1. Regulation of motor functions by the basal ganglia - cerebral cortex → basal ganglia → thalamus → cerebral cortex circuit

2. Abnormality

Basic lesions

biochemical changes

It mostly occurs in middle-aged and elderly people over 50 years old; the onset is insidious and slow, and gradually worsens

motor symptoms

non-motor symptoms

Biochemistry/Imaging

Step Ⅰ (required): Move less, choose 1 from 3 (resting tremor, muscle rigidity, unstable posture)

Step II (Exclusion): Such as a history of repeated strokes, repeated brain injuries, etc. (see the textbook for details)

Step III (supporting evidence): Choose 3 from 8 (unilateral onset, resting tremor, gradual development, mostly persistent asymmetric involvement after onset, very good response to levodopa treatment, severe levodopa-induced dyskinesia, the therapeutic effect of levodopa lasts for more than 5 years, and the clinical course is more than 10 years)

1. Secondary Parkinson's syndrome: caused by infection, drugs, poisons (such as MPTP), vascularity, trauma (such as boxing encephalopathy); characteristics: ① often characterized by rigidity and lack of movement; ② resting tremor is rare Rare; ③ Insensitive to levodopa treatment

2. Essential tremor: Except for obvious tremor (positional tremor), there are no other main symptoms of PD; there is often a family history

3. Progressive supranuclear palsy: Parkinson's syndrome, vertical eye movement disorder, and early balance disorder, but the efficacy of dopa preparations is poor, the clinical symptoms are symmetrical, the trunk posture is extended, and there is usually no tremor, movement fluctuations, and abnormal movements.

4. Multiple system atrophy: Parkinson's syndrome, but with autonomic nervous system dysfunction, pyramidal tract signs, and cerebellar signs. The disease progresses rapidly and has poor efficacy on dopa preparations.

5. Diffuse Lewy body dementia: Parkinson's syndrome, but accompanied by rapidly progressing dementia and hallucinations, poor response to dopa preparations

Treatment principles

medical treatement

Other treatments

[Cause and pathogenesis] AD; the causative gene is IT15, and the expression product is Huntingtin (containing CAG repeat sequence copies, the more copies, the earlier the onset and the more severe the symptoms)

[Pathology] Mainly located in the striatum and cerebral cortex; manifested as GABA/ACh↓, dopamine↑, endorphin/substance P↓, somatostatin/neuropeptide Y↑ in striatal efferent neurons

[Clinical manifestations] Most common in 30 to 50 years old; often have a family history; early onset of disease (early onset phenomenon) occurs in consecutive offspring; the main manifestation is choreiform involuntary movements; as the disease progresses, choreiform involuntary movements may gradually Reduced, while parkinsonian syndromes such as dystonia, bradykinesia, myotonia, and postural instability gradually become more apparent.

[Auxiliary examination] Genetic testing (CAG repeat sequence>36)

[Definition] It is a liver cirrhosis caused by an inherited copper metabolism disorder and a degenerative brain disease mainly involving the basal ganglia (especially the putamen); clinical manifestations include progressively aggravated extrapyramidal symptoms, psychiatric symptoms, and liver disease. Sclerosis, renal impairment, K-F ring

[Pathogenesis] An AR hereditary disease. Due to abnormalities in the P-type ATP7B gene, Cu2 cannot be delivered to ceruloplasmin and excess copper is excreted through bile and accumulated in the body.

[Clinical manifestations]

[Auxiliary examination] Serum ceruloplasmin <200 mg/L (but its value has nothing to do with the condition, course of disease, and copper-removing effect) ①, copper oxidase absorbance <0.17, 24-hour urine copper (can be used as a reference for adjusting the dosage of clinical copper-removing drugs) indicators), liver copper level (gold standard for diagnosis), liver and kidney function, imaging examination, isolated skin fibroblast culture, genetic testing

[Treatment] Promote copper excretion (penicillamine is preferred ②), reduce copper absorption (zinc sulfate), low-copper diet, traditional Chinese medicine treatment, symptomatic treatment, surgical treatment (splenectomy, liver transplantation)

[Cause] Related to rheumatic fever

[Clinical manifestations] More common in girls aged 5 to 15 years old; often have upper sense before onset; main manifestations are chorea, hypotonia (pronator sign, milkmaid's maneuver/profit and loss sign), and may have psychiatric symptoms

[Treatment] Symptomatic treatment (chorea symptoms → DA receptor antagonists (such as chlorpromazine, haloperidol), DA depleting agents (such as reserpine)), etiological treatment (penicillin, glucocorticoids), immunotherapy

[Definition] It is a group of symptoms caused by the incoordination of the actuators and antagonists of the body's skeletal muscles, and the intermittent and sustained contraction of the body's skeletal muscles, resulting in repeated involuntary movements and abnormal twisting postures.

[Clinical manifestations]

[Treatment] ① Treatment of the cause; ② Focal dystonia: Except for spastic torticollis, which requires surgery when necessary, botulinum toxin type A injection is the first choice for the rest, supplemented by drug treatment; ③ Segmental and generalized dystonia : Mainly drug treatment, such as carbamazepine, clonazepam, trihexyphenidyl; ④Dopa-responsive dystonia: Madopa can be used