mask face

Introduction
Cause
Examine
Diagnosis

Introduction

Introduction Increased muscle tone can occur in the muscles of the extremities, neck and face. The facial muscles are reduced, the eyes are rarely rubbed, the rotation of the eyes is reduced, the expression is sluggish, like wearing a mask, commonly known as the "mask face." The patient's facial muscles are stiff, rarely rubbing his eyes, his eyes are turning less, his expression is dull, and he seems to wear a mask. This is what medically calls a "mask face." "Mask face" is a typical symptom of Parkinson's, and it is one of the manifestations of exercise retardation. Parkinson's disease, also known as idiopathic Parkinson's disease (PD), referred to as Parkinson's disease, also known as paralysis agitans (shaking palsy), is a common neurodegenerative disease in middle-aged and elderly people. The most common extrapyramidal disease in humans. The main lesions are in the substantia nigra and striatum pathways, and dopamine production is reduced. The prevalence rate of people over 65 years old is 1000/100,000. With age, men are slightly more than women. The main clinical features of the disease: resting tremor, slowness and reduction of movement, increased muscle tone, and instability of posture are the main features.

Cause

Cause

(1) Causes of the disease

The etiology of idiopathic Parkinson's disease has not been known so far. Some central nervous system degenerative diseases with Parkinson's disease symptoms, mainly degeneration of different parts of the central nervous system, there are other clinical features, it can be called symptomatic Parkinson disease, such as progressive supranuclear palsy (PSP), lines Substantia nigra degeneration (SND), Shy-Drager syndrome (SDS) and olive pons cerebellar atrophy (OPCA). There are also some diseases or factors that can produce clinical symptoms similar to PD, such as infection, drugs (dopamine receptor blockers, etc.), poisons (MPTP, carbon monoxide, manganese, etc.), vascular (multiple cerebral infarction) and brain trauma. Etc., clinically known as Parkinson's syndrome (Palkinsonism).

So far, the cause of PD remains unclear. Current research tends to be associated with a combination of ageing, genetic susceptibility, and exposure to environmental toxins.

1, ageing: Parkinson mainly occurs in middle-aged and elderly people, the incidence is rare before the age of 40, suggesting that age is related to the disease. Studies have found that from the age of 30, dopaminergic neurons, tyrosine oxidase and dopa decarboxylase activity, striatum dopamine transmitter levels gradually decrease with age. However, only a small number of elderly people suffer from this disease, indicating that physiological dopaminergic neurons are not enough to cause disease, and age aging is only a trigger for the onset of this disease.

2, environmental factors: epidemiological investigation found that the prevalence of Parkinson's disease has regional differences, so people suspect that there may be some toxic substances in the environment, damage to the brain's neurons.

3. Genetic susceptibility. In recent years, Al53THr mutation of a common nuclear gene has been found in patients with familial Parkinson's disease. But it has not been confirmed many times in the future.

4, family hereditary: medical experts in the long-term practice found that Parkinson's disease seems to have a tendency to family aggregation, the family members of patients with Parkinson's disease have a higher incidence of relatives than the normal population.

It is now widely accepted that Parkinson is not a single factor and multiple factors may be involved. Genetic factors can increase the susceptibility to disease, and only through interaction with environmental factors and aging, through oxidative stress, mitochondrial failure, calcium overload, excitatory amino acid toxicity, apoptosis, immune abnormalities and other mechanisms lead to black A large number of degeneration of dopaminergic neurons are lost and the disease occurs.

(two) pathogenesis

1. The pathogenesis is very complicated and may be related to the following factors.

(1) Age aging: PD mainly occurs in middle-aged and elderly people. It is rare before 40 years old, suggesting that aging is related to the disease. The study found that from the age of 30, the activity of DA neurons, tyrosine hydroxylase (TH) and dopa decarboxylase (DDC), striatum DA transmitter decreased, DAD1 and D2 receptor density decreased. However, the elderly suffer from PD, after all, is a minority, indicating that physiological DA can degenerate neurons to cause the disease. In fact, only the substantia nigra DA neurons can be reduced by more than 50%, and the striatum DA transmitters are reduced by more than 80%. The symptoms of PD will appear in the clinic, and aging is only the triggering factor of PD.

(2) Environmental factors: Epidemiological surveys have shown that long-term exposure to pesticides, herbicides or certain industrial chemicals may be a risk factor for PD. In the early 1980s, some drug users in California used a neurotoxic substance, the pyridine derivative 1-methyl 4-phenyl 1,2,3,6-tetrahydropyridine (MPTP), which appeared to be like primary. Some pathological changes, biochemical changes, symptoms and drug treatment responses of PD have similar effects on monkeys injected with MPTP. Neurotropic MPTP and certain insecticides and herbicides may inhibit the activity of NADH-CoQ reductase (complex I) in the mitochondrial respiratory chain of the substantia nigra, resulting in decreased ATP production and increased free radical production, leading to degeneration of DA neurons. There is significant lipid peroxidation in PD substantia nigra, and reduced glutathione is significantly decreased, suggesting that antioxidant mechanisms and oxidative stress may be related to PD.

(3) Genetic factors: About 10% of patients have a family history, with incompletely explicit autosomal dominant or recessive inheritance, and the rest are sporadic PD. Twin-consistent studies have shown that genetic factors may play an important role in some young (<40 years old) patients. It has been determined to date that 10 single genes, such as PARK 1 to 10, are associated with PD, and it has been confirmed that three gene products are related to familial PD:

1-synuclein is a mutation in the PARK1 gene, and the gene is located on the long arm 4q21-23 of chromosome 4, and -synuclein may increase the sensitivity of DA neurons to neurotoxin.

2Parkin is a mutation in the PARK2 gene, which is located on the long arm 6q25.2~27 of chromosome 6.

3 Ubiquitin C-terminal hydroxylase-L1 is a mutation in the PARK5 gene, which is located on the short arm 4p14 of chromosome 4. Cytochrome P45O2D6 gene and some mitochondrial DNA mutations may be one of the susceptible factors of PD, which may decrease the activity of P450 enzyme, impair the detoxification function of liver, and easily cause damage to nigrostriatal striatum by MPTP and other toxins.

(4) Oxidative stress and free radical formation: Free radicals can cause lipid peroxidation (LPO) of unsaturated fatty acids, which can oxidize damage to proteins and DNA, leading to cell degeneration and death. Due to the increased activity of type B monoamine oxidase (MAO-B), patients with PD can produce excess OH groups and destroy cell membranes. At the same time of oxidation, the DA oxidation product in the substantia nigra cells polymerizes to form neuromelanin, which combines with iron to produce a Fenton reaction to form OH. Under normal circumstances, there are enough antioxidants in the cells, such as glutathione (GSH), glutathione peroxidase (GSH-PX) and superoxide dismutase (SOD) in the brain, DA Oxidation produces free radicals that do not produce oxidative stress and are protected from free radical damage. PD patients have reduced GSH and increased LPO in the substantia nigra, increased iron ion (Fe2) concentration and decreased ferritin content, making the substantia nigra a site susceptible to oxidative stress.

(5) Mitochondrial function defects: In recent years, it has been found that mitochondrial function defects play an important role in the pathogenesis of PD. The understanding of mitochondrial dysfunction in PD patients stems from a study of the mechanism of action of MPTP, which causes Parkinson's disease by inhibiting the activity of the mitochondrial respiratory chain complex I. In vitro experiments confirmed that MPTP active ingredient MPP can cause a decrease in mitochondrial membrane potential (m) and an increase in oxygen free radical production in MES 23.5 cells. The activity of mitochondrial complex I in the patients with PD can be reduced by 32% to 38%, and the decrease in the activity of the complex alpha increases the sensitivity of the nigral cells to free radical damage. No change in complex I activity was observed in the substantia nigra of patients with multiple system atrophy and progressive supranuclear palsy, suggesting that the decrease in PD substantia nigra I activity may be a relative specific change in PD. The presence of mitochondrial dysfunction in PD patients may be related to genetic and environmental factors. Studies suggest that mitochondrial DNA mutations exist in PD patients. Complex I is encoded by both nucleus and mitochondria. Any fragment defect in both groups can affect complex I function. .

(6) Excitotoxicity: Some authors used microdialysis and HPLC to find that the content of excitatory amino acids (glutamate, aspartic acid) in the striatum of PD monkey model prepared by MPTP was significantly increased. If the extracellular space glutamate concentration is abnormally increased, the receptor will be over-stimulated and it will have a significant toxic effect on the CNS. Animal experiments have found that intracerebral injection of trace glutamate can cause large neuronal necrosis. Glutamate neurotoxicity acts through receptors. NMDA receptor-mediated excitotoxicity is associated with DA neuron degeneration. Glutamic acid can damage nerve cells by activating nitric oxide (NO) by activating NMDA receptors, and releasing more excitatory amino acids, further aggravating neuronal damage.

(7) Cytotoxicity of calcium: human aging may be associated with increased concentration of free Ca2 in nerve cells, decreased Ca2/Mg2-ATPase activity, and decreased mitochondrial calcium storage capacity. Changes in intracellular Ca2 concentration affect many important functions of neurons, such as cytoskeletal maintenance, neurotransmitter function, protein synthesis and Ca2-mediated enzyme activity, calcium-binding protein, especially 28KD vitamin D-dependent calcium-binding protein (Calbindin-D28K) May play an important role, related to calcium / magnesium - ATPase activation, with neuroprotective effects. Icopini and Christakos reported that the content of Calbindin-D28K and mRNA in the substantia nigra, hippocampus and dorsal nucleus of PD patients were significantly lower than those in normal subjects, suggesting that the decrease of calbindin gene expression may also lead to cytotoxicity.

(8) Immunological abnormalities: Abramsky (1978) proposed that PD is associated with immune abnormalities. Clinical studies have found that PD patients have decreased cellular immune function and decreased IL-1 activity. McRae-Degueurce et al reported the presence of anti-DA neuron antibodies in cerebrospinal fluid (CSF) of PD patients. Cell culture found that PD plasma and CSF inhibited the function and growth of DA neurons in the midbrain of rats. Stereotactic injection of blood IgG into PD patients was observed, and the tyrosine hydroxylase (TH) and DA neurons were significantly reduced, suggesting that it may initiate or participate in immune-mediated nigral cell damage. Tumor necrosis factor- (TNF-), IL-6, epidermal growth factor (EGF), transfer growth factor- (TGF-) and 2-microglobulin (2-MG) may be involved in the pathogenesis of PD.

(9) Apoptosis: Studies have shown that there are apoptosis, free radicals, neurotoxins and neurotrophic factors in the pathogenesis of PD. Agid (1995) detected the apoptotic morphological and biochemical features of DA neurons in patients with PD, and found that about 5 neurons in the brain of PD patients have characteristic apoptosis and TNF- receptor (-TN- FR) and bcl-2 proto-oncogene expression, apoptosis may be a basic step in the degeneration of DA neurons.

It is generally believed that PD is not a single factor causing disease and may involve multiple factors. Genetic factors increase the susceptibility to disease, and under the combined action of environmental factors and ageing, the degeneration of DA neurons can be induced by oxidative stress, mitochondrial function, calcium overload, excitatory amino acid toxicity and apoptosis. Causes the disease.

2. Pathological changes: The main lesion of PD is degeneration and loss of pigment-containing neurons, and the DA neurons of the substantia nigra pars compacta are most prominent. Microscopically, neuronal cells were reduced, melanocytes in the substantia nigra disappeared, and melanin particles were scattered in tissues and macrophages, with varying degrees of gliosis. Normal human substantia nigra cells decrease with age. When the substantia nigra cells are 80 years old, they are reduced from the original 425,000 to 200,000, and there are less than 100,000 PD patients. When symptoms appear, DA neurons can lose more than 50%, blue spots. Mild changes were also observed in the nucleus of the nucleus, the dorsal nucleus of the vagus nerve, the globus pallidus, the putamen, the caudate nucleus, and the subthalamic nucleus. Lewy corpuscles in the cytoplasm of residual neurons are important pathological features of this disease. Lewy bodies are glass-like masses composed of cytoplasmic proteins with a dense core in the center and a filamentous halo around them. . A cell can sometimes be seen in a number of different sizes of Lewy bodies, found in about 10% of residual cells, black matter is obvious, globus, striatum and blue spots are also visible, -synuclein and ubiquitin are Lewy small An important component of the body.

3. Neurochemical changes: DA and acetylcholine (Ach) act as two important neurotransmitters in the striatum, and their functions are mutually antagonistic. Maintaining the balance between the two plays an important role in regulating the activity of the basal ganglia. The DA transmitter pathway in the brain is mainly a substantia nigra-striate system. The DA neurons of the substantia nigra pars compacta take L-tyrosine from the bloodstream and form levodosine under the action of intracellular tyrosine hydroxylase (TH). L-dopa; further produces dopamine (DA) by dopamine decarboxylase (DDC); through the substantia nigra-striatum bundle, DA acts on the putamen, caudate nucleus, and is decomposed into High vanillic acid (HVA).

Due to a decrease in TH and DDC in idiopathic Parkinson's disease, DA production is reduced (L-dopa is reduced by L-tyrosine production, and DA production is decreased). Monoamine oxidase B (MAO-B) inhibitor can reduce DA catabolism in neurons and increase DA content in brain. The catechol-oxygen-methyltransferase (COMT) inhibitor reduces peripheral metabolism of L-dopa and maintains a stable plasma concentration of L-dopa.

Degeneration of the substantia nigra DA neurons in PD patients, degeneration of the nigrostriatal striatum DA pathway, and a significant decrease in the striatum DA content (>80%), which makes the Ach system function relatively hyperactive, resulting in increased muscle tone, decreased movement, etc. The biochemical basis of motor symptoms. In recent years, the DA content in the midbrain-marginal system and the midbrain-cortex system has also been significantly reduced, which may lead to advanced neurological activity disorders such as mental decline, behavioral abnormalities, and speech disorder. The degree of DA transmitter reduction is consistent with the patient's symptom severity. The early stage of the lesion is increased by the rate of DA renewal (pre-synaptic compensation) and the DA receptor is hyper-sensitive (post-synaptic compensation), and the clinical symptoms are not obvious. Reimbursement), typical PD symptoms (decompensation period) with disease progression. Other basal ganglia or neuropeptides such as norepinephrine (NE), serotonin (5-HT), substance P (SP), enkephalin (ENK), and somatostatin (SS) also vary.

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Clinical manifestation

PD usually develops from 40 to 70 years old, and the incidence increases after 60 years of age. It is rare in 30 years old, and only 4 cases in a group of 380 PD patients; slightly more males. Insidious onset, slow development, mainly manifested in resting tremor, increased muscle tone and retarded exercise, etc., the symptoms appear first and then vary from person to person. The first symptom was the most tremor (60% to 70%), followed by walking disorders (12%), myotonia (10%), and bradykinesia (10%). Symptoms often start from one upper limb, gradually affecting the ipsilateral lower limb, contralateral upper limb and lower limb, showing an "N" shape progression (65% to 70%); 25% to 30% of cases can start from one lower limb, two Lateral lower limbs are rare at the same time. In many cases, there are still left and right differences in the symptoms of advanced disease.

However, no matter how to treat, chronic progressive disease, and many patients need help after a few years is its inherent clinical features. Based on the typical performance of PD and the positive response to dopa drugs, a definitive diagnosis can generally be made. However, some subclinical symptoms or atypical cases are difficult to recognize at an early stage, and early diagnosis and early treatment have an important impact on the quality of life in the later stage. This is also the focus of current clinical research. For most patients and clinicians, it is difficult to determine and determine the onset date of the PD, the initial symptoms, and the time to determine slow movements and tremor symptoms. According to a report by Li Danian et al., it is speculated that the preclinical symptoms of PD may be 3 to 5 years. For this reason, PD symptoms can be divided into two stages: preclinical symptoms and clinical symptoms.

1. Preclinical Symptoms The earliest preclinical symptoms were only reported in Fletcher (1973) et al., but the symptoms they raised have not yet received attention. These symptoms mainly include the following two aspects:

(1) Paresthesia: In fact, as early as Parkinson's book "Vibration Paralysis", "Some PD cases may have rheumatoid pain before their onset of motor symptoms." In the same year, Charcot also described the same for 2 patients with PD. . Until the 1970s, Fletcher and Snider made a detailed description of the preclinical symptoms and sensory disturbances of PD. By the 1980s, William et al. combined electrophysiology to classify sensory disturbances. He reported that the sensory symptoms were mainly caused by numbness, tingling, ant sensation and burning sensation at the joints of the affected limbs. At the beginning, it is mostly intermittent or migratory, and the latter performance is fixed. Conventional nervous system examination showed no obvious objective dysfunction, and electrophysiological examination showed the somatosensory evoked potential (SEP) of some cases, especially the latency and conduction time of the lower limbs. By the early 1990s, we conducted a retrospective survey of 150 patients. The result was that all patients experienced sensory abnormalities of the limbs before the onset of clinical symptoms of PD, and this abnormality can continue, but with exercise. Obstacles are not parallel. Electrophysiological examinations are mainly somatosensory, cortical evoked potentials with a central delay and conduction delay and prolonged latency.

(2) Restless limbs and easy fatigue: In addition to subjective sensory abnormalities, about 1/2 patients experienced discomfort such as acid, swelling, numbness or pain that is difficult to describe in the early stage, and this discomfort is mostly tired. After the break occurs or is obvious, after knocking and beating, it can be relieved, and it is like the performance of restless legs syndrome. In addition, some patients' limbs are prone to fatigue, especially in the upper limbs of the wrist joints, shoulder joints, lower limbs of the ankle joints and knee joints. When tired, these parts may have a slight tremor that is difficult to find. It is effective to take general analgesics at the beginning of these symptoms, and it has no effect after several months. At this time, the drug can be obviously treated after taking the drug.

2. Clinical symptoms: There are obvious individual differences in the initial symptoms. There are reports of subjective sensory abnormalities of 85%, tremors of 70.5%, muscle stiffness or slow movement of 19.7%, dysfunction and/or writing disorder of 12.6%. Gait disorder was 11.5%, myalgia and pain were 8.2%, mental disorders such as depression and anxiety were 4.4%, language disorder was 3.8%, general malaise or muscle weakness was 2.7%, drooling and mask face were 1.6%.

(1) Static tremor: often the first symptom of PD, a small number of patients, especially those over 70 years old, may not have tremor. The mechanism is caused by the irregular and alternating activities of the affected muscle group and the antagonistic muscle group. Early manifestations often occur at the distal end of the limb, starting at one side. The tremor of the hand in the upper limb is more common, and some patients start at the knee of the lower limb. When the component with the rotation participates, the thumb and the index finger tremor may appear. The tremor frequency is generally 4 to 8 Hz, which occurs when it is at rest. It stops when it is vigorously moved, it is intensified when it is stressed, and disappears when it is sleeping. After several years, the upper and lower limbs or the contralateral side of the same side are involved. In severe cases, the head, jaw, lips, tongue, throat, and limb tremor may appear. The limbs of the patient, such as fists or loose fists, can cause tremors on the other limbs. This test helps to detect early mild tremors. In addition to static tremor in the late stage, some patients may be combined with action or posture tremor.

(2) muscular rigidity: myotonia is one of the main symptoms of PD, mainly due to the increased balance of active muscle and antagonist muscle. If it exists in passive motion, it is called lead tube-like rigidity or tension. If it is accompanied by tremor, it can feel a gear-like feeling when passively moving, so it is called gear-like rigidity or tension. . The muscle rigidity occurs in the wrist and ankle of the affected side at the earliest, especially after the patient is tired, and the muscle-like muscle tension is increased when the passive passive wrist and ankle joint are gentle. Due to the increase in muscle tone, the patient can be brought a series of abnormal symptoms, such as blinking, chewing, swallowing, walking and the like.

The following clinical trials have helped to find mild muscle rigidity:

1 Let the patient exercise the contralateral limbs, and the muscles of the tested limbs can be more obvious.

2 head dropping test: The patient is in the supine position, and the head often falls slowly when the head pillow is quickly removed, rather than falling quickly.

3 Let the patient put the elbows on the table, make the forearms perpendicular to the tabletop, and relax the arms and wrist muscles as much as possible. Normally, the wrist and the forearm are about 90° flexed, and the PD patients have more or less wrists. Keeping it straight, if it is erected, it is called a road sign phenomenon. In elderly patients, muscle rigidity causes joint pain, which is caused by increased muscle tension and obstruction of joint blood supply.

(3) bradykinesia (bradykinesia): reduced voluntary movements, including difficulty in initial movement and slowness of movement, due to increased muscle tone, posture reflex disorder, a series of characteristic dyskinesia symptoms, such as getting up, turning over, walking and changing direction Slowness, facial expression muscle activity is reduced, often binocular gaze, blinking reduction, masked face, finger fine movements such as button buttons, laces, etc., the writing is getting smaller and smaller, writing a small sign ( Micrographia) et al.

The movement of PD patients is slow or not the main cause of disability. In the past, it was thought that the movement of PD could not be caused by muscle rigidity. In fact, there is no causal relationship between the two. It has been preliminarily demonstrated that the reduction in the movement of PD is not a very complex symptom, and it is mainly related to the function of the driver of the subcortical extrapyramidal system or the obstacle of the extrapyramidal motor activation device. Because the symptoms of myotonia are significantly improved after surgical treatment of patients who cannot exercise, the frequency of exercise is not as consistent as the administration of dopa.

(4) Postural gait abnormality: Postural reflex disorder is the main symptom of life difficulties for PD patients, which is second only to exercise reduction or exercise failure. The patient's limbs, trunk and neck muscles are in a particularly flexed posture, the head is tilted forward, the trunk is flexed, the upper elbow flexes, the wrists are straight, the forearms are adducted, the interphalangeal joints are straight, the thumb is palm, and the lower limbs are hips. The joints and knee joints are slightly curved, the early lower limbs are dragged, and gradually become a small gait. It is difficult to start. After the start, the front rushes, and the faster the faster, the more can not stop or turn in time, called "panic gait" (festination) ), the upper limb swings are reduced or disappeared while walking; the trunk is stiff due to the torso, and the trunk and the head are accompanied by a small step of turning, which is related to the instability of the center of gravity caused by the balance of posture. The patient is afraid of falling, and he has to stop when he encounters a small obstacle. With the progress of the disease, the posture disorder is aggravated, and it is difficult to stand up from the sitting position and the lying position in the late stage. At present, there is no clear explanation for the mechanism of this inherent posture reflex disorder in PD patients. Some people think that this symptom is mainly related to the rupture of the globus pallidus from the thalamus to the cortex.

(5) Other symptoms:

1 repeatedly tapping the upper edge of the patient's eyebrow arch can induce blinking (Myerson sign), normal people do not continue to respond; may have eyelid palsy (closed eyelids mild tremor) or eyelids (eyelid involuntary closure).

2 mouth, pharynx, diaphragmatic dyskinesia, slow speech, low monotonous voice, hooliganism, etc., severe dysphagia.

3 common sebaceous glands, sweat glands secrete hyperactivity caused by oily face (oily face), sweating, gastrointestinal motility disorders caused by intractable constipation, sympathetic dysfunction leading to orthostatic hypotension, sphincter function is not tired.

4 psychiatric symptoms are more common with depression, anxiety and agitation may occur, and some patients have mild cognitive decline and visual hallucinations in the late stage, usually not serious.

3. The clinical classification and classification of PD was written by Wang Xinde, and was established in October 1984 by the National Extrapyramidal Conference.

(1) Primary (Idiopathic Parkinson's disease, ie tremor paralysis):

1 according to the disease type:

A. Benign type: The course of disease is longer, with an average of 12 years of fluctuations in motor symptoms and later onset of mental symptoms.

B. Malignant type: The course of disease is short, with an average of 4 years. Exercise symptoms and mental symptoms appear earlier.

2 according to symptom classification:

A. Tremor type.

B. Less movement and rigidity.

C. tremor less movement and tonic type with dementia type.

D. tremor less movement and rigidity type without dementia type.

3 by genetic classification:

A. Familial Parkinson's disease.

B. Juvenile Parkinson's disease.

(2) secondary (Parkinson's syndrome, symptomatic Parkinson's syndrome):

1 Infectious (including chronic viral infection) Parkinson's syndrome (sleeping encephalitis, other encephalitis, etc.) after encephalitis.

2 Toxic (carbon monoxide, manganese, carbon disulfide, hydride, methanol, etc.).

3 drug-based (antipsychotic drugs such as phenothiazines, butyrylbenzenes, Rauvolfia alkaloids and -methyldopa, etc.).

4 cerebrovascular disease.

5 brain tumors (especially brain midline tumors).

6 brain trauma.

7 middle brain cavity.

8 Metabolic (hypothyroidism, basal ganglia calcification, chronic hepatic degeneration, etc.).

(3) Symptomatic Parkinson's syndrome (heterogeneous system degeneration):

1 progressive supranuclear palsy.

2 striatum substantia nigra degeneration.

3 cortical dentate nucleus degeneration.

4 olive pons cerebellar atrophy.

5Shy-Drager positional hypotension syndrome.

6 dementia [Guam Parkinson-Dementia-Amyotrophic lateral sclerosis syndrome, Jacob-Creutfeldt disease (cortical striatal spinal cord degeneration), Alzheimer and Pick disease, normal intracranial pressure hydrocephalus].

7 hereditary diseases (hepatolenticular degeneration, Hallerrorden-Spatz disease, Huntington's disease, spinal cord cerebellar degeneration, etc.).

diagnosis

1. Diagnosis basis

(1) Middle-aged and elderly patients have a slow onset of sexual disease.

(2) There are at least two items in the four main signs (stationary tremor, myotonia, bradykinesia, and posture gait disorder). The first two items have at least one of them, and the symptoms are asymmetrical.

(3) levodopa treatment is effective, levodopa test or apomorphine test positive support primary PD diagnosis.

(4) The patient has no extraocular muscle paralysis, cerebellar signs, orthostatic hypotension, cone system damage and muscle atrophy. The clinical diagnosis of PD and autopsy confirmed the coincidence rate of 75% to 80%.

2. Commonly used diagnostic and differential diagnostic criteria at home and abroad

(1) Diagnosis of primary Parkinson's disease (IPD): Wang Xinde wrote the criteria set by the National Extrapyramidal Conference in October 1984 as follows:

1 At least two of the following four typical symptoms and signs (stationary tremor, less motion, stiffness, positional reflex disorder) should be available.

2 Are there any atypical symptoms and signs that do not support the diagnosis of IPD, such as pyramidal tract signs, misuse gait disorders, cerebellar symptoms, intentional tremors, gaze palsy, severe autonomic dysfunction, obvious dementia with light Degree of extrapyramidal symptoms.

3 The reduction of high vanillic acid in cerebrospinal fluid is helpful for the diagnosis of early Parkinson's disease (PD) and essential tremor (ET), drug-induced Parkinson's syndrome and PD.

In general, ET is sometimes difficult to identify with early IPD, and ET is often characterized by hand and head positional and motor tremor without increased muscle tone and less movement.

(2) Diagnosis of secondary Parkinson's syndrome (SPDS):

1 Drug-induced PS (MPS): Drug-induced PS and IPD are difficult to distinguish clinically. It is important to rely on whether or not to take antipsychotic medication history. In addition, the symptoms of drug-induced PS are bilaterally symmetrical, and sometimes symptoms may appear first on the side of ADHD. If the clinical identification is difficult, the antipsychotic drug can be suspended. If it is drug-based, the symptoms of PS will generally disappear within weeks to 6 months.

2 vascular PS (VPS): The characteristics of this sign is more tremor-free, often accompanied by signs of focal nervous system (such as pyramidal tract sign, pseudobulbar paralysis, emotional instability, etc.), the course of the disease is mostly step-like progress L-dopa preparations are generally ineffective.

(3) Diagnosis of symptomatic Parkinson's disease syndrome (heterogeneous system degeneration):

1 progressive nuclear degeneration: sometimes difficult to identify with Parkinson's disease. The clinical features of progressive supranuclear palsy are mainly reduced movement, neck stiffness and later elevation of pseudobulbar paralysis and upward gaze paralysis.

2 olive pons cerebellar atrophy: primary Parkinson's disease should be identified with the disease. Olive pons cerebellar degeneration can also be manifested as less movement, rigidity, and even static tremor. However, there are many cerebellar symptoms such as ataxia. Significant changes can also be seen in the CT examination. Blood glutamate decarboxylase activity is reduced.

3 striatum substantia nigra degeneration: this disease and primary Parkinson's disease is very imaginary, clinically difficult to identify, mainly rely on pathological diagnosis. If clinical L-dopa treatment is ineffective, the possibility of striatum substantia nigra degeneration should be considered.

4Shy-Drager positional hypotension syndrome: clinical manifestations of positional hypotension, incontinence, no sweat, small muscle atrophy at the distal end of the limb. Sometimes it can be accompanied by Parkinson's disease syndrome. If patients with clinical manifestations of Parkinson's disease syndrome and mild autonomic dysfunction, they need to be differentiated from primary Parkinson's disease.

5 dementia: dementia with Parkinson's syndrome is not uncommon. A. Alzheimer's disease: In addition to dementia, advanced Alzheimer's disease has extrapyramidal symptoms such as less movement, rigidity and hyperactivity of the mouth. In addition, because Parkinson's disease can be accompanied by dementia in the early stage, it is necessary to rely on follow-up to identify the two; B. Normal intracranial pressure hydrocephalus: the disease manifests as gait disorder, urinary incontinence and dementia. Symptoms of Parkinson's disease, such as less movement, rigidity, and resting tremor, can sometimes occur. CT examinations are helpful for identification. Radionuclide cerebral angiography is also important for the diagnosis of normal intracranial pressure hydrocephalus.

6 genetic degenerative diseases:

A. Hallervorden-Spatz disease.

B. Huntinton chorea.

C. Lubag (X-connected muscle tension disorder - PDS).

D. Mitochondrial cell disease with striatal necrosis.

E. Neuroacupuncture (-lipoprotein deficiency).

F. Hepatolenticular degeneration (Wilson's disease).

Primary PD accounts for 75% to 80% of these clinical types; secondary (or symptomatic) PD is relatively rare; genetic degenerative disease and Parkinson's superposition syndrome account for 10% to 15%.

For most middle-aged and elderly patients with obvious slow motion, reduced muscle tone, and tremor, IPD is considered, and those with early or atypical symptoms are sometimes misdiagnosed. To this end, Takahashi et al. (1992) and Calne et al. (1992) proposed preliminary criteria for the early diagnosis of primary Parkinson's disease (IPD) and preliminary criteria for deletion criteria.

Diagnosis

Differential diagnosis

Idiopathic PD must be differentiated from familial PD and Parkinson syndrome. Early atypical cases must be differentiated from genetic disease or degenerative disease with Parkinson syndrome.

1. Familial PD accounts for about 10%, which is an incomplete penetrance autosomal dominant inheritance. It can detect -synuclein gene, Parkin gene mutation, susceptibility gene by Southern blotting, PCR and DNA sequence analysis. Analysis such as cytochrome P450-2D6 gene mutation and the like.

2. Parkinson syndrome has a clear cause, secondary to drugs, infections, poisoning, stroke and trauma.

(1) Parkinson syndrome after encephalitis: von Economo encephalitis, which is prevalent in the first half of the 20th century, often leaves Parkinson's syndrome and is currently rare.

(2) drug or toxic Parkinson syndrome: neuroleptics (phenothiazines and butyrylbenzene), reserpine, metoclopramide, -methyldopa, lithium, flunarizine, etc. can cause Jinsen syndrome; MPTP, manganese dust, CO, carbon disulfide poisoning or welding fumes can also be caused.

(3) arteriosclerotic Parkinson syndrome: multiple cerebral infarction leads to Parkinson syndrome, patients with hypertension, arteriosclerosis and stroke history, pseudobulbar paralysis, pathological signs and neuroimaging can provide evidence.

(4) Traumatic such as boxing encephalopathy, other such as hypothyroidism, hepatic degeneration, brain tumors and normal pressure hydrocephalus can cause Parkinson syndrome.

3. Genetic disease with Parkinson syndrome

(1) diffuse Lewis body disease (DLBD): more common in 60 to 80 years old, dementia, hallucinations, Parkinson's syndrome dyskinesia as a clinical feature, early dementia, rapid progress, may have myoclonus Levodopa reacts poorly, but the side effects are extremely sensitive.

(2) Hepatolenticular degeneration (Wilson's disease): can cause Parkinson's syndrome, adolescent onset, one or both upper extremity gross tremor, muscle rigidity, slow movement or involuntary movement, liver damage and corneal KF ring, serum copper , ceruloplasmin, copper oxidase activity decreased, urinary copper increased. (3) Huntington's disease: dyskinesia is mainly caused by muscle rigidity and exercise, and is easily misdiagnosed as PD.

4. Degenerative disease with Parkinson syndrome

(1) Multiple system atrophy (MSA): involving the basal ganglia, pons, olives, cerebellum, and autonomic nervous system, may have PD-like symptoms and is not sensitive to levodopa.

Including: striatum substantia nigra degeneration (SND), showing bradykinesia, muscle rigidity, can have cone system, cerebellum and autonomic symptoms, tremor is not obvious. Shy-Drager syndrome (SDS), prominent autonomic symptoms, orthostatic hypotension, no sweat, dysuria and impotence, as well as pyramidal tract, lower motor neurons and cerebellar signs. Olive pons cerebellar atrophy (OPCA), cerebellum and cone system symptoms, MRI shows cerebellum and brain stem atrophy.

(2) progressive supranuclear palsy (PSP): may have bradykinesia and muscle rigidity, early posture gait instability and fall, vertical gaze can not, with frontotemporal dementia, pseudobulbar paralysis, dysarthria and cone The bundle sign, the tremor is not obvious, and the levodopa reaction is poor.

(3) Cortical basal ganglia degeneration (CBGD): manifestations of muscle rigidity, bradykinesia, postural instability, dystonia and myoclonus, etc., may have cortical complex sensation loss, one limb neglect, disuse, aphasia and dementia, etc. Symptoms of cortical damage, eye movement disorders and pathology, levodopa treatment is ineffective. (4) Alzheimer's disease with Parkinson syndrome.

(5) Depression: There may be poor expression, monotonous speech, and reduced autonomic movement. PD patients often coexist. Depression without myotonia and tremor, antidepressant trial treatment may help identify.

(6) idiopathic tremor: more early onset, postural or motor tremor, affecting the head caused by nodding or shaking, PD typically affects the face, lips. The disease has no muscle rigidity and bradykinesia. About one-third of the patients have a family history, and the drinking or the dying of the heart is significantly reduced.

Clinical manifestation

2. There are obvious individual differences in the first symptoms of symptoms in the clinical period. There are reports of subjective sensory abnormalities of 85%, tremors of 70.5%, muscle stiffness or slow movement of 19.7%, dysfunction and/or writing disorder of 12.6%. The disorder was 11.5%, myalgia and pain were 8.2%, mental disorders such as depression and anxiety were 4.4%, language barrier was 3.8%, general malaise or muscle weakness was 2.7%, drooling and mask face were 1.6. %.

The following clinical trials have helped to find mild muscle rigidity:

1 to make the patient move the contralateral limb, the limb muscle strength of the tested limb can be more obvious;

2 head dropping test: The patient is in the supine position, and the head often falls slowly when the head pillow is quickly removed, rather than falling quickly;

(5) Other symptoms:

2 mouth, pharynx, diaphragmatic dyskinesia, slow speech, low monotonous voice, hooliganism, etc., severe dysphagia.

3.PD198410

(1)()

A.12

B.4

(2)()

()()

()

(-)

()

(3)()

Shy-Drager

[--Jacob-Creutfeldt()AlzheimerPick]

(Hallerrorden-SpatzHuntington)

diagnosis

1. Diagnosis basis

(1) Middle-aged and elderly patients have a slow onset of sexual disease.

(2)()2

(3) levodopa treatment is effective, levodopa test or apomorphine test positive support primary PD diagnosis.

(4)PD75%80%

(1)(IPD)198410

4()2

IPD

(PD)(ET)PD

ETIPDET

(2)(SPDS)

PS(MPS)PSIPDPS6PS

PS(VPS)()L-

(3)()

L-

Shy-Drager

A.AlzheimerAlzheimer;B.CT

A.-(Hallervorden-Spatz disease)

B.Huntinton

C.Lubag(X--PDS)

E.(-)

F.(Wilson)

PD75%80%;()PD;10%15%

IPDTakahashi(1992)Calne(1992)(IPD)

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