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Motor Neuron Disease (MND)

Name of disease:

Motor Neuron Disease (MND)

ICD-10 diagnosis code:

G12.2

Causes:

Motor neuron disease is a rare disease, Damage to upper and lower motor neurons. Information about the brain and spinal cord fails to reach muscle tissue throughout the body, affecting the movement of various muscle activities. Upper motor neurons are located in the brain, while lower motor neurons are located in the brain trunk and spinal cord There are three main types of the disease, depending on which nerves are implicated:1. Spinal muscular dystrophy (Amyophic Lateral Sclerosis, ALS)2. Progressive muscular dystrophy (Progressive Muscular Atrophy)3. Bulbar Palsy

Mode of inheritance:

Some MNDs are inherited, most MNDs are not known, may relate to environmental, toxic, viral, or genetic factors.

Prevalence:

<1 / 1 000 000

Diagnosis:

The initial stage of the disease is difficult to diagnose, easy to be confused with cervical vertebral disease. There are no tests to directly diagnose MND, but gene tests for SMA. Mostly based on symptoms, neurological reflexes, and the exclusion of other diseases. After physical examination, process thorough neurological exam. Muscle currents (EMG) to measure the muscles strength, NCV nerve conduction test muscle or nerve biopsy can be extracted Laboratory tests of blood, urine, or other substances; Magnetic resonance imaging (MRI); Anterior horn cell disease / Progressive neurological disorders

Age of onset:

Childhood and adult onset In children is familial inherited, symptoms present at birth or before the child learns to walk. In adults, commonly in men than in women,symptoms appear after age 40.

Common signs and symptoms:

There are no particularly obvious signs in the early stages of the disease. Patients feel tired at an early stage, gradually find that their fingers cannot bend and cannot hold the fist, after a while, there will be difficulty speaking, speech and swallowing difficulties. Motor neuron disease affects only the motor nerve and not the sensory nerve, so most patients do not experience hand or foot paralysis or needle sensation. Signs of damage to upper and lower motor neurons: 1. Damage to lower motor neurons: Usually there is a problem with the tongue muscle, tongue muscle atrophy and tremor, followed by weak palate, pharynx, throat muscle and chewing muscle, the patient cannot speak clearly,difficult to swallow. 2. Damage to upper motor neurons: Usually the limb is weak and the movement is slow. 3. Damage to upper and lower motor neurons: Usually the hand muscle weakness, atrophy symptoms, from one hand to the other hand. Sleep disturbance from hypopnea and apnea

Available treatments (medicinal and non-medicinal):

Riluzole (Rilutek®) Nusinersen (Spinraza ™) Edaravone (Radicava™) Palliative

Disease management tips:

Weakness in the neck, throat and chest muscles Nutrition - Soft diet Dysphagia on chew or swallow, requires insertion of a feeding tube and percutaneous gastronomy tube for feeding Sleep disturbance from hypopnea and apnea muscle weakness in the neck, throat, and chest, require full day assisted ventilator Physical therapy, occupational therapy, and rehabilitation may help to improve posture, pevent joint immobility, and slow muscle weakness and atrophy. Stretching and strengthening exercises help reduce spasticity, increase range of motion, and keep circulation flowing. Additional therapy for speech, chewing, and swallowing difficulties. Applying heat to relieve muscle pain. Retain independence assistive devices Such as supports or braces, orthotics, speech synthesizers, and wheelchairs

References:

National Institute of Neurological Disorders and Stroke

https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Motor-Neuron-Diseases-Fact-Sheet%20%20 https://www.slideshare.net/johnywilbert/motor-neuron-diseasepptx-new https://www.movementdisorders.org/MDS-Files1/MDSSSA/12.MotorNeuronDisease_AKPALU.pdf

Other useful websites:

更多疾病資料: 脊醫王鳳恩軟骨減壓脊醫中心http://www.drbackpain.com.hk/