalence of neurodevelopmental disorders (NDD) at 15 % of births. The two largest populations of newborns at highest risk of NDD are premature babies and babies born into siblings with one or more infants who already have an autism spectrum disorder or another NDD. The high prevalence of these disorders justifies a health promotion policy, centred on the child and his or her family. Prevention is based on the early identification of high-risk factors, by informing families and training pregnancy and early childhood professionals, and implementing perinatal prevention protocols for high-risk newborns (antenatal corticosteroid therapy and magnesium sulfate for women at risk of preterm delivery before 32 weeks, developmental care, therapeutic hypothermia for full-term infants with early neonatal encephalopathy presumed to be anoxic). Preventing the severity of NDD depends on their early identification, as early as possible in the highest plastic "1000 days" developmental window, a smooth flow of diagnosis and care for mothers and children, and the establishment of an ecosystem that includes multi-modal early intervention, at the best in multi-disciplinary teams such as the early medical and social action centres, support for families through guidance programs and inclusion in the community, first in day-care centers and then in nursery schools.

Postoperative outcomes for unilateral congenital trochlear nerve palsy: A retrospective cohort study.

PURPOSE: Congenital trochlear nerve palsy is the most common cause of vertical strabismus. The goal of this study was to investigate surgical outcomes after superior oblique tendon plication with or without inferior oblique recession in children and adults with unilateral congenital trochlear nerve palsy. METHODS: Data and outcomes were collected in patients with a diagnosis of unilateral congenital superior oblique palsy during a retrospective single-center study conducted at the University Hospital of Tours. A reproducible, standard ophthalmological and oculomotor examination was performed pre- and postoperatively at 1 year, including presence or absence of diplopia, vertical and horizontal deviations, and compensatory head posture. Surgical success, defined as an endpoint including absence of diplopia in primary position, absence of head tilt, and vertical deviation at distance fixation<5 prism diopters (PD), was analyzed. RESULTS: A total of fifty-seven patients (median [IQR] age of 11 years [5-42]) were analyzed. Patients experienced a significant reduction in vertical distance and near deviations (p<0.001), compensatory head tilt (p < 0.001), and diplopia after surgery (p < 0.001). Surgical success was higher in adults (17/24, 70.8%) than in children (15/33, 45.5%), although this did not reach statistical significance (p=0.0657). CONCLUSION: This study suggests that plication of the superior oblique muscle tendon, with or without recession of the inferior oblique muscle, can be effective in treating unilateral congenital trochlear nerve palsy. Further studies are necessary to compare surgical procedures and investigate their efficacy in adults compared to children in the short and long term.

Efficacy of Kinesio® tapes in obstetrical brachial plexus injury: a randomized controlled trial.

OBJECTIVES: The purpose of this study was to investigate the effectiveness of Kinesio® tape application associated to exercise in patients with obstetric brachial plexus injury (OBPI). MATERIALS AND METHODS: Ninety patients with Erb-Duchenne palsy secondary to OBPI participated in a 3-month study, in 2 groups; study group (n = 50) and control group (n = 40). Both followed the same physical therapy program, while the study group also received Kinesio® taping over the scapula and forearm. The patients were evaluated, pre- and post-treatment, using the Modified Mallet Classification (MMC), Active Movement Scale (AMS), and active range of motion (ROM) of the plegic side. RESULTS: There were no statistically significant intergroup differences in age, gender, birth weight or plegic side (p > 0.05), or in pre-treatment MMC and AMS scores (p > 0.05). There were significant differences in favor of the study group for Mallet 2 (external rotation) (p = 0.012), Mallet 3 (hand on the back of the neck) (p < 0.001), Mallet 4 (hand on the back) (p = 0.001) and total Mallet score (p = 0.025), and for AMS shoulder flexion (p = 0.004) and elbow flexion (p < 0.001). ROM results before and after treatment (within groups) showed significant improvement in both groups (p < 0.001). CONCLUSION: Since this a was a preliminary study, the results should be interpreted with caution in terms of clinical efficacy. The results suggest that associating Kinesio® taping to conventional treatment helps functional development in patients with OBPI.'

Etiologies, periocular manifestations and surgeries for congenital and childhood acquired facial nerve palsy.

AIM: To report periocular surgeries performed for patients with congenital and childhood acquired facial nerve palsy (FNP). METHODS: A retrospective case series of pediatric patients who presented with FNP over the last 34 years, was conducted at two tertiary eye hospitals in Riyadh. Data were collected from electronic charts, hospital records and external photos. Main outcome measures were visual acuity, lagophthalmos, eyelid abnormalities, Bell's phenomena, exposure keratopathy, and corneal scar; in these cases, periocular surgeries were required. RESULTS: Among the 90 recruited subjects; the mean age of onset was 4.8±5.4 years old (range, 0.01 to 17.76 years). Traumatic and congenital causes of FNP were the most common, representing over 80% of the cases. Seventy-one patients developed lagophthalmos, 26 with severe exposure that resulted in scarring. Thirty-six (40%) cases had associated strabismus. Lower lid retraction was the most common eyelid abnormality noted in 23 cases, followed by entropion in 16 and ectropion in 6 cases. Temporary tarsorrhaphy was performed in three patients (3.3%), while 18 patients (20%) needed permanent tarsorrhaphy. Gold weight implants were placed in 17 patients (18.9%). Lower lid retraction repair was performed in twelve patients (13.3%). Five patients (5.6%) underwent lower eyelid entropion repair, and three patients (3.3%) underwent lower eyelid ectropion repair. CONCLUSIONS: Lagophthalmos is the most common finding in children presenting with FNP and needs to be managed early to prevent permanent visual loss. Compared to adults, children may present with a different spectrum of eyelid abnormalities, with lower lid retraction and entropion being the most common eyelid malpositions.

Radiation therapy-induced left vocal cord paralysis following lung stereotactic body radiation therapy: A case report and review of the literature.

We report the case of a 50-year old women with an oncological history of metastatic breast carcinoma who underwent lung stereotactic body radiation therapy (SBRT) of 60Gy in 8 fractions for a left upper lobe metastatic lesion. Seven months later, she complains about hoarseness and weakness of voice. Tumoral relapse and other frequent etiologies were excluded. The diagnosis of radiation induced left recurrent laryngeal nerve paralysis causing left vocal cord paralysis (VCP) was made. The symptomatology did not improve till the disease progression and death of the patient 29 months after SBRT. VCP after lung SBRT is a rare adverse event that has not yet been well described in the medical literature.

Le suivi de l'extrême prématuré après le congé des soins intensifs néonatals.

La survie des extrêmes prématurés (moins de 28 semaines d'âge gestationnel) s'est améliorée au fil du temps. Bon nombre s'en sortent bien et ont une bonne qualité de vie. Ils demeurent toutefois vulnérables à des problèmes de santé, y compris des difficultés neurosensorielles et neurodéveloppementales, que les médecins de première ligne, les pédiatres et les cliniques spécialisées doivent surveiller. Le présent document de principes passe en revue les conséquences médicales et neurodéveloppementales potentielles pour les extrêmes prématurés dans les deux ans suivant leur congé et fournit des stratégies de counseling, de dépistage précoce et d'intervention. Parce qu'ils sont tous liés à l'extrême prématurité, la dysplasie bronchopulmonaire ou les troubles respiratoires, les problèmes d'alimentation et de croissance, le développement neurosensoriel (vision et audition), la paralysie cérébrale et le trouble du spectre de l'autisme doivent faire rapidement l'objet d'une évaluation. Pour évaluer la croissance et le développement, il faut corriger l'âge chronologique en fonction de l'âge gestationnel jusqu'à 36 mois de vie. Par ailleurs, l'attention au bien-être émotionnel des parents et des proches fait partie intégrante des soins de qualité de l'extrême prématuré.

[Buccinator muscle: A new target for botulinum toxin injections in the treatment of facial paralysis sequelae]. / Le muscle buccinateur : une nouvelle cible pour les injections de toxine botulique dans le traitement des séquelles de paralysie faciale.

AIM OF THE STUDY: Facial paralysis can cause aesthetic and functional sequelae such as spasms and synkinesis. Botulinum toxin injection is one of the key treatments for these sequelae. Its use is widely reported in the literature for many muscles of the face but little for the buccinator muscle. However, its hyperactivity can be disabling, generating chewing and smile disorders in particular. The aim of this prospective study is to report the effects of botulinum toxin using a questionnaire specifically for the buccinator muscle. PATIENTS AND METHODS: The study was conducted on 13 patients. The facial paralysis was of various origins. The questionnaire consisted of 10 questions each assessing a facial mimic involving the buccinator muscle. The rating scale ranged from -10 (major deterioration) to 10 (major improvement) for each question. The evaluation was done at 1 and 4 months after the injection. We found an improvement for all the mimics and gestures evaluated (0 to 7.38 at 1 month and 0.15 to 6.62 at 4 months). This improvement was greater at 1 month than at 4 months. Few side effects were reported. CONCLUSION: Botulinum toxin injections are an effective, safe therapeutic solution for the treatment of the facial paralysis sequelae of the buccinator muscle through a new specific questionnaire. Subsequently, it would be interesting to carry out an evaluation on a larger population and to compare it with other recognized scores in facial paralysis.

[Stabilization and reanimation of the lower lip in facial palsy. Retrospective study about 66 patients and literature review. Proposition of a decision algorithm]. / Stabilisation et réanimation de la lèvre inférieure paralysée. Étude rétrospective de 66 cas et revue de la littérature. Proposition d'un algorithme décisionnel.

Several techniques for the stabilization and the reanimation of the paralyzed lower lip have been proposed, sometimes combined but the authors are rather vague. Thanks to our cases and a literature review, we suggest an algorithm. Sixty-six patients haven been reviewed. The static and dynamic clinical evaluation have been done thanks to the study of the medical files, photographs and videos samples. The different techniques we have used were: (a) botulinum toxin in the contralateral depressor labii inferioris (DLI) (n=66), contralateral DLI myectomy (n=1); (b) a strip of fascia lata (n=16) either combined with a lengthening temporalis myoplasty (MAT) (n=10) or only realizing a passive suspension thanks to one vector (n=4), or a passive suspension with two strips (n=2); (c) digastric muscle transfer (n=4). When the elevator muscles are sounds, botulin toxin (± myectomy) is proposed if the lower lip is not inverted. Otherwise, a suspension thanks to a digastric muscle transfer or a passive suspension with a horizontal and a vertical strips are recommended. If the elevator muscles are no more operational, a MAT with a passive suspension with one vector is considerated and if the lower lip is inverted, a digastric muscle transfer with a fascia lata strip or a MAT with two strips is proposed. This algorithm submission allows to refine the treatment of the stabilization and the reanimation of the lower lip.

Comparison of outcome between operative treatment and constraint-induced movement therapy for forearm and wrist deformities in cerebral palsy. A randomized controlled trial.

Several treatment strategies for upper extremity deformities in cerebral palsy have been studied for their effectiveness in terms of function and quality of life. There are a few comparative studies between nonoperative and operative management for upper extremity deformities. This study aimed to compare the outcomes between conservative methods using constraint-induced movement therapy (CIMT) and operative methods using pronator teres rerouting, flexor carpi ulnaris transfer to extensor carpi radialis brevis (FCU transfer to ECRB) and fractional lengthening in cerebral palsy patients with forearm pronation and wrist flexion contracture. A total of 19 participants aged 7-15 years were randomized to either CIMT or surgery. The primary endpoint was changes in Shriners Hospital Upper Extremity Evaluation (SHUEE) functional score. Secondary endpoints comprised range of motion, Volkmann's angle, and the Pediatric Outcomes Data Collection Instrument (PODCI). At 1-year follow-up, there was no significant difference between the nonoperative and operative groups in any SHUEE dimensions, range of motion or PODCI. However, there was significant improvement in Volkmann's angle in the operative group compared to the CIMT group: CIMT = 11.11 ± 9.92, surgery = -13.00 ± 4.72: p = 0.04 (95% CI = 1.68-46.54). Operative treatment provided more improvement in wrist posture, represented by Volkmann's angle, in cerebral palsy patients. However, SHUEE, range of motions and PODCI were comparable between the CIMT and operative groups.

Palsy of elbow extension.

Elbow extension palsy is generally well tolerated, because when standing up, it is alleviated by gravity. In the case of trunk paralysis or brachial plexus palsy, standing is possible, thus the restoration of active elbow extension improves the hand's positioning above the shoulder, and allows the elbow to be locked in extension, which is necessary during certain activities such as cycling. In these palsy cases, the triceps brachii will be reinnervated by nerve transfers if surgery is performed early enough before irreversible atrophy of the effector muscle sets in. In these situations, secondary tendon transfers are rarely indicated. Few available muscles can be harvested without deleterious consequences on the donor site. Finally, in patients with a very deficient upper limb but with a healthy contralateral limb, when nerve transfers are no longer possible, elbow extension will not be restored. In the tetraplegics using a wheelchair, elbow extension becomes essential for positioning the hand in space and for potentiating the transferable muscles to activate the hand. As nerve transfers have rare indications and are currently being validated in this population, palliative tendon transfers are the reference technique. They must be integrated into an overall upper limb reconstructive surgery program that takes into consideration the potentially usable muscles and the presence of elbow flexion contracture and supination deformity of the forearm. Elbow extension restoration techniques are based on the transfer of two muscles, the posterior deltoid and the biceps brachii. The first is very effective and has very specific requirements, notably good anterior stabilization of the shoulder by the pectoralis major, while the second has broader indications, notably in the case of elbow contracture and inability to stabilize the shoulder anteriorly.

Tendon transfers for C8-T1 palsy of the brachial plexus in adults.

Brachial plexus palsy after C8-T1 nerve root injury is rare, but causes a loss of finger flexion and extension, which greatly limits the patient's grip function. It can benefit from nerve transfers if the diagnosis is made early. Otherwise, tendon transfers may be proposed. Transfers of the extensor carpi radialis longus and brachioradialis to the flexor digitorum profundus and the flexor pollicis longus, respectively, restores finger flexion and thumb flexion. Tenodesis of the extensor digitorum communis allows passive extension of the fingers during active wrist flexion. Translocation of the flexor pollicis longus and the creation of a "lasso equivalent" on the flexor digitorum superficialis provides some recovery of the intrinsic function of the fingers and thumb. Finally, a nerve transfer of the lateral cutaneous nerve of forearm on the superficial branch of the ulnar nerve can improve sensitivity on the ulnar edge of the hand to limit the risk of cutaneous lesions, which frequently occur in this type of paralysis.

Tendon transfer surgery for radial nerve palsy.

Palliative tendon transfer is an integral part of radial nerve palsy treatment. It can be considered in the first weeks when the possibility of nerve repair by direct suture or nerve grafting is not feasible or reasonable. Mostly, it is discussed secondarily when it is too late for nerve surgery and motor recovery cannot be expected, or after failure or incomplete recovery after nerve repair. The goal of tendon transfers is to restore wrist, finger and thumb extension. For wrist extension, the use of pronator teres is well accepted. The best tendon transfer for finger extension is debated. This can be restored doing a flexor carpi ulnaris (FCU), flexor carpi radialis or flexor digitorum superficialis (FDS) to extensor digitorum communis transfer. Regarding thumb extension and abduction, a palmaris longus (PL) or one FDS tendon to the rerouted extensor pollicis longus (EPL) transfer can be performed. If a transfer is done on the EPL without rerouting it, abduction can be restored by doing a tendon transfer to the abductor pollicis longus (APL) or an APL tenodesis. The different tendon transfer options are selected based on the surgeon's preference, and most importantly, discussed with the patients to define the objectives together. The transfer is chosen based on the clinical examination (high or low radial nerve palsy, tendon available for transfer like PL, wrist mobility) and based on the patient's needs and expectations (activities requiring the FCU, finger independence, independence of thumb extension or abduction). If the surgical rules and the postoperative instructions for rehabilitation are followed, tendon transfers for radial nerve palsy regularly produce very satisfactory results.

Palliative surgery for foot drop.

Dysfunction of the common peroneal nerve is the most common mononeuropathy in the lower limb and a source of significant disability for patients. The nerve can be damaged at various levels for various reasons (direct or indirect trauma, extrinsic compression, anatomical variant, endocrine, rheumatological, or neurological disease). Clinical evidence of foot drop with steppage gait is very typical. Conservative treatment should be considered as a first step (avoidance of the contributing factors, functional rehabilitation, foot drop brace ± injection). If properly conducted conservative treatment is not successful, palliative surgery is indicated: either tendon transfer using the posterior tibial tendon or ankle arthrodesis.

[Augmented psychotherapy with rTMS in functional neurological disorder]. / Psychothérapie augmentée par rTMS pour les troubles neurologiques fonctionnels.

BACKGROUND: We report the observation of a 47-year-old woman with functional neurological disorder (tetraparesis, mixed tremors and non-epileptic seizures) treated with a protocol of augmented psychotherapy in combination with repeated transcranial magnetic stimulation (rTMS). INTERVENTION: We carried out a biofeedback psychotherapy protocol with rTMS (twenty sessions, two sessions per day for ten days; 1Hz, 150% of the motor threshold, twenty minute sessions, 300 pulses per session) in which the patient visualized the motor activity of her upper limbs during stimulation of the primary motor area (PMA). The evolution of neurological symptoms was assessed using the Medical Research Council Scale for Muscle Strength (MRC). RESULTS: Symptoms were improved between the 4th and 6th days of treatment (8th and 12th sessions) with first a relief of paresis, then a secondary cascade improvement of other functional symptoms. At two months the patient no longer presented any functional neurological symptoms. DISCUSSION & CONCLUSION: We propose several hypotheses concerning the effectiveness of this type of biofeedback protocol using rTMS. We also suggest that this type of protocol should be systematically associated with psychotherapeutic support on biographical elements for holistic management. This observation underlines the interest of potentiating cognitive-behavioral therapies using the principle of operant conditioning with the aid of brain stimulation in functional neurological disorders, and motivates the realization of future studies.

Correction of ulnar claw hand and Wartenberg's sign.

Ulnar claw hand usually occurs when the ulnar nerve is damaged distally. Claw hand deformity is characterized by metacarpophalangeal hyperextension and interphalangeal flexion, making it impossible to oppose the fingers and thumb. Bouvier's test is used to guide the procedure. Palliative surgery requires prior preparation of paralytic hands. In case of a positive Bouvier's test, Zancolli's lasso technique is preferred because of its effectiveness. Capsuloplasty with anteroposterior transosseous fixation is used if the bone is strong enough and when flexor digitorum profondus muscle is inactive and does not allow flexor digitorum superficialis tendon transfer. In case of a negative Bouvier's test with interphalangeal extension deficit of 45° of less, direct interossei muscle restoration techniques by active transfers are performed. If the interphalangeal extension deficit is more than 45°, proximal interphalangeal arthrodesis is indicated. Wartenberg first described actively irreducible abduction of the little finger. Wartenberg's sign is seen when ulnar paralysis occurs, and during ulnar nerve regeneration. Treatment of isolated Wartenberg's sign consists of re-routing the extensor digiti minimi. Among the other techniques, Belmahi's "tie lasso" is preferred when flexible claw hand is associated with Wartenberg's sign.

Restoration of the first dorsal interosseous muscle.

Motor dysfunction of the 1st dorsal interosseous (DIO) muscle is typically observed in low and high ulnar nerve palsy. This causes weak thumb-index pinch, which can be disabling for the patient. Various reconstructive techniques have been described; however, the choice often depends on the surgeon's experience, the presence of associated neurovascular and musculotendinous injuries, as well as the requirements of the palliative surgery schedule. Nerve transfers can be proposed when patients present early in the course of the disease. Tendon transfers are often a last resort when late presentation occurs. Tendon transfers must follow general principles: the insertion is made on the 1st DIO terminal tendon; the tension must be adjusted in a neutral position to avoid excessive tension, and immobilization is maintained for 4 weeks. Although many transfers are possible, the extensor pollicis brevis transfer is our preferred option. This donor does not require additional tendon grafting, has a direct line of pull close to that of the 1st DIO and is not often used for other reconstructive purposes.

Wrist fusion in peripheral paralysis.

In cases of paralysis of the upper limb, wrist fusion is useful in selected indications, especially when there are little to no tendon transfers available to restore finger function and wrist extension. Wrist fusion is particularly useful in the sequelae of brachial plexus lesions and in total paralysis of the radial nerve with hand drop and preserved wrist flexors. Numerous fusion techniques have been proposed. In cases of sequelae of brachial plexus lesions, locking of pronation-supination is associated with the wrist fusion. The use of anatomical plates has dropped the non-union and complication rates.

Indications for amputation after traumatic brachial plexus injury in adults: Case report and review of new prosthetic technologies.

Hand amputation can be discussed after traumatic brachial plexus injury when the patient's hand is non-functional, painful and/or insensitive. That indication is more common in English-speaking countries than in European or Latin countries. New prostheses are now on the market and can be used after hand amputation to improve the functional prognosis in well-selected patients. A 26-year-old male was involved in a road accident in January 2016. He had a traumatic brachial plexus injury and underwent nerve surgery to restore the biceps brachii and triceps brachii. One year after nerve transfer, his hand was insensitive and non- functional. After multidisciplinary consultation, it was decided to amputate his hand to replace it with a myoelectric prosthesis. Shoulder subluxation pain and complications associated with an insensitive and heavy arm were improved after hand amputation. Functional outcomes were improved with this revolutionary myoelectric prosthesis technique. The patient was able to use his prosthesis for many daily activities. Selective amputation indications after traumatic brachial plexus injury are a heavy and stiff arm with shoulder subluxation pain and an unusable claw hand. The patient's motivation must be high.

Surgical restoration of the upper limb in cervical spinal cord injury patients.

Prior to the 1950s, relatively few patients who suffered a transection of the cervical spinal cord survived their injury. Improved medical care and better coordination have resulted in greater numbers of patients surviving and leaving the hospital. The pioneering work of individual surgeons during the 1960s and 1970s stimulated interest in surgical restoration of upper limb function in tetraplegic patients. Since the publication of Moberg's monograph in 1978, surgical improvement of the upper limbs is regarded as one of the options that should be offered to tetraplegic individuals to improve their function. Patients are classified according to the level of spinal cord injury and the residual motor function (international classification: groups 1-9). Surgical procedures are adapted to the motor level for each group of patients. Indications for these procedures are well standardized, the techniques are well mastered, and predictable results can be expected. New nerve transfer techniques have been developed in recent years; they are currently being evaluated.