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Examination of a Child with Cerebral Palsy
Published in Nirmal Raj Gopinathan, Clinical Orthopedic Examination of a Child, 2021
Tone is resistance felt to a passive stretch in a relaxed state of muscle activity and is best demonstrated by passive movement about a joint. The child is assessed in a relaxed state and can be examined in the parent’s lap. It may be increased in the form of spasticity, dystonia, or rigidity. The criteria described by Sanger et al.6 to assess hypertonia can be followed: Palpate the muscle to be examined to assess whether there is muscle contracture at rest.Define the available passive range of motion by moving the limb gently.Now move the limb at different speeds through the available range to look for catch, i.e., sudden resistance offered to the passive range and further possible slow stretch.Now the patient is asked to move the same joint on the other side and look for an alteration in resistance to movement or involuntary movement on the side being examined, which may indicate the presence of dystonia.
Musculoskeletal and Soft-Tissue Emergencies
Published in Anthony FT Brown, Michael D Cadogan, Emergency Medicine, 2020
Anthony FT Brown, Michael D Cadogan
Tetanus itself is rare, but worldwide it is an important cause of death in parts of Asia, Africa and South America. The incubation time from injury to first symptoms ranges from 3 to 21 days (usually about 10 days).The most common symptoms are jaw stiffness (trismus), dysphagia, neck stiffness and abdominal and back pain. Hypertonia is found on examination.Localized or generalized painful spasms follow within 24–72 h, becoming more severe and prolonged from minimal stimuli.Death may occur from laryngospasm, respiratory failure or autonomic dysfunction.There is no rapid diagnostic test to prove the diagnosis, therefore admit a suspected case immediately to the intensive care unit (ICU).
Clinical Manifestation of Mitochondrial Disorders in Childhood
Published in Shamim I. Ahmad, Handbook of Mitochondrial Dysfunction, 2019
Clinical symptoms vary broadly. They represent continuous spectrum covering neonatal onset of the disease with poor prognosis, as well as milder forms with dystonia as a isolated disease manifestation in adulthood (Bachmann-Gagescu et al., 2009). The most common features are developemental delay, epilepsy, neurodevelopement disorders or cerebellar atrophy. Patients can also present with hypotonia, less commonly hypertonia, hearing and visual disorders and failure to thrive. Neurological manifestations include microcephaly, ataxia, neuropathy, ptosis, dystonia, dystonic hemiplegia and choreoathetoid movements. Epilepsy is particulary frequent, manifesting with spasm and focal seizures Respiratory dysfunction (Kussmaul breathing, respiratory failure) may occur, especially in children with neonatal-onset of the disease. Mild craniofacial dysmorphy is more frequent in boys than in girls, with narrow head, head frontal bossing, prominent philtrum, and a depressed flat nasal bridge (Sperl et al., 2015). Most patients have elevated lactate level in blood with decreased ratio between lactate and pyruvate. Leigh syndrome may be sometimes found also in milder forms of the disease (Magner et al., 2011). Diagnosis can be proved by measurement of PDHC activity in muscle tissue or cultivated fibroblasts, but the diagnosis is based upon molecular-genetic testing, next-generation sequencing technologies come into focus.
An expansion of phenotype: novel homozygous variant in the MED17 identified in patients with progressive microcephaly and global developmental delay
Published in Journal of Neurogenetics, 2022
Rafiullah Rafiullah, Alia M. Albalawi, Sultan R. Alaradi, Majed Alluqmani, Muhammad Mushtaq, Abdul Wali, Sulman Basit
Perinatal history of both affected individuals was unremarkable. Head circumference in both affected individuals was more than 2 SD below the mean. Patient IV:3 was diagnosed at the age of 4 months with severe ID and progressive microcephaly. The onset of seizures was noted at the age of 2 years. The EEG showed multifactorial spikes and poly spikes during sleep. Magnetic resonance imaging (MRI) findings at the age of 6months revealed mild atrophy of vermis, cerebellar, and cerebral as well as mild delay in myelination, small thalami, and thin brainstem (Figure 1(B)). Both patients never acquire speech and were unable to walk at the age of 3 and 5 years. Moreover, both patients experience severe eye tracking problems. Muscle tone was found high (hypertonia). Patients failed to move their upper or lower limbs and were completely bedridden.
Relationship between subarachnoid and central canal hemorrhage and spasticity: A first experimental study
Published in The Journal of Spinal Cord Medicine, 2021
Selim Kayaci, Mehmet Dumlu Aydin, Baris Ozoner, Tayfun Cakir, Orhan Bas, Sare Sipal
Spasticity is defined as muscle hypertonia that is characterized by muscle resistance, which increases in response to an externally applied movement that occurs when the muscle is tighter than its threshold level or rotation angle.1 Such condition is a characteristic of upper motor neuron syndrome.2 Clinically, this phenomenon refers to increased muscle tone, and widened reflex areas with increased deep tendon reflexes and clonus and is characterized by an increase in muscle resistance in response to passive muscle tension.3 It is an isokinetic movement disorder. Unlike that noted in hyperkinetic disorders (such as dystonia or chorea) the amount of movement does not increase.3 This condition is distinguished from rigidity based on the speed of muscle tension and its correlation to the existence of other positive upper motor neuron symptoms.4
Classification of Congenital Zika Syndrome: Muscle Tone, Motor Type, Body Segments Affected, and Gross Motor Function
Published in Developmental Neurorehabilitation, 2021
Janiele de Sales Tavares, Gabriela Lopes Gama, Marcia Cristina Dias Borges, Agda Cristina de Sousa Santos, Jousilene de Sales Tavares, Melania Maria Ramos Amorim, Adriana Melo
Although a wide spectrum of motor impairment has already been described in children with CZS, the typical phenotype of this syndrome remains to be established. Studies have described severe motor impairment,8 associated with hypertonia.15,28,29 However, the fact that the methods used to evaluate muscle tone in those studies were insufficiently described renders comparison impossible. Furthermore, the children evaluated were younger than those in the present sample, also hampering comparison,13 since, during motor development, other disorders such as the occurrence of convulsive seizures, sensory impairment and delayed motor development may affect muscle tone.30 Moreover, this is a disease that has only recently been described and the resulting brain abnormalities are severe, with little information available on how peripheral innervation is affected; therefore, characterizing muscle tone remains challenging.