What kind of patients does the term “acromegaly” usually refer to?
The term “acromegaly” is only found in Chinese and is not a translation of any foreign language. In Taiwan, the term “acromegaly” refers to patients with amyotrophic lateral sclerosis (ALS).
”Motor Neuron Disease (MND), also known as Amyotrophic Lateral Sclerosis (ALS) in terms of location, is a progressive motor neurotropic disorder that causes progressive neuromuscular It is a progressive motor neurological atrophy that causes progressive neuromuscular atrophy and disability, as if the limbs are frozen, hence the common name “acromegaly.
Here, the same term has three names, of which “motor neuron disease” (MND) and “amyotrophic lateral sclerosis” (ALS) are the medical names and “acromegaly” syndrome is the common name. It can be seen that in common language, “acromegaly” refers to what is commonly referred to as “motor neuron disease” (MND), which is also called “amyotrophic lateral sclerosis” (ALS) according to the location of the lesion. “(ALS). It is also known as “acromegaly” because the muscles of the patient’s body gradually atrophy and gradually become unable to move, speak, chew, or breathe as if they were frozen. “The special thing about “acromegaly” is not only that it is rare, but also that it is a terminal disease, but that it is particularly cruel. Therefore, although they cannot move their limbs or breathe on their own, their minds are normal, their consciousness is clear, and their senses are as sharp as those of normal people. The “Ice Bucket Challenge” was originally intended to draw the attention of the community to this disease (ALS), and the ice water dunking form was chosen, perhaps to experience the feeling of being frozen. It seems that the people of the world have thought of the same thing!
Originally, it is clear and unquestionable that the term “acromegaly” refers to patients with amyotrophic lateral sclerosis (ALS). But people often call this disease “motor neuron disease” (MND), why? As I understand it, this is particularly indicative of the mechanism underlying acromegaly: the loss or death of “motor neurons” in the body for unknown reasons! The “taper” is the external manifestation of the damage to motor neurons. The problem is that, strictly speaking, motor neuron disease (MND) is a disease of motor neuron damage, which is “a group of diseases that cause progressive degeneration of motor neurons resulting in generalized muscle atrophy and weakness”, although “Although ALS is the most common motor neuron disease in adults and is often referred to as “motor neuron disease”, ALS is not, after all, “amyotrophic lateral sclerosis” (ALS). “ALS is the most common MND, but MND is not ALS, MND includes ALS, so what other motor neuron diseases or other conditions might also be called What other diseases may be called “acromegaly”?
What other patients may be called “acromegaly”?
ALS is called “acromegaly” because of its “acromegaly” characteristic: the muscles of the whole body gradually atrophy and gradually become unable to move, speak, chew, or breathe, as if they were frozen, and the disease lasts about 3-5 years. Respiratory muscle involvement leads to respiratory paralysis or secondary lung infection and death. I think we can agree that whether other motor neuron diseases can be called “acromegaly” depends on the extent to which they share the “acromegaly” characteristics of ALS.
When the concept of motor neuron disease (MND) was first introduced by neurologists, there were four main types of MND.
(1) Amyotrophic lateral sclerosis (ALS)
(2) Progressive muscular atrophy (PMA)
(3) progressive medullary palsy (PBP)
(4) primary lateral sclerosis (PLS).
Both PMA and PBP usually progress to ALS, and it is generally accepted that ALS, PMA, and PBP are different subtypes of the same disease, so there should be no problem calling all three types of patients “acromegaly”.
A significant number of patients with primary lateral sclerosis (PLS) progress to ALS after 3 years, so they can also be called “acromegaly”. But those who do not progress to ALS have a better prognosis, with a median survival of ≥20 years! And there is no obvious muscle atrophy, so it is hard to be considered as “progressive”!
Motor neuron diseases also include.
(5) Frozen arm and leg syndrome (FAS, FLS). (5) FAS and FLS, which are relatively benign, although they show signs of “taper”, so many researchers tend to consider them to be a benign variant of ALS and a lucky “taper”!
Some motor neuron diseases are inherited.
(6) Spinal muscular atrophy (SMA), a congenital genetic disorder, manifests clinically as lower motor neuron involvement, i.e. hypotonia, muscle weakness and myasthenia, with more proximal than distal involvement. The first two types have a short course of disease starting in infancy, while type IV can start in adulthood.
(7) Spastic paraplegia (HSP) is a congenital genetic disease, and the symptoms are mainly in the lower extremities, so the characteristics of “progressive freezing” are greatly reduced, and I am afraid it is difficult to be called “progressive freezing”.
(8) Kennedy’s disease (KD, also known as spinal muscular dystrophy BSMA), is a congenital genetic disease, only seen in men, mostly in the age of 30-40 years old, the lower motor neurons are mainly involved, there are “progressive” manifestations, but the progress of the disease is much slower than ALS. I know a patient who thought it was ALS for more than 10 years, but when he was diagnosed with Kennedy’s disease, he was a little excited to see me and said, “I’ve got my hat off!” He has been very passionate about caring for people with ALS for many years, and the nice girl from CCTV, Zhang Quanling, did the “ice bucket challenge” right in front of his house. The Kennedy patient is not considered a “frozen person”? For example, I know a patient who thinks he is better than ALS and is not a “taper”.
The following two diseases belong to the so-called “ALS-like syndrome”.
(9) Post-polio syndrome (PPS). Some polio patients may experience muscle weakness and atrophy at the original site or at a new site decades after their symptoms have improved, which is called post-polio syndrome. The clinical presentation is a slowly progressive manifestation of lower motor neuron involvement, without involvement of upper motor neurons. The disease progresses slowly, and there is no specific treatment, but the overall prognosis is good, so it is not a “taper”.
(10) Multifocal motor neuropathy (MMN), a rare demyelinating peripheral neuropathy recognized in recent years, involves only the motor system and presents with asymmetric muscle weakness and atrophy of the distal upper extremities, gradually involving the proximal and lower extremities, and also the lower extremities. The prognosis is relatively good, with a long course and slow progression, no upper motor nerve involvement, no ball involvement, and MMN is treatable and effective, not “acromegaly”.
Other diseases with symptoms of muscle atrophy include.
(11) peronial myoatrophy, also known as CMT, is the most common group of familial peripheral neuropathies, accounting for about 90% of all hereditary neuropathies. The common features are chronic progressive peroneal muscle atrophy with childhood or adolescent onset, with peroneal muscle atrophy as the main clinical feature, but there is often a clear boundary between lower and upper extremity atrophy. In private opinion, this is clearly not an “acromegaly” disease.
(12) Hirayama disease, also known as benign youth hand muscle atrophy, is a symptom that appears due to something or other (we lay people can’t understand), and it occurs in young men, with a male to female ratio of about 20:1. However, Hirayama disease has an earlier age of onset, a benign course of the disease that usually stops after several years of onset, and more limited sites of muscle weakness and atrophy. In my private opinion, this is clearly not an “acromegaly” disease.
Which patients are definitely not “tachyphylaxis”?
”The main manifestation of ALS is neuromuscular disorders. People usually know very little about ALS, but they are relatively familiar with some myopathies, the most common ones are “myasthenia gravis” and “progressive myotonic dystrophy”. In the past 5 years, my wife has been suffering from ALS, there are often people who are kind and enthusiastic to recommend doctors, saying who has cured this disease, always mistaking my wife for myasthenia gravis or progressive muscular dystrophy!
(13) Myasthenia gravis (MG) is an autoimmune disease characterized by easy muscle fatigue, light in the morning and heavy in the evening, and reduced after rest or with cholinesterase inhibitors. It often involves the extraocular muscles, masticatory muscles, swallowing muscles and respiratory muscles. In severe cases, bulbar palsy may occur. The distribution of affected muscles varies from person to person and from time to time, and is not a manifestation of paralysis that occurs when a particular nerve is damaged. Although the current cure rate is relatively low, there are ways to treat it in both Western and Chinese medicine.
(14) Progressive myodystrophy is a group of primary skeletal muscle disorders caused by genetic factors, whose clinical manifestations are slowly progressive muscle atrophy, muscle weakness, and varying degrees of motor impairment. The disease can be caused by multiple genetic modalities, each with different clinical manifestations, resulting in many types.
Although the symptoms of these two diseases are similar to those of acromegaly, modern medicine considers these three diseases to be completely different, why? Science (may not be entirely accurate).
The activity of human muscles requires three links.
① motor neurons (nerve cells) to send motor commands.
② motor commands are transmitted to the muscles through the neuromuscular junction.
③ Normal muscles receive motor commands and move.
The three links are indispensable, and any problem with any of them will lead to muscle atrophy and weakness, and any problem with link 1 is motor neuron disease, link 2 is myasthenia gravis, and link 3 is progressive muscular dystrophy. In other words, motor neuron disease is a lesion of the nervous system, the neuromuscular junction and the muscle itself are not diseased, and myasthenia gravis is caused by neuropathy; myasthenia gravis is caused by problems in the neuromuscular junction, resulting in the muscle not receiving motor commands, and the motor neuron (nerve cell) and the muscle itself are not diseased; progressive myotonic dystrophy is a lesion of the muscle itself directly caused by a genetic disorder of the muscle cell.
In addition, motor neurons are divided into upper motor neurons in the brain and lower motor neurons in the brainstem and spinal cord. The upper motor neurons control the lower motor neurons, which are directly connected to the muscles through the neuromuscular junction and send motor signals to the muscles. If the upper motor neuron is diseased, the lower motor neuron loses control of the “superior” and sends motor signals to the muscle “unconsciously and continuously”, causing the muscle to tense and spasm. In the case of ALS, both the upper and lower neurons have lesions, so there is both muscle tonicity and spasm, as well as muscle weakness and atrophy. In myasthenia gravis, because the muscles do not receive motor signals, there is only muscle weakness and atrophy; in progressive muscular dystrophy, because the muscles themselves are diseased, there is only muscle weakness and atrophy.
Therefore, although all three diseases belong to neuromuscular diseases and all have muscle atrophy, they are completely different in terms of pathogenesis, diagnosis and treatment. Myasthenia gravis” and “progressive muscular dystrophy” are not motor neuron diseases, and they do not have obvious “progressive” characteristics, so they are not “progressive” diseases. They are not “tardive dyskinesia”.
As a family member of a “progressive thaw” patient, I have often visited the neurology department of Beihang Hospital, which is very authoritative in the treatment of “this disease”, for 5 years. What they expect is that the Fan Hospital will say that they are not suffering from “this disease”, that is to say, they are not ALS or “acromegaly”! I am also glad to see that patients who have long suspected this disease are relieved that they are finally diagnosed as not having this disease. Interestingly, recently, some patients who were not suffering from this disease (such as singer Wang Hailong, a patient with progressive muscular dystrophy) have repeatedly claimed to be “acromegaly”.
IV. Conclusion
”The term “acromegaly” originally referred to patients with amyotrophic lateral sclerosis (ALS).
ALS is the most common type of motor neuron disease (MND). What is commonly referred to as “motor neuron disease” (MND) and “amyotrophic lateral sclerosis” (ALS) “are the same disease”!
As for other kinds of motor neuron diseases that damage motor neurons, after our identification above, (2) progressive muscular atrophy (PMA), (3) progressive medullary palsy (PBP), and partly (4) primary lateral sclerosis (PLS) are extremely close to ALS, and are almost the same disease, also “acromegaly ” disorder. Another part (4) of PLS and (5) patients with linked arm and linked leg syndrome (FAS, FLS) may be called benign or good luck “acromegaly”. (6) spinal muscular atrophy (SMA), (7) spastic paraplegia (HSP), (8) Kennedy’s disease (KD, also known as spinal muscular atrophy BSMA), (9) post-polio syndrome (PPS), (10) multifocal motor neuropathy (MMN), (11) peronial myoatrophy (also known as CMT), and (12) Hirayama disease (HAS, FLS). (12) Hirayama disease (Hirayama disease) is not “acromegaly”. (13) myasthenia gravis, and (14) progressive muscular dystrophy are not motor neuron diseases and are not tachyphylaxis.