progressive supranuclear palsy
Introduction
Introduction to progressive supranuclear palsy Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disease characterized by cerebral bridge and midbrain neuron degeneration and neurofibrillary tangles (NFT) as the main pathological changes. The dystonia posture and ocular dyskinesia, also known as ocular dystonia (oculocervicaldystonia). Posey (1904) first reported that in 1963 Richardson and Steele and Olszewski used progressive supranuclear palsy as an independent disease of clinical pathology. In 1972, Steele detailed the clinicopathological features of the disease, also known as Steele-Richardson-Olszewski synthesis. At the time, there were 73 cases of PSP cases in the medical literature, including 22 cases of autopsy, and there were several cases in almost every major neurological center. Therefore, PSP is not uncommon. The main clinical features of this disease are posture instability, dyskinesia, vertical supranuclear palsy, pseudobulbar palsy and mild dementia. basic knowledge The proportion of illness: 0.003% Susceptible people: no special people Mode of infection: non-infectious Complications: bulbar paralysis pneumonia
Cause
Progressive supranuclear palsy
(1) Causes of the disease
The cause of PSP is unknown. Although there are few familial clues, there is no genetic evidence. The disease has similar narcolgic encephalitis-like neurofibrillary tangles in the brainstem. Some people suspect that it is related to lentivirus infection, but it is currently poisoned. There are no clues about encephalitis, ethnicity and geographic factors.
(two) pathogenesis
At PSP, the striatum reduced 18F-dopa uptake, D2R density decreased, dopamine (DA) and high vanillic acid (HVA) levels decreased; cholinergic neurons were also involved, choline acetyltransferase activity decreased, frontal lobe , striatum, thalamus, cerebellar glucose metabolism or glucose utilization and oxygen metabolism were significantly reduced, with the most prominent frontal lobe, a small number of patients can show diffuse glucose metabolism, but the frontal and striatum are more obvious, with PD When the striatum metabolism is normal or increased, it may help to distinguish between the two.
Pathological features: extensive brain atrophy can be seen by the naked eye, including globus pallidus, substantia nigra, etc., the lateral ventricle and the third ventricle are enlarged, the substantia nigra can be seen under the microscope, the globus striatum-striatum pathway, the quadrangular upper mound, and the white matter around the aqueduct is obvious. Pathological changes, dense NFT is characteristically distributed, nerve fiber mesh formation, the latter is embedded in the filamentous structure of the nerve fiber network, independent of NFT alone, suggesting that PSP is a diffuse disease originating from the cytoskeleton, in addition, Tau-positive astrocytes are also found in the basal ganglia and brainstem. Other non-specific pathological changes include neuronal loss and gliosis, and the brain and cerebellar cortex are not affected.
Prevention
Progressive supranuclear palsy prevention
There are no good preventive measures, and some measures should be taken to prevent patients from falling; those with early dysphagia should be given a soft or mushy diet, and patients with advanced disease should have a nasogastric tube to prevent aspiration pneumonia.
Complication
Progressive supranuclear palsy complications Complications, medullary pneumonia
Common complications include: arrhythmia caused by pseudobulbaric paralysis, cognitive decline, decreased emotional activity, infection, and falls.
Symptom
Progressive supranuclear palsy symptoms Common symptoms Fatigue easy to fall, lethargy, articulation, gait, instability, dementia, old man, slow movement, pharyngeal reflex, illusion, mental retardation
1. The patient is mostly in the range of 45 to 75 (average 50) years old, with a course of 6 to 10 years; the onset of insidious disease, the course of the disease is slow and continuous progress, the male is slightly more, the common initial symptoms are fatigue, lethargy, falling for no reason (often toward After the fall, etc., the symptoms are symmetry about 81%, early dyskinesia manifests gait instability and balance disorder, about 63% of the first symptoms of the case is gait instability, walking is a big gait, both knees are straight and stiff In the shape of the body, the lower limbs cross and fall easily. Due to eye-vestibular dysfunction, torso stiffness and less movement, this gait is different from the small gait, rapid gait and turning difficulty of Parkinson syndrome patients.
2. Eye movement disorder is a characteristic manifestation of this disease. Both eyes gaze upwards and downwards. Generally, they start from the follow-up of the two eyes. The main complaint is that they cannot see the difficulty of walking on the toes, or they can not see the food on the table. Difficulty in eating, progressive loss and upper vision function become complete vertical gaze, the eyeball is fixed in the median position, patients with more than 2/3 in the late stage may have bilateral paralysis of the eyes, and 1/3 of the patients have internuclear ophthalmoplegia. In some patients, the two eyes could not be converged, the pupils were reduced, the light reflection and the radiation reflection existed. There were head-eye reflexes and Bell phenomenon indicating nuclear, and the late-head reflexes disappeared into nuclear lesions.
3. Common unclear articulation, difficulty in swallowing, hyperreflexia, hypertrophy of the pharyngeal muscles, emotional instability, and other symptoms of pseudobulbaric paralysis, can cause aspiration pneumonia, hyperreflexia, Babinski sign and other cone bundle damage Symptoms, emotional disorders, a small number of patients due to rigidity, less movement and high facial muscle tension caused facial wrinkles, showing amazed face.
4. Cognitive and behavioral disorders appear later, about 52% of patients appear in the first year of the disease, showing cognitive decline, decreased emotional activity, poor dementia and spatial orientationmemory test, etc., about 8 % of patients have this as the first symptom, which may be speech vague, difficult to pronounce, slow or accelerated speech rate, repeated speech or imitative speech and ataxia speech, etc. The frontal lobe symptoms show fluency in speech and decline in image thinking ability. Speech imitation or retelling difficulties, personality changes, etc.
Examine
Progressive supranuclear palsy
Cerebrospinal fluid examination revealed an increase in CSF protein content in approximately one-third of patients.
1. About 1/2 of patients have non-specific diffuse abnormalities in EEG.
2. Head CT examination showed brain atrophy, MRI examination showed atrophy of the midbrain, with posterior enlargement of the third ventricle and atrophy of the anterior temporal lobe; some patients on T2WI could show low signal of the nucleus.
Diagnosis
Diagnosis and identification of progressive supranuclear palsy
Diagnostic criteria
The diagnosis of this disease is more difficult. The clinical diagnosis of PSP mainly depends on clinical manifestations. The middle-aged and elderly patients have insidious onset, clinically intelligent disorder, nuclear gaze paralysis, gait instability, rigidity, easy to fall, less need to consider PSP possibility.
In 1996, the National Institute of Neurological Disorders and Stroke (NINDS) and the Progressive Nuclear Paralysis Society (SPSP) jointly recommended the PSP diagnostic criteria, which were divided into suspicious PSP, proposed PSP and confirmed PSP. NINDS - The exclusion criteria in the SPSP diagnostic criteria are important to improve specificity.
Suspicious PSP
(1) Prerequisites:
After the age of 140, the disease progresses gradually;
2 vertical upward or downward nuclear gaze paralysis or obvious posture instability with repeated falls;
3 The above clinical manifestations cannot be explained by the diseases listed in the exclusion conditions.
(2) Auxiliary conditions:
1 symmetry movement can not be stiff or straight, the proximal end is heavier than the distal end;
2 abnormal neck position, especially the neck back;
3PDS has poor or no response to levodopa;
4 early dysphagia and dysarthria;
5 Early cognitive impairment such as apathy, weak abstract thinking ability, lack of speech, application or imitation behavior, frontal lobe release symptoms, and at least two of the above symptoms.
(3) Exclusion conditions:
1 recent history of encephalitis, or limb syndrome, cortical sensory defect, localized frontal lobe or temporal lobe atrophy;
2 hallucinations and delusions unrelated to dopaminergic drugs, AD type cortical dementia;
3 early manifestations of cerebellar dysfunction or unexplained autonomic dysfunction;
4 severe asymmetry PDS such as slow movement;
5 neurological radiology of brain structural damage (such as basal ganglia or brain stem infarction, cerebral atrophy); 6 if necessary, polymerase chain reaction (PCR) can be used to exclude Whipple disease.
2. Proposed diagnosis of PSP
(1) Prerequisites:
After 140 years of age;
2 the course of disease gradually progresses;
3 vertical upward or downward nuclear gaze palsy, the first year of the disease appeared obvious posture instability with repeated falls;
4 The above clinical manifestations cannot be explained by the diseases listed in the exclusion conditions.
(2) Auxiliary conditions and exclusion conditions: the same diagnostic criteria as the suspected PSP.
3. The diagnosis of PSP must be confirmed by histopathological examination.
Differential diagnosis
Clinically, PSP should be noted with PD, post-encephalitis or arteriosclerotic pseudo-Parkinson's syndrome, cortical basal ganglia degeneration (CBGD), (MS multisystem atrophy A), diffuse Lewy body disease (DLBD), Identification of Creutzfeldt-Jacob disease (CJD).
