striatal nigra degeneration
Introduction
Introduction to striatum substantia nigra Striatonic degeneration (SND) was first reported by Adams et al in 1961. In 1961 and 1964, the authors reported three patients with extrapyramidal damage, autonomic failure, and cerebellar ataxia. The symptoms of autonomic failure were milder than those of Shy-Drager syndrome. Pathological changes, but the symptoms of prenatal cerebellar ataxia were not severe in patients with OPCA. The disease belongs to the degenerative diseases of the nervous system and is classified into multiple system atrophy. basic knowledge The proportion of illness: 0.0035% Susceptible people: no special people Mode of infection: non-infectious Complications: urinary tract infection multiple lung infections
Cause
Striatum degeneration
(1) Causes of the disease
See Causes Multiple System Atrophy (MSA) for the cause.
(two) pathogenesis
1. SND and sporadic OPCA, SDS are classified in MSA, belonging to three different syndromes of MSA, the pathogenesis of which is referred to MSA.
2. Pathological changes: pathological changes are mainly manifested in the dense part of the substantia nigra and the lenticular nucleus associated with specific areas of the body. The substantia nigra neurons are widely lost, no Lewy bodies, no neurofibrillary tangles in the remaining cells, beans The nucleus degeneration was most obvious, the bilateral nucleus symmetry was severely atrophied and reduced, gray, while the caudate nucleus was less obvious, and the secondary globus pallidus was atrophy (mainly striatum globus fiber loss).
Microscopically, the basal ganglia showed loose nucleus, almost all of the neurons were lost, and a large number of extracellular brown coarse particles scattered widely. These particles were positive for iron staining, and the globus pallidus was mildly extensive. And sparsely distributed iron-positive particles, midbrain melanoid pigment neurons and cerebral leucocyst pigment neurons are severely lost, with mild stellate gliosis, and some manifest as extensive cerebral pons cerebellar degeneration, and, in the cortex-grain The cytoplasmic inclusion bodies of oligodendrocytes were observed in the corpuscle-pallidal cortex, which is associated with basal dysfunction.
Prevention
Striatum degeneration prevention
There is no effective prevention method, and symptomatic treatment is an important part of clinical medical care.
Complication
Striatum black matter degeneration complications Complications, urinary tract infection, multiple lung infections
As the disease progresses, various symptoms and signs can be manifested in the disease, and can also be regarded as complications of the disease (see clinical manifestations).
In addition, attention should be paid to secondary lung infections, urinary tract infections, etc.
Symptom
Striatum substantia nigra degeneration symptoms common symptoms dysphagia, dementia, tremor, muscle fiber tremor, difficulty in turning over ataxia, dysarthria
Generally in the pre-senile (25 to 68 years old, an average of 52 years old) onset, sporadic, insidious onset, slowly progressing, with Parkinson's syndrome as the first symptom, in this context, involving other parts of the central nervous system, patients gradually Reduced exercise, akinesia, rigidity, limb and trunk flexion, dull expression, abnormal posture and gait changes, dysarthria, difficulty swallowing and difficulty in turning over, about 2/3 patients can observe tremor during the course of the disease , but the tremor is not significant, and 75% to 100% of patients with extrapyramidal symptoms are asymmetrical, generally early onset, and have no response or minimal response to levodopa treatment, presumably due to dopamine receptors Due to the reduction of striatum neurons, Gonzalez et al. believe that it is associated with a significant reduction in dopamine D2 receptors in patients with SND, and also has a significant effect on levodopa. Such patients are more likely to be misdiagnosed as Parkinson's disease, but post-disease symptoms Progress is faster than Parkinson's disease.
On the basis of impaired extrapyramidal symptoms, cerebellar ataxia symptoms may occur, or symptoms of Parkinson's syndrome appear earlier, but more late and lighter, showing unstable balance and ataxia.
The symptoms of OPCA can also be combined. In addition, half of the patients have Shy-Drager syndrome. Many patients have urinary dysfunction, which may be due to degeneration of Onuf's nuclear neurons in the ankle, and may also have pyramidal tract signs. Due to striatum striatum dysfunction, some patients present with subcortical dementia and neuropsychological disorders, but dementia is not common. Some cases have Babinski sign, mild muscle atrophy, upper eye paralysis, and the course of disease is progressive. Usually 3 to 8 years, the average age of death is 57 years.
Examine
Examination of striatum substantia nigra
1. Upright experiment: The supine position, sitting position and upright blood pressure were measured separately. The systolic blood pressure decreased more than 30 mmHg within 2 to 3 minutes, the diastolic blood pressure decreased more than 20 mmHg, and the heart rate was unchanged.
2. Blood biochemical examination: Determination of plasma norepinephrine content, 24h urine catecholamine content determination was significantly reduced.
3. Cerebrospinal fluid examination: except for the individual reports of decreased acetylcholinesterase in cerebrospinal fluid, most patients have normal cerebrospinal fluid.
4. MR is the most valuable diagnostic method. The T2-weighted image shows the low density of the lenticular nucleus, the high signal loss normally present between the red nucleus and the substantia nigra, and shows an increase in iron deposition due to glial cells, glial fibrosis and Increased capillary volume increases the water content of the diseased tissue, prolonging the Tl and T2 values, showing a low signal in the T1-weighted image and a high signal in the T2-weighted image.
O'Brien reported a case of SND. MRI showed a decrease in the nucleus signal, especially in the posterolateral T2-weighted image, which was lower than the globus pallidus. In addition, the nucleus was significantly reduced, and the midbrain was reduced due to the substantia nigra and red nucleus. Small and unclear, the authors believe that the symptoms of SND seem to be related to the special structural function, shell atrophy is related to muscle rigidity and drug insensitivity, the severity of myotonia is closely related to the degree of reduction of shell signal, Dai Qilin reported 6 patients with SND MRI examination was abnormal. Two cases of bilateral external capsules showed symmetrical degeneration of white matter. Four cases had irregular T1W1 low signal and T2W1 high signal. One case was accompanied by brain stem and cerebellar atrophy, and 6 cases were CT examinations were normal.
5. PET studies have shown that striatum, frontal lobe, cerebellum and brainstem glucose metabolism disorders are caused by the loss of functional neuronal components.
Diagnosis
Diagnosis and differentiation of striatum substantia nigra
Diagnostic criteria
1. Parkinson's syndrome with sporadic adult latent onset is not effective for levodopa treatment.
2. Have obvious autonomic dysfunction syndrome.
3. There is a cerebellum sign.
4. A pyramidal sign appears.
Of the above 4 items, 1 is required, 2 and 3 are both available, and 4 is used as a reference.
Differential diagnosis
Clinically, it is sometimes difficult to identify with OPCA. Miwa reported that 55% of patients with SND have cerebellar symptoms, while 55% of patients with OPCA have Parkinson's syndrome, but the age of onset of SND patients (60.7 years ± 8.7 years) is higher than that of OPCA patients (55.4 years old). ± 7.9 years old), OPCA patients with cerebellar symptoms, and SND with Parkinson's syndrome symptoms, the first symptoms are mostly Parkinson's gait or tremor, no horizontal ocular dyskinesia, in addition, brain MRI performance is focused different.
