Heald's disease
Introduction
Introduction to Hilde's disease Hilde disease, also known as diffuse sclerosis, is a subacute or chronic extensive white matter demyelinating disease. Schilder (1912) first reported with diffuse periarthritis (encephalitis periaxialisdiffusa), hence Hilde disease. The term diffuse sclerosis was first used by Strumpell to describe the texture of isolated fresh brain in patients with alcoholism, and was later used for diffuse gliosis in any cause. In 1912, Schilder described a case of progressive debilitating, a 14-year-old girl who had an increase in intracranial pressure and died after 19 weeks. The autopsy revealed a large white matter of bilateral cerebral hemispheres, a clear myelin deprivation zone, and many small myelin detachment foci, similar to those of normal MS. basic knowledge Sickness ratio: 0.0001% Susceptible people: no special people Mode of infection: non-infectious Complications: multiple lung infections urinary tract infections acne
Cause
The cause of Hilde's disease
Cause:
The cause is not yet clear. According to the pathology and clinical findings, it is consistent with demyelinating diseases. It is considered that this disease is a variant of multiple sclerosis.
Pathogenesis:
Some reports suggest that the disease is a variant of MS that occurs in juvenile or adolescents. The pathological change is that a large number of demyelination occurs in the brain, which may be multifocal or a single large lesion. The characteristic lesion is a large, border. Clear, asymmetric myelin loss, lesions often involve the entire brain or one side of the cerebral hemisphere, typically affected by the corpus callosum to the contralateral side, sometimes bilateral hemisphere symmetry involved.
White matter lesions are often dominated by one occipital lobe, with distinct boundaries. The optic nerve, brainstem and spinal cord can also be found in lesions similar to MS. Fresh blood vessels can be seen in perivascular lymphocytic infiltration and macrophage response, and advanced glial cells are proliferative. Tissue necrosis and voids can also be seen.
Balo concentric sclerosis of Balo is rare. It is a demyelinating lesion of the white matter with specific pathological changes. The pathological features are demyelination and normal myelin preservation area, forming a neat concentric circle. It is like a tree ring, hence the name. Under the microscope, the inflammatory cell infiltration of lymphocytes mainly around the small vein can be seen. The lesion distribution and clinical features are similar to multiple sclerosis. It is generally considered that this disease is a variation of Schilder disease. Variant of MS.
Prevention
Hilde disease prevention
Prevention of infection, cold and cold or hot and other predisposing factors are the focus of prevention and treatment; prevention and treatment of complications is also an important part of clinical medical care.
Complication
Hilde disease complications Complications Multiple lung infections Urinary tract infections Acne
With the development of the disease, or the degree of disease, the symptoms and signs may be manifested by the disease itself, or as a complication (see clinical manifestations). In addition, secondary lung infections, urinary tract infections, hemorrhoids, etc. should be noted.
Symptom
Hilde's disease symptoms Common symptoms Dementia response Obtuse organic mental disorder Mental disorder Visual impairment edema Ataxia Dizziness Auditory weakness
1. Infants or adolescents, males, no familial, the course of the disease is similar to acute MS, mostly subacute, chronic progressive deterioration, pause or improvement is extremely rare, rarely relieved - recurrence, very few cases can be more The condition stopped working during the year, or the condition was relieved within a certain period of time.
2. The onset of the disease to the hospital for 10 to 40 days, mostly with mental disorders, can also have headaches, dizziness, fatigue and weakness before the emergence of mental symptoms.
3. The main manifestations are organic mental disorders and limb paralysis, normal or slightly elevated body temperature, patients are silent, indifferent, unresponsive, dazed, repeated speech and auditory hallucinations, urinary incontinence is more common, it is worth mentioning that Some patients have clear consciousness and urinary incontinence. Visual impairment can occur early in such as visual field defects, homonymous hemianopia and cortical blindness, as well as dementia or mental retardation, mental disorders, cortical spasm, varying degrees of hemiplegia or quadriplegia and pseudo. Medullary paralysis, etc., may also have seizures, ataxia, pyramidal tract signs, optic disc edema, ophthalmoplegia or internuclear ophthalmoplegia, nystagmus, facial paralysis, aphasia and urinary incontinence, etc. Pathological reflex on one side or both sides, and suction reflex, palmar reflex and so on.
4. Severe cases are de-cortical. Most patients die within months or years, but they also survive for 10 years or longer.
5. CSF changes are similar to chronic recurrent MS, but there is often no oligoclonal band. CSF often finds a large amount of basic myelin protein. EEG can be found in high-amplitude slow wave, and the former head area is obvious. CT scan sees white matter in the brain. Multiple scattered low-density stoves, the former head is obvious, MRI can find multiple abnormal signal areas of white matter in the brain, and can display concentric circular abnormal signals, which is valuable for clinical diagnosis. Brain biopsy is very reliable for the diagnosis of this disease.
Examine
Hilde's disease check
1. Cerebrospinal fluid examination: CSF changes are similar to chronic recurrent MS, but often there is no oligoclonal band, and CSF often finds a large amount of basic myelin protein.
2. Blood immunological examination: There is a differential diagnosis value.
3. EEG can see non-specific changes of high amplitude and slow wave predominance, and more common visual evoked potential (VEP) abnormalities, consistent with visual field and visual impairment, indicating optic nerve damage.
4. CT showed large white-like low-density areas of the white matter, mainly in the occipital, apical and temporal regions, involving one or both hemispheres, and more asymmetry. MRI showed diffuse lesions with low T1 signal and high T2 signal.
5. CSF-MNC is normal or slightly increased, protein is slightly increased, and oligoclonal bands are generally not present.
Diagnosis
Diagnosis of Hilde's disease
According to the medical history, the course of the disease and the characteristic clinical manifestations such as cortical blindness, mental retardation and mental disorders, combined with neuroimaging, CSF, EEG and other auxiliary examinations, can make a clinical diagnosis.
The clinical manifestations of patients are similar to acute demyelinating encephalopathy and viral encephalitis. Herpes simplex encephalitis is the most common in viral encephalitis. Although herpes simplex encephalitis is acute, psychiatric symptoms are common and similar to this disease. Where, but the body temperature is higher, various types of seizures are more common; cerebrospinal fluid has more cells, increased protein, and common red blood cells, CT or MRI can be seen in temporal lobe and / or frontal lobe lesions, and gray matter can be changed .
The disease is also easily confused with adrenal white matter malnutrition (ALD). ALD is sexually linked, involving only males, adrenal atrophy with peripheral nerve involvement and NCV abnormalities, and extremely long chain fatty acids (VLCFA).
The general classification of diffuse sclerosis also includes white matter dystrophic diseases such as Krabbe globular leukodystrophy and Greenfield metachromatic leukodystrophy. This group of diseases can affect peripheral nerves, and can also involve CNS tissues, white matter nutrition. Adverse clinical features are progressive vision loss, mental decline and spastic paralysis, pathology is large, more or less bilateral symmetry of cerebral hemisphere white matter destruction, each type of white matter dystrophy has a specific hereditary medulla Physiological defects in sphingolipid metabolism.
