Neuromuscular Diseases PDF
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Bahçeşehir Üniversitesi Diş Hekimliği Fakültesi
Busra YILMAZ
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Summary
This document presents information on various neuromuscular diseases, providing details on topics such as cerebrovascular disease, Parkinson's disease, multiple sclerosis, myasthenia gravis, and epilepsy. It includes outlines, clinical presentations, treatment options, and dental care considerations for patients with these conditions.
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ORAL DIAGNOSIS AND DENTAL RADIOLOGY-II Neuromuscular Diseases Assoc. Prof. Busra YILMAZ School of Dental Medicine Department of Oral and Maxillofacial Radiology [email protected] OUTLINE CEREBROVASCULAR DISEASE PARKINSON’S DISEASE MULTIPLE SCLEROSIS MYASTHENIA GRAVIS EPILEPSY CEREBRO...
ORAL DIAGNOSIS AND DENTAL RADIOLOGY-II Neuromuscular Diseases Assoc. Prof. Busra YILMAZ School of Dental Medicine Department of Oral and Maxillofacial Radiology [email protected] OUTLINE CEREBROVASCULAR DISEASE PARKINSON’S DISEASE MULTIPLE SCLEROSIS MYASTHENIA GRAVIS EPILEPSY CEREBROVASCULAR DISEASE Cerebrovascular disease refers to disorders of the cerebral blood vessels that cause impaired cerebral circulation. Stroke is an impairment in blood flow to the brain due either to lack of blood flow (ischemia) or hemorrhage. Transient ischemic attack (TIA) is defined as a reversible acute short duration focal neurologic deficit (mini stroke). Clinical Manifestations Common signs and symptoms – headaches, dizziness, vertigo, drowsiness, chills, nausea, vomiting. Loss of consciousness and convulsive movements are less common. Weakness or paralysis of extremities occurs in contralateral side. Speech defects may be seen Neurological signs and symptoms – paralysis of one side of body, difficulty in breathing and swallowing, inability to speak or slurring of speech, loss of bladder and bowel control, Infarction – gradual onset of signs and symptoms Embolism and hemorrhage – abrupt onset of signs and symptoms Treatment Early intervention is critical to prevention, treatment and recovery. Reduce bleeding/ thrombolysis with i.v tissue plasminogen activator within 3hrs of a stroke can improve reperfusion. Reduction in hypertension. (diet, exercise, smoking cessation, stress reduction) Antihypertensives Anticoagulants/ antiplatelet therapy. Neuroprotective drugs Dental Considerations Length of time elapsed since the CVA – should not undergo elective dental care within 6 months of the episode Minimization of stress – morning appointments, effective pain control, psychosedation during treatment Assessment of bleeding – 1) Prothrombin time 2) Reduction in levels of anticoagulant if necessary 3) Post surgical bleeding control measures PARKINSON’S DISEASE A progresssive neurodegenerative disorder of neurons that produce dopamine The Dopaminergic Neurons in the Basal Ganglia Are mainly affected Signs & Symptoms Tremor Rigidity Bradykinesia (slownesss of voluntary movement) Facial impassiveness Falls Mood disturbances Insomnia Fatigue MANAGEMENT Increasing the synthesis of dopamine - Levodopa Inhibiting the catabolism of dopamine - Selegiline Stimulating the release of dopamine - Amphetamine Stimulating the dopamine receptor sites directly - Bromocriptine Pramipexole Blocking the uptake and enhancing the release of dopamine Amantadine Oral Consideration The typical “masklike” facial appearance with infrequent blinking and lack of expression is caused by bradykinesis. The muscle rigidity also causes difficulty in swallowing, resulting in drooling. Speech becomes labored because of the lack of muscle control, and mandibular tremor results in masticatory difficulties, especially in those with removable dental appliances. Abnormalities in oral behavior, such as purposeless chewing, grinding, and sucking movements. Antiparkinson’s drugs may cause xerostomia, nausea, tardive dyskinesia *Tardive dyskinesia: a neurological disorder characterized by involuntary movements of the face and jaw. Dental Management Upright position. Anxiety can increase both the tremor and the degree of muscle rigidity. Dysphagia and an altered gag reflex- special precautions must be taken to avoid the aspiration. In patients with hypersialorrhea, maintaining a dry field can be difficult Xerostomia, is a common side effect of antiparkinsonism medications; the consequent root caries and recurrent decay must be diligently treated. Difficulty maintaining their dentition because of their physical disability. Levodopa has a significant orthostatic hypotensive effect MULTIPLE SCLEROSIS Most common autoimmune disease of the nervous system Destroys the fatty tissue called myelin that insulates an axon/nerve, and is called demyelination. Affects the central nervous system and inflames the white matter in the brain which creates plaques. Signs & Symptoms Disturbances in visual function Abnormal eye movements(nystagmus and double vision) Incordination Spasticity Difficulty in walking Loss of balance Vertigo Tremor or paralysis of the limbs Oral Complications and Manifestations Dysarthria produces slow, irregular speech with unusual separation of words referred to as scanning speech Facial Parethesia Numbness of orofacial structures Trigeminal neuralgia Dental Management Patients with stable disease and little motor spasticity or weakness can receive routine dental care With more advanced disease may require help in transferring to and from the dental chair, may have difficulty maintaining oral hygiene, and may be poor candidates for reconstructive and prosthetic procedures Short morning are advised because fatigue is often worse in the afternoon MYASTHENIA GRAVIS (MG) is a disease characterized by progressive muscular weakness on exertion, secondary to a disorder at the neuromuscular junction. Acetylcholine normally transmits the impulse from nerve to muscle at the neuromuscular junction, and cholinesterase hydrolyzes acetylcholine. In patients with MG, autoantibodies that combine with and may destroy the acetylcholine receptor sites at the neuromuscular junction are present, preventing the transmission of nerve impulses to the muscle. Clinical Manifestations The initial signs of this disease commonly occur in areas innervated by the cranial nerves (frequently, the eye muscles). Patients present with ptosis, diplopia, difficulty in chewing or swallowing, respiratory difficulties, limb weakness. In severe advanced cases, respiratory difficulty arises. Diagnosis is made initially on the basis of clinical presentation. The inability of a patient to continually blink the eyes voluntarily is highly suggestive of MG. The clinical diagnosis can be confirmed by improvement of symptoms with the administration of a short-acting anticholinesterase Signs & Symptoms In most cases, the first noticeable symptom is weakness of the eye muscles Diplopia (blurred or double vision) Ptosis (drooping of one or both eyelids) Majority also have weakness of face and throat muscles Dysphagia (difficulty in swallowing) Dysarthria (slurred speech) Dysphonia (voice impairment) Therefore, RISK of choking + aspiration The degree of muscle weakness involved in MG varies greatly among patients Within a year of onset, approximately 85–90% will develop Generalized myasthenia gravis, which is characterized by weakness in the trunk, arms, and legs May lose muscle strength for breathing and need ventilator Management Anticholinesterase drugs such as neostigmine, pyridostigmine provide symptomatic. In patients with more severe disease, remission may be achieved by thymectomy. In other cases, long-term cortico-steroids and immunosuppressive drugs are necessary. Plasmapheresis has been of temporary value in patients with severe exacerbations of MG Oral Health Considerations Oral and facial signs are an important component of the clinical picture of MG. The facial muscles are commonly involved, giving the patient an immobile and expressionless appearance. Tongue edema may also be present in MG, making eating difficult for patients. Patients whose muscles of mastication are weakened can also experience difficulty in chewing. The patient’s masticatory muscles may become so tired that the mouth remains open after eating When treating known MG patients, the dentist must be aware that a respiratory crisis may develop from the disease itself or from overmedication. If a patient is at risk for developing a respiratory crisis, dental treatment should be performed in a hospital where endotracheal intubation can be performed. Adequate suction and the use of a rubber dam The dentist should avoid prescribing drugs that may affect the neuromuscular junction, such as narcotics, tranquilizers, and barbiturates. Certain antibiotics, including tetracycline, streptomycin, sulfonamides, and clindamycin, may reduce neuromuscular activity and should be avoided EPILEPSY It is a paroxysmal disorder of the brain that results from sudden, uncontrolled discharges of cortical or subcortical neurons in the central nervous system, which can lead to disturbances in consciousness. The clinical picture can vary from a simple distraction to a major motor convulsion. The onset of epileptic seizures is sudden and often repetitive. EPILEPSY Stages of seizures: Prodrome stage (the patient may be restless, behavioral changes), Aura period (the patient feels that the seizure is coming), Ictal period (motor signs, tonic-clonic contractions or a picture in which a change in consciousness occurs), Postictal period (the patient is tired, sluggish, unconscious. ) Seizures may be characterized by one or more of the symptoms such as loss of consciousness, localized or generalized tonic spasms, muscle (clonic) contractions, or both, sensory disorders, psychic disorders. It is divided into 2 main groups: 1- Idiopathic epilepsy 2- Symptomatic epilepsy 1- Idiopathic epilepsy: This term is used for epileptic seizures that cannot be directly attributed to an organic brain disorder. It begins in the first 20 years of life and can continue for a lifetime. Seizures occur in two ways: a) Grand Mal b) Petit Mal 1- Idiopathic epilepsy: a)Grand Mal seizure: The tonic-clonic phases are followed by a short-term coma and postictal sleep. The patient falls to the ground with sudden loss of consciousness and the tonic phase begins, which lasts less than a minute. All muscles contract. When the body is in head and neck extension, the extremities are usually straight, sometimes the arms and hands may be flexed. Most of the time the eyes are open. Following the tonic phase, the clonic phase, which can last up to one minute, begins with periodic relaxation and contractions of all muscles. Following the clonic phase, the patient is still unconscious. All extremities are relaxed. Breathing became regular, and the patient's color due to cyanosis became lighter. 1- Idiopathic epilepsy: b) Petit Mal type seizure: There is a hereditary predisposition. It is mostly seen in children (5-12 years old). It is a short-term seizure of unconsciousness limited to seconds (5-10 seconds). In the meantime, the patient's activity stops, unaware of what is going on in the environment and what is being said, he looks at it with his eyes open. Sudden onset seizure ends abruptly. It may disappear spontaneously during adolescence. The postictal state does not occur following this type of seizure. 2- Symptomatic Epilepsy is a term used for epileptic seizures that can be attributed to an identified brain disease. Birth traumas (hypoxia), congenital malformations (such as Sturge-weber disease), brain tumors, cerebrovascular diseases, central nervous system infections are some of the diseases in the etiology. 2- Symptomatic Epilepsy: Generalized convulsions seen in this epilepsy type are no different from grand mal seizures of the idiopathic type. Auras can be in the form of vegetative or psychic manifestations such as unpleasant smell, fear or taste hallucinations, hot flashes, epigastric sensation, and pain. Status Epilepticus: A single clinical seizure lasting more than or equal to 30 minutes or repeated seizures without regaining conscioussnes over a period of more than equal to 30 minutes. It is a very dangerous picture that threatens life. If not treated, the patient may die with deep coma, collapsed fever. Dental Considerations The most important risk for these patients is the occurrence of a seizure during dental treatment. If the patient has seizures more than once a day, this indicates that the patient is not under control. Non-emergency treatments should be postponed. Dental treatment stress, hunger, fatigue, flickering bright lights, epilepsy treatment that has just been stopped or recently changed can stimulate the onset of seizures. Dental Considerations Epileptogenic drugs (such as methohexidon, enflurane, ketamine) should not be used. Drug interactions between the drugs used by the patients and the dentists are important. If the patient is using phenobarbital, pyrimidone; morphine-derived analgesics should not be given to this patient. Because the use of these drugs together causes depression in the Central Nervous System. In addition, in patients using phenytoin, phenobarbital, and pyridone; antibiotics that are metabolized in the liver such as tetracycline and doxycycline should not be given, since these drugs accelerate drug degradation in the liver. Dental Considerations Phenytoin, which is used by most epileptic patients, can cause hyperplasia, especially in young people. This situation mostly occurs 2-18 months after the start of drug use. It should be known that phenytoin may cause bone changes by blocking the effects of parathyroid hormone. Dental Considerations If an epileptic seizure develops during dental treatment: The patient is prevented from harming himself. A tampon should be placed between his teeth, It should be laid on its side so that if it vomits, it does not aspirate, Surrounding items should be removed. If a crisis occurs at the time of treatment, everything in the mouth should be removed immediately, If the seizure is prolonged, I.V or I.M. 10 mg of diazepam can be given If Status Epilepticus is suspected, the patient should be hospitalized immediately. Alzheimer’s disease Most common form of dementia 5-10% in the +60 yrs population 45% in +85 years population Characterized by the progressive failure of recent memory and loss of intellectual functions Symptoms of Alzheimer’s Disease Alzheimer's disease is a brain disorder in which nerve cells in the brain die, making it difficult for the brain's signals to be transmitted properly. A person with Alzheimer's disease has problems with memory, judgement, and thinking, which makes it hard for the person to work or take part in day-to-day life. Most patients' symptoms progress slowly over a number of years. Symptoms may not be noticed early on. Sometimes, it is only when family members look back that they realize when the changes started to occur Impaired memory and thinking -- The person has difficulty remembering things or learning new information. In the later stages of the disease, long-term memory loss occurs, which means that the person can't remember personal information, such as his or her place of birth or occupation, or names of close family members. Disorientation and confusion -- People with Alzheimer’s disease may get lost when out on their own and may not be able to remember where they are or how they got there. They may not recognize previously familiar places and situations. They also may not recognize familiar faces or know what time of the day it is, or even what year it is. Oral consideration and management No unique oral manifestations. Cholinesterase inhibitors may cause sialorrhea, whereas antidepressants and antipsychotic cause xerostomia. Mercury has been implicated as a causative agent for the development of AD. Appropriate history should be elicited from a near relative or the attending nurse. If the patient is uncooperative for essential dental procedures then sedation, with a rapid acting sedative, should be considered. Possibility of GI irritation and bleeding when Anticholinesterases are used with NSAIDS. LA with epinephrine is used with caution. References • Michael Glick (ed.); Martin S. Greenberg (ed.); Peter B. Lockhart (ed.); Stephen J. Challacombe (ed.). Burket's Oral Medicine. 13th edition. Wiley-Blackwell. June 2021. ISBN: 9781119597780