Acute disseminated cerebral cremasteric myelitis, also known as post-infection cerebral cremasteric myelitis and post-vaccination cerebral cremasteric myelitis, is a demyelinating disease within the central nervous system caused by immune dysfunction secondary to acute rash diseases such as measles, rubella, chickenpox, smallpox, or post-vaccination.
Disease name: Acute disseminated cerebral cremasteric myelitis
Primary cause: Acute disseminated cerebral myelitis is generally considered to be an immune-mediated demyelinating disease of the central nervous system.
Incidence probability: No authoritative and more comprehensive incidence statistics have been identified. Prior to widespread measles vaccination, neurological complications in measles patients ranged from 1 in 2000 to 1 in 800 cases; the morbidity and mortality rate with neurological complications was 10% to 20%; a similar number of patients were left with permanent neurological deficits. Cerebral cremasteric myelitis is less common after varicella and rubella, and even less common after mumps.
It can be divided into two types: post-vaccination cerebral cremasteric myelitis and post-infection cerebral cremasteric myelitis. In both types of patients, high cerebral crest fluid pressure, normal or mildly increased cerebral crest fluid leukocyte count and protein measurement, and diffuse wave activity in EEG tracings are common during the acute phase.
The majority of cases are in children and young adults, with acute onset 1-2 weeks after infection or vaccination, mostly disseminated, non-seasonal, and severe. The first symptom is encephalitis.
The first symptoms of encephalitis are headache, fever and confusion, and in severe cases, rapid coma and deactivation, epileptic seizures, headache, vomiting and meningeal irritation. The cremasteric type is commonly associated with partial or complete spastic paraplegia or quadriplegia, conduction bundle-type or lower extremity sensory disturbances, pathological signs, and urinary retention. Neurological signs of optic nerve, cerebral hemisphere, brainstem, or cerebellar involvement may be seen. Pain in the midline of the back may be a prominent symptom during the onset of the disease.
3. Acute necrotizing hemorrhagic cerebral cremasteritis, also known as acute hemorrhagic leukoencephalitis, is considered to be an ADEM fulminant form. The onset of the disease is acute, the condition is dangerous, and the mortality rate is high. The symptoms include high fever, progressive deepening of consciousness or coma, agitation, epileptic seizures, hemiparesis or tetraplegia; increased CSF pressure, increased cell count, diffuse slow activity of EEG, and irregular hypodense areas in the brain, brainstem and cerebellar white matter seen on CT.
As the disease progresses, the signs and symptoms that appear can be manifestations of the primary disease or can be seen as complications (see clinical manifestations). In addition, secondary pulmonary infections, urinary tract infections, and decubitus ulcers should be noted.
The disease is monophasic with signs and symptoms peaking in a few days associated with viral infections, especially measles or varicella virus. Cerebral cremasteric myelitis is also seen after rabies smallpox vaccination and has occasionally been reported after tetanus antitoxin injection. Many patients with cerebral cremasteric myelitis develop secondary to common whistling infections like EBV, cytomegalovirus, and mycoplasma pneumoniae virus infections.
The disease has a monophasic course, with signs and symptoms peaking in a few days, and is associated with viral infections, especially measles or varicella virus. Neurological dysfunction improves or partially improves after several weeks of onset. Immunization of animals with brain tissue and Freund’s complete adjuvant results in an experimental animal model of EAE with the same characteristic perivascular demyelination and inflammatory lesions as human MS, presumably a T cell-mediated immune response, suggesting that ADEM is acute MS or a variant thereof.
Pathological manifestations include small and moderate perivenous demyelinating lesions scattered throughout the brain and cristae, ranging from 0.1 mm to several mm (when fused), with microglia visible in the demyelinated areas with an inflammatory response and lymphocytes forming vascular cuffs. Multifocal meningeal infiltrates were common, and the extent was mostly not severe.
Immunization of animals with brain tissue and Freund’s complete adjuvant can result in an experimental animal model of EAE with the same characteristic perivascular demyelination and inflammatory lesions as human MS, presumably as a T-cell-mediated immune response to ADE as acute MS or its variants.
The pathology is characterized by extensive scattering of numerous foci of demyelination in the brain and crestal medulla, and in some cases the foci are limited to the cerebellum and crestal medulla These foci range from 0.1 mm to several millimeters and are located around the small middle vein. The intracerebral lesions were multiple and bilaterally symmetrical, with a tendency to fuse with a predominantly semi-oval central involvement, spreading to the frontoparietal and occipital lobes as well as the insula, optic nerve, optic cross and brainstem; severe demyelination and necrosis of the white matter of the crestal medulla, involving the cervical and thoracic segments and the lumbar segment; the lesions were equally old and new, which differed from multiple sclerosis in that axons and neuronal cells were largely preserved, and in severe lesions there was also slight destruction of axons with marked inflammatory cell infiltration The inflammatory exudation of small perivascular veins is a cellular reaction consisting of polymorphic microglia in the corresponding areas of myelin loss; perivascular sets consisting of lymphocytes and monocytes are seen; multifocal meningeal exudation is another necessary feature, but is generally not severe.
1. Increased peripheral blood leukocytes and accelerated hematocrit. Brain crest fluid pressure is increased or normal, CSF-MNC is increased, and protein is mildly to moderately increased, with IgG predominantly increased, and oligoclonal bands may be found.
EEG shows common theta and σ waves, and spike and slow spike complex waves.
3.CT shows diffuse multifocal large or patchy hypointense areas in the white matter, with obvious enhancement effect in the acute phase.
MRI shows scattered multifocal T1 low signal and T2 high signal lesions in the white matter of brain and crestal medulla.
1, peripheral blood leukocytosis blood sedimentation is accelerated.
2. Increased or normal cerebral crest fluid pressure, increased CSF-MNC, mild to moderate increase in protein, predominantly increased IgG, and oligoclonal bands may be found.
The clinical diagnosis can be made on the basis of diffuse brain parenchymal damage of acute onset after infection or vaccination with increased meningeal involvement and crestal medullary inflammatory symptoms CSF-MNC, extensive to moderate abnormalities in EEG, and CT or MRI showing multiple scattered lesions in the brain and crestal medulla.
The disease needs to be differentiated from encephalitis B and herpes simplex virus encephalitis. Encephalitis B has a distinct epidemic season, while ADE is sporadic; encephalitis and cremasteric myelitis occurring simultaneously can be differentiated from viral encephalitis as follows.
1. Herpes simplex virus encephalitis. It can be disseminated, with recurrent orofacial herpes seen before or during the onset of the disease, and other prodromal symptoms are not obvious. The most prominent psychiatric symptoms are hyperthermia and convulsions and high cranial pressure, which can quickly lead to coma. These are the differences from acute disseminated cerebral cremasteric myelitis.
2. Epidemic B encephalitis.
Seasonal onset, predominantly from July to September, with insect-borne transmission. The acute onset of the disease is characterized by high fever, headache and convulsions, and high cranial pressure, and can involve multiple parts of the brain, cerebellum, brainstem, and cremaster. The symptoms of systemic toxicity may be manifested, and the peripheral blood leukocytes are increased, with neutrophils predominating in the brain crest fluid. MRI is symmetrical bilateral thalamic and basal nucleus lesions.
3. Acute hemorrhagic white matter encephalopathy.
Most scholars believe that it is a fulminant type of acute disseminated cerebral cremasteric myelitis. The onset of the disease is rapid and dangerous, and the death rate is extremely high, mostly within a few days after the onset of the disease. Symptoms of cremaster involvement are less common than or masked by cerebral symptoms. Peripheral blood and cerebral crest fluid may show a marked increase in white blood cells, with predominantly neutrophils as a reflection of an abnormally active immune system. Hemorrhagic foci may be seen on imaging within or around softening and necrotic foci, which are also diffuse in appearance, mostly in a patchy distribution. Cases of progressive progression from ADE to acute hemorrhagic leukoencephalitis have been reported by MRI. The possible causes were described in Hurst’s earliest pathological reports as small venous and peripapillary erythrocyte exudates, vessel wall necrosis, polymorphonuclear leukocyte infiltration, and glial cell reactions. It is possible that in more severe cases, the demyelination was accompanied by damage around the tiny vessels, vascular stroma edema, and gradual fusion of the lesions to form larger lesions, leading to the development of hemorrhage. Magnetic resonance findings demonstrate acute hemorrhagic leukoencephalitis as acute dissemination.
4. Multiple sclerosis.
Acute disseminated is the main difference between this disease and MS MS is theoretically a scattered, multiple foci rather than diffuse and occurs multiple times with a relapsing-remitting course. Some patients with MS do have an acute onset and lack of relapsing-remitting features, and have a relatively short course, showing a monochromatic course. This type of patient is difficult to distinguish from ADE in terms of pathogenesis and pathology as well as pathophysiology, and some scholars consider it a transitional type. From the perspective of clinical practice, demyelinating disease is the common denominator, and for patients with a relatively rapid onset and progressive course, timely and specific therapeutic measures to save endangered tissues are of utmost importance.
The differential diagnosis of ADE and MS is very important and difficult for the prognosis. In terms of first presentation, ADE often presents with a more diffuse CNS disorder with coma, lethargy, convulsions and multifocal damage involving the brain, cremaster and optic nerve. In contrast, MS often presents with a single symptom, either optic nerve damage or subacute cremasteropathy. In ADE, the optic nerve damage is usually bilateral and the cremaster lesion is often complete, whereas in MS, the optic nerve damage is often unilateral and the cremaster lesion is often incomplete, whereas in MS, the onset of ADE is often preceded by infection or vaccination, whereas MS does not always have such antecedents, but these factors cause MS symptoms to recur and therefore are not absolutely different. Although oligoclonal bands are a feature of MS, they are also present in ADE. However, the oligoclonal bands in MS can be more persistent, and follow-up observations can be useful to differentiate between the two. Therefore, a single episode of ADE is not effectively distinguished from MS by clinical symptoms and crestal fluid indicators alone. Typical ADE is a relatively symmetrical lesion with extensive involvement of the cerebral and cerebellar white matter and has also been reported with involvement of the basal nucleus, the latter being extremely rare in MS where lesions are mostly asymmetrical and vary in size and newness. ADE differs from MS in that it is monophasic, so multiple MRI examinations during the prognosis and sequelae can help in the differential diagnosis. According to Poser’s criteria, the reappearance of symptoms in MS more than 1 month apart is considered a relapse, and thus in demyelinating disease, clinical and MRI follow-up at least every 6 months for 2 years is preferable.
Extensive invasion in the CNS such as multiple metastases and hematologic tumors, as well as rare diseases such as acute encephalopathy associated with vitamin deficiencies should also be taken into account in considering neurological diseases while not neglecting the possible effects of medical conditions.
High-dose corticosteroids are commonly used in the acute phase of treatment, but have little benefit. Small sample studies have found immunoglobulin intravenous drips or plasma exchange to be effective.
Examination.
1, Laboratory tests: brain crest fluid examination.
2.Brain biopsy.
3, CT and MRI of the head.
Treatment.
1.Intravenous or drip injection of adequate amount of steroid hormone drugs in the acute stage, also can be combined with the application of azathioprine to control the development of the disease as soon as possible.
2, symptomatic treatment.
In the recovery period, cerebrofacial, cytarabine and vitamin B drugs can be used.
Acute disseminated cerebral cremasteric myelitis mostly starts acutely 4-14 days after viral infection or vaccination, and should be considered in most patients with headache, vomiting, confusion, convulsions, and limb paralysis after fever or vaccination. The onset of this disease is more dangerous and should be seen at the earliest possible time at a hospital in a position to do so. Depending on the severity of the disease and the cause, the effect of treatment is not consistent, the majority of patients have a considerable degree of recovery after treatment, some patients may have motor or intellectual impairment. The mortality rate of the disease is about 10-30%.