User:Crowen4/Akinetic mutism

Akinetic mutism is a medical term describing patients tending neither to move (akinesia) nor speak (mutism). Akinetic mutism was first described in 1941 by Cairns et al. as a mental state where patients lack the ability to move or speak. However, their eyes may follow their observer or be diverted by sound. Patients lack most motor functions such as speech, facial expressions, and gestures, but demonstrate apparent alertness. They exhibit reduced activity and slowness, and can speak in whispered monosyllables. Patients often show visual fixation on their examiner, move their eyes in response to an auditory stimulus, or move after often repeated commands. Patients with akinetic mutism are not paralyzed, but lack the will to move. Many patients describe that as soon as they ‘will’ or attempt a movement, a ‘counter-will’ or ‘resistance’ rises up to meet them.

Description


Akinetic mutism varies across all patients; its form, intensity, and clinical features correspond more closely to its functional anatomy rather than to its pathology. However, akinetic mutism most often appears in two different forms: frontal and mesencephalic.

Frontal akinetic mutism
Akinetic mutism can occur in the frontal region of the brain and occurs because of bilateral frontal damage. Akinetic mutism as a result of frontal lobe damage is clinically characterized as hyperpathic. It occurs in patients with bilateral circulatory disturbances in the supply area of the anterior cerebral artery.

Mesencephalic akinetic mutism
Akinetic mutism can also occur as a result of damage to the mesencephalic region of the brain. Mesencephalic akinetic mutism is clinically categorized as somnolent or apathetic akinetic mutism. It is characterized by vertical gaze palsy and ophthalmoplegia. This state of akinetic mutism varies in intensity, but it is distinguished by drowsiness, lack of motivation, hyper-somnolence, and reduction in spontaneous verbal and motor actions.

Symptoms
Symptoms of akinetic mutism progress over time.
 * Lack of motor function (but not paralysis)
 * Lack of speech
 * Apathy
 * Slowness
 * Disinhibition

Causes


Akinetic mutism can be caused by a variety of things. It often occurs after brain injury or as a symptom of other diseases. The occurrence of akinetic mutism takes place approximately four months after the symptoms first appear.

Frontal lobe damage
Akinetic mutism is often the result of severe frontal lobe injury in which the pattern of inhibitory control is one of increasing passivity and gradually decreasing speech and motion.

Thalamic stroke
Many cases of akinetic mutism occur after a thalamic stroke. The thalamus helps regulate consciousness and alertness.

Ablation of cingulate gyrus
Another cause of both akinesia and mutism is ablation of the cingulate gyrus. Destruction of the cingulate gyrus has been used in the treatment of psychosis. Such lesions result in akinesia, mutism, apathy, and indifference to painful stimuli. The Anterior cingulate cortex is thought to supply a "global energizing factor" that stimulates decision making. When the Anterior cingulate cortex is damaged, it can result in akinetic mutism.

Other causes
Akinetic mutism is a symptom during the final stages of Creutzfeldt-Jakob Disease (a rare degenerative brain disease) and can help diagnose patients with this disease. It can also occur in a stroke that affects both anterior cerebral artery territories. Another cause is neurotoxicity due to exposure to certain drugs such as tacrolimus and cyclosporine.

Other causes of akinetic mutism are as follows:
 * Thalamic stroke
 * Respiratory arrest and cerebral hypoxia
 * Acute cases of encephalitis lethargica
 * Meningitis
 * Hydrocephalus
 * Trauma
 * Tumors
 * Aneurysms
 * Olfactory groove meningioma
 * Cyst in third ventricle
 * Toxical lesions and infections of central nervous system
 * Delayed post-hypoxic leukoencephalopathy (DPHL)
 * Creutzfeldt-Jakob Disease (mesencephalic form)

Treatments
Akinetic mutism can be misdiagnosed as depression, delirium, or locked-in syndrome, all of which are common following a stroke. Patients with depression can experience apathy, slurring of speech, and body movements similar to akinetic mutism. Similarly to akinetic mutism, patients with locked-in syndrome experience paralysis and can only communicate with their eyes. Correct diagnosis is important to ensure proper treatment. A variety of treatments for akinetic mutism have been documented, but treatments vary between patients and cases.

Magnesium sulfate
Treatments using intravenous magnesium sulfate have shown to reduce the symptoms of akinetic mutism. In one case, a 59 year old woman was administered intravenous magnesium sulfate in an attempt to resolve her akinetic mutism. The patient was given 500mg of magnesium every eight hours, and improvement was seen after 24 hours. She became more verbal and attentive, and treatment was increased to 1000mg every eight hours as conditions continued to improve.

Cyst puncture
As seen below in the case of Elsie Nicks, the puncture or removal of a cyst causing akinetic mutism can relieve symptoms almost immediately. However, if the cyst fills up again, the symptoms can reappear.

Dopamine agonist therapy
Symptoms of akinetic mutism suggest a possible presynaptic deficit in the nigrostriatal pathway, which transmits dopamine. Some patients with akinetic mutism have shown to improve with levodopa or dopamine agonist therapy, and repleting dopamine in the motivational circuit with stimulants, antidepressants, or agonists such as bromocriptine or amantadine.

Other treatments include amantadine, carbidopa-levodopa, donepezil, memantine, and oral magnesium oxide.

Elsie Nicks
14 year old Elsie Nicks was the first patient to be diagnosed with akinetic mutism by Cairns in 1941. She suffered from severe headaches her entire life and was eventually given morphia to help with treatment. She began to enter a state of akinetic mutism, experiencing apathy and loss of speech and motor control. A cyst on her right lateral ventricle was tapped, and as soon as the needle advanced toward the cyst, she let out a loud noise and was able to state her name, age, and address. After her cyst was emptied, she regained her alertness and intelligence, and she had no recollection of her time spent in the hospital. The cyst was drained two more times over the next seven months and was eventually removed. After eight months of rehabilitation, Elsie no longer experienced headaches or akinetic mutism symptoms.

Mr. H
Mr. H was a 35 year old man whose frontal lobe was damaged in a motorcycle accident, causing akinetic mutism. Mr. H became mute, apathetic, and responded to questions with small hand gestures or the nod of his head. Mr. H was unable to speak and answer questions from his observer. However, if his observer stepped outside and called him on the telephone, Mr. H would pick up the telephone and answer any questions he was asked. As soon as the observer returned to the room, Mr. H was once again unable to answer any questions, returning to his state of mutism. This phenomenon is referred to as the Telephone Effect. Mr. H was treated with amantadine and became more interactive over the course of his treatment.

Telephone Effect
The Telephone Effect is related to a dopamine response. The unpredictability of an event is proportionally related to the amount of a dopamine response. An unexpected event, such as a ringing telephone, may cause an increase in dopamine release and activate the motivational circuit. This also suggests dopamine or levodopa as a treatment for akinetic mutism. Although the Telephone Effect is not a treatment, it can be used as a means of communication with a patient experiencing akinetic mutism.

OJ
54 year old patient, OJ, had a thalamic stroke causing frontal lobe dysfunction and somnolent akinetic mutism. He became drowsy, his speech was reduced to mutism, and he experienced behavioral changes such as apathy, slowness, lack of spontaneity, and disinhibition. Over the course of three months, his symptoms worsened until his verbal and motor movements were almost completely reduced. There is no mention of treatment for OJ.

46 year old male
One case describes a 46 year old male admitted to the hospital with fever and meningism. An MRI showed he suffered from a thalamic stroke. His eyes remained open, but he could not comprehend or follow instructions, perform voluntary movements, or feed himself. However, he showed unceasing grasping and unrestrainable manipulation of whatever object (such as clothes, chairs, tubes, etc.) that the examiner placed in his personal space. He did not respond to any pharmacological interventions such as dopamine, amantadine, and acetyl-choline-esterase inhibitors. This case also does not mention any successful treatments.

38 year old male
A 38 year old male suffered from akinetic mutism and dysarthria. His speech was reduced to hypophonia, remained alert, and was able to communicate poorly through eye movements. He suddenly died two weeks after observation, but no autopsy was performed to determine cause of death.