Spinal cavernous disease is a chronic, progressive lesion of the spinal cord. The cause is not well understood, but the lesion is characterized by the formation of tubular cavities in the spinal cord (mainly in the gray matter) and glial (non-neural) proliferation. It is often found in the cervical spinal cord. When the lesion involves the medulla oblongata, it is called medullary cavernous disease.
Etiology
The exact etiology is unclear, and can be divided into two categories: congenital abnormal development and secondary spinal cord cavitation, the latter being rare.
1, congenital spinal cord neural tube atresia insufficiency
This disease is often accompanied by other congenital anomalies such as spina bifida, cervical ribs, scoliosis, and circumoccipital malformation to support this view.
2.Spinal cord blood circulation abnormalities
It causes ischemia, necrosis, softening and cavity formation in the spinal cord.
3.Mechanical factors
The cerebrospinal fluid flow from the fourth ventricle to the subarachnoid space is blocked due to congenital factors, and the cerebrospinal fluid pulsation wave impacts downward on the central canal of the spinal cord, causing the central canal to expand and break through the wall of the central canal to form a cavity.
4.Other
Such as spinal cord tumor cystic degeneration, injury myelopathy, radiation myelopathy, spinal cord infarction softening intra-spinal cord hemorrhage, necrotizing myelitis, etc.
Clinical manifestations
The clinical manifestations of spinal cord cavitation are threefold, and the degree of symptoms is greatly related to the early and late development of the cavity, which generally progresses slowly. When the cavity is further enlarged, the gray matter in the medulla and the white matter outside the cavity are also involved, and the conduction bundle dysfunction appears below the cavity cavity.
1.Sensory symptoms
According to the cavity is located in the cervical and upper thoracic segments of the spinal cord, the cavity is on one side or in the center, there is unilateral segmental sensory disorder of the upper limbs and upper thoracic segments, often characterized by segmental dissociative sensory disorder, pain and temperature sensation is reduced or disappeared, deep sensation exists, the symptoms can also be bilateral.
2.Motor symptoms
The cervicothoracic cavity affects the anterior horn of the spinal cord, resulting in flaccid partial paralysis of one or both upper limbs, manifested by muscle weakness and decreased muscle tone, especially the interosseous muscle and interosseous muscle atrophy of both hands are the most obvious, and the severe cases present claw-shaped hand deformity;
If the lower trigeminal nerve root is affected, there is a central type of pain and warmth sensation disorder on the same side of the face, and the facial detached sensory loss forms the so-called “onion-like distribution”, accompanied by weak masticatory muscles. In advanced cases, the paresis is more aggravated.
3.Symptoms of autonomic nerve damage
The cavity involves the sympathetic spinal center of the lateral horn of the spinal cord (cervical 8 and thoracic 1), and Horner’s syndrome appears, and the lesion damages the corresponding segment, and the skin of the limb and trunk may have abnormal secretion;
Another bizarre sweating phenomenon is increased sweating when cold, accompanied by a decrease in temperature, hyperkeratosis of the fingertips and nails, atrophy, loss of luster, and a tendency to burns and trauma due to the loss of pain and temperature sensation. Recurrent urinary tract infections.
Examination
MRI: The cavity is low-signal, sagittal position appears in the longitudinal axis of the spinal cord, and the transverse section can clearly show the size and morphology of the cavity in the plane where it is located.
Diagnosis
According to the characteristics of chronic onset and clinical manifestations, there are segmental dissociative sensory disorders, lower motor neuron motor disorders occurring in the upper extremities and upper motor neuron motor disorders occurring in the lower extremities, etc. A clear diagnosis can mostly be made, and further definite diagnosis can be made by combining with the manifestations of imaging.
Treatment
1.General treatment
The use of neurotrophic drugs, radiation therapy has been tried in the past, but the efficacy is inaccurate. In view of the slowly progressive nature of the disease, as well as the frequent combination of crico-occipital malformation and subungual herniation malformation of the cerebellum, and these are considered to be related to the etiology, surgery should be adopted after a clear diagnosis.
The rationale for surgery is.
(1) To perform decompression of the craniocervical junction region, to deal with possible malformations and other pathological factors in this area, to eliminate the etiology and to prevent the development and deterioration of the lesion;
(2) To do cavity incision and shunt to reduce the size of the cavity and release the intrinsic compression factors to relieve the symptoms.
2.Other treatments
Including vitamin B, vasodilators, nerve cell metabolic function activators, etc., can be applied. Body therapy, physiotherapy and acupuncture therapy can also be used according to the condition to promote postoperative nerve function recovery.