Neuromuscular disorders
Ashley W. Blom, David Warwick, Michael R. Whitehouse in Apley and Solomon’s System of Orthopaedics and Trauma, 2017
Most patients with cerebral palsy have a spastic diplegia where the lower limbs are more affected than the upper limbs. Treatment is concentrated on the lower limbs but upper-limb function is also important particularly if the child requires walking aids. In the young child, treatment consists of physiotherapy, the use of AFOs and tone-reducing medications to prevent fixed contractures. By 3–4 years of age the sitting and walking patterns can be observed, and particular attention should be paid to the interrelationship between the various postural defects, especially lumbar lordosis/hip flexion and knee flexion/ ankle equinus. The muscles that cross two joints (iliopsoas, hamstrings and gastrocnemius) are influential in the development of the muscle and joint contractures characteristic of children with a spastic form of cerebral palsy. Surgery is indicated either to correct structural defects (e.g. a fixed contracture or hip subluxation) or to improve gait.
Brain Injury and Infant Cardiac Surgery: Overview
Richard A. Jonas, Jane W. Newburger, Joseph J. Volpe, John W. Kirklin in Brain Injury and Pediatric Cardiac Surgery, 2019
The neurological features of this lesion, i.e., particularly spastic diplegia, relate to the topography of the injury.18,19 Thus, in spastic diplegia, the lower extremity is involved more than the upper extremity. Because of the focal necrosis in the periventricular white matter, the motor fibers emanating from the cerebral cortex and subserving lower extremity function are preferentially affected. Fibers subserving the function of upper extremities course more laterally and thus are less likely to be injured or are injured less. However, because there is also more diffuse injury to cerebral white matter, association fibers are affected, and this involvement may account for at least some of the cognitive deficits that one sees on follow-up in affected infants.
Interferons
Sarah H. Wakelin, Howard I. Maibach, Clive B. Archer in Handbook of Systemic Drug Treatment in Dermatology, 2015
Large infantile haemangiomas are a therapeutic challenge and may cause life-threatening complications. Several studies have reported the effectiveness of IFN-α (2a and 2b) in treating these vascular lesions. IFN-α inhibits endothelial cell migration and proliferation and specific vascular growth factors. As this mode of action is different to corticosteroids, it can be used in lesions that are unresponsive to steroids, and it does not need to be given during the proliferation phase in order to be effective. The onset of action is slower than corticosteroids, and treatment may need to be continued for up to 12 months. Due to toxicity, IFN-α is only indicated for induction of early regression in life-threatening corticosteroid-resistant infantile haemangiomas where propranolol (see Propranolol) has failed or is contraindicated. The most serious adverse effect in infants is a potentially irreversible spastic diplegia.
Unusual gait disorders: a phenomenological approach and classification
Published in Expert Review of Neurotherapeutics, 2019
Vijayashankar Paramanandam, Karlo J. Lizarraga, Derrick Soh, Musleh Algarni, Mohammad Rohani, Alfonso Fasano
Besides ‘scissoring’, further characteristics of the ‘spastic diplegic gait’ pattern have been described. ‘Crouch gait’ or ‘flexed-knee gait’ is one of the earliest gait patterns described in these patients and is characterized by excessive knee flexion during stance (Figure (1b)) [11]. The gait of patients with spastic diplegia usually evolves as the child grows due to the combination of weakness, worsening spasticity with subsequent contractures and orthopedic deformities. Sutherland and Davids [12] described four typical abnormalities of gait affecting the knees in children with spastic diplegia: ‘jump knee’, ‘crouch knee’, ‘stiff knee’ and ‘recurvatum knee’. Based on gait kinematics of 187 patients, Rodda and Graham [13] subsequently classified spastic diplegic gait into 5 subtypes: ‘true equinus’, ‘crouch gait’, ‘apparent equinus’, ‘jump gait’, and ‘asymmetrical gait’. Their classification did not include ‘stiff knee’ and ‘recurvatum knee’ because it was felt to be the result of hamstring surgery. Calf surgery, on the other hand, can play a role in transforming the gait subtype from either ‘true equinus’ or ‘jump gait’ to ‘crouch gait’ or ‘apparent equinus’ [14].
A case of late-onset OCD developing PLS and FTD
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2018
Enrica Bersano, Maria Francesca Sarnelli, Valentina Solara, Fabiola De Marchi, Gian Mauro Sacchetti, Alessandro Stecco, Lucia Corrado, Sandra D’alfonso, Roberto Cantello, Letizia Mazzini
A 64-year-old woman presented to our tertiary amyotrophic lateral sclerosis (ALS) Center reported an 8-month history of muscular weakness in the left leg and, more recently, dysarthria and occasional dysphagia. No history of neurodegenerative or psychiatric disease was present in her relatives. At the age of 57, the patient developed a late-onset OCD, characterized by high state of anxiety and ego-dystonic obsessions and repetitive behaviors (frequent hand washing and object cleaning). She was treated with medication and psychotherapy without benefit. She had no previous medical history or history of substance abuse. On initial examination, she had a spastic diplegia, mild weakness of proximal muscles and asymmetric pyramidal signs in upper and lower limbs side. Needle electromyography (EMG) showed no signs of active denervation, motor-evoked potentials (MEP) detected a delay of the central motor conduction time to the tibialis anterior and first dorsal interosseous on the left side. Hematological screenings (neoplastic and infection markers and antibodies anti-antigens onco neuronalis) were normal, as well as cerebrospinal fluid findings (including dosage of protein Tau, P-Tau, ABeta-42, and isoelectrofocusing). Extensive neuropsychological examination of executive, memory, language, associative, and global functions revealed intact cognitive functioning. Psychological interview supported by administration of Yale–Brown obsessive–compulsive scale, Hospital Anxiety, and Depression Scale and Neuropsychiatric Inventory confirmed OCD with a high state of anxiety.
Daily activities, participation, satisfaction, and functional mobility of adults with cerebral palsy more than 25 years after selective dorsal rhizotomy: a long-term follow-up during adulthood
Published in Disability and Rehabilitation, 2021
Berendina E. Veerbeek, Robert P. Lamberts, A. Graham Fieggen, Peter P. J. L. Verkoeijen, Nelleke G. Langerak
Overall adults with CP showed some differences for daily activities and social roles subscales compared to their typically developing peers. These differences were relatively small; however, the differences for accomplishment of Mobility and Recreation were more substantial showing that adults with CP were more dependent and faced more difficulties for these domains compared to the typically developing adults. This can be explained by the fact that 50% of our subjects with CP were classified as GMFCS level II or III. However, the reported 61% of the adults with CP that experienced difficulties with Mobility in our study is lower than the 77% determined in a study of van der Slot et al. [13], who studied adults with spastic diplegia aged 25–45 years. This in contrast to Donkervoort et al. [22], who reported percentages of 27%, but their study cohort was much younger (16–20 years) and also included hemiplegia (50%) and quadriplegia (25%). Both studies did not report on interventions given in childhood.
Related Knowledge Centers
- Hypertonia
- Neuromuscular Junction
- Spastic Quadriplegia
- Pelvis
- Cerebral Palsy
- Spasticity
- Chronic Condition
- Leg
- Hip
- Spastic Cerebral Palsy