Muscles are the integral part of the body and the functioning of vital organs and vital processes are not possible without the involvement of muscles. Any problem in the structure of muscle leads to variety of symptoms, some of them are lethal. Brain is also affected by muscular dystrophy.
Does Muscular Dystrophy Affect The Brain?
Due to the lack of dystrophin protein in brain, which provides stretching to the muscles, muscular dystrophy has an impact on the brain and it affects it. The effect of muscular dystrophy is more prominently seen on the congenital muscular dystrophy rather than the acquired form.1 Following are the effects of muscular dystrophy on brain:
- Cognitive Impairment: Severe mental retardation may occur in the patients with muscular dystrophy. The severity varies from person to person. There is a delay in mental development and the patient has the difficulty in reading and writing. The comprehension skills are at low level.
- Lower IQ: Patients, especially children with muscular dystrophy are having lower IQ levels relative to the normal person at the same age. Thus, the brain development is impaired.
- Neural Shrinkage: Neural shrinkage may also occur in the patient with muscular dystrophy as the protein dystrophin required to strengthen the muscle is absent. Further, the stretching power of the neurons is reduced. Muscular dystrophy may also result in reduction in the number of neurons. Neural shrinkage is especially seen in the region of brain stem and cerebrum.
- Effect On CNS Make-Up: The patient with muscular dystrophy can experience seizures or epilepsy. This is due to the fact that the insulating power of the nerve fibers are reduced due to low level of myelin and the brain generates untriggered signals. Further, there is an increase in choline compounds indicating brain abnormalities.
- Loss Of Neurons: Loss of neurons may also be caused by muscular dystrophy. This may be due to the absence of various important proteins such as dystrophin.
- Abnormal Dendrites: Reduction in dendritic length, branching of the apical dendrites and the dendritic arborization are also seen in muscular dystrophy. Further, the dendritic abnormalities also negatively impact the cognitive development of the patient.
- Synaptic Function: Muscle weakness and degeneration due to muscular dystrophy affects the synaptic function. Some studies conclude the presence of myasthenia like synaptic dysfunction in cases of muscular dystrophy.
- Effect On EEG: Progressive muscular dystrophy has an impact on the brain function and that is directly diagnosed through EEG. It symptomizes slow dominant rhythm and diffuse slow activity.
- Muscle Eye Brain Disease: This disease is typically found in Japanese people. In this disease, the brain cells fail to identify the right place of growth during the fetal development in uterus, this result in severe mental retardation and visual problems.
- White Matter Abnormalities: White matter abnormalities are associated with the muscular dystrophy and the problem with myelin. Myelin is the substance that is coated around the nerve fibers and is responsible for electrical insulation. However, the impact of the white matter abnormalities not necessarily affects the brain functions.
Symptoms Of Muscular Dystrophy
Following are the symptoms of muscular dystrophy:
- Pain: As the skeletal muscles are also affected by the disease, any movement results in pain. Further, due to the absence of the dystrophin, which provides stretching power to the muscles, the muscles failed to contract and relax.
- Muscle Wastage: As there is no physical activity due to mild to moderate pain, there is a wastage of muscle.
- Respiratory Depression: Respiration is the process done through the coordination of the muscles. Thus, if the muscles fail to contract and relax due to non-stretching it may lead to respiratory depression.
- Cardiac Complications: This is the most serious symptoms of muscular dystrophy as the disease also involves the cardiac muscles.2
- Muscle Spasm: The patient of muscular dystrophy also experiences muscle spasm.
- Difficulty Walking: Due to weakness and denegation of muscles, the patient is either unable to walk or have difficulty in walking.2
- Scoliosis: Spine curvature is one of the main symptoms of muscular dystrophy.
The effects of muscular dystrophy on brain include cognitive impairment, lower IQ, neural shrinkage, loss of neurons, abnormal dendrites and synaptic dysfunction. Various drugs are used to manage the effects such as anticonvulsants are used to manage seizures.