Syringomyelia is a chronic and progressive disease of the spinal cord. Its pathological features are the formation of a tubular cavity in the spinal cord (mainly gray matter) and the proliferation of glial (non-neuronal cells). The tubular cavity expands from the inside to the outside over time, compressing and injuring the spinal cord, leading to weakness, numbness, loss of bladder and sphincter function, headache, loss of warm feeling and other clinical manifestations. Syringomyelia mainly includes communicative syringomyelia. Non-communicating syringomyelia, syringomyelia parenchyma, atrophic syringomyelia, and neoplastic syringomyelia have their characteristic pathogenesis and imaging manifestations. Among them, communicative syringomyelia refers to the enlargement of the central canal of the spinal cord caused by the obstruction of the outlet of the fourth ventricle and the cerebrospinal fluid circulation. Common pathogenic factors are meningitis and subarachnoid hemorrhage caused by hydrocephalus and hindbrain malformations such as Chiari II malformation, occipital meningocele, Dandy-Walker malformation and so on.
Is it acute myelitis or communicative syringomyelitis?
Here is a selection of a clinical case encountered by the author to talk about the diagnosis and treatment of communicative syringomyelia:
- Summary of medical records:
The patient, a male, 72 years old, a farmer, was admitted to the hospital because of “unstable walking, blurred vision for 20 days, and numbness and weakness of his limbs for 3 days”.
Twenty days ago, the patient had no obvious inducement to walk instability, widened stride, with unresponsiveness, urinary incontinence, blurred vision, no headache, nausea, or vomiting, which gradually aggravated and did not pay attention to it; three days ago, the patient developed limbs Numbness and weakness, unable to stand alone and holding objects, lying in bed, and going to our hospital, head CT showed dilation of the whole ventricle system, changes after interventional embolization of anterior communicating aneurysm, income from “numbness of limbs, weakness waiting for diagnosis, hydrocephalus” My department. One month ago, he was cured and discharged from the hospital due to “anterior communicating artery aneurysm, subarachnoid hemorrhage” underwent interventional embolization and lumbar drainage. At that time, the head CT showed that the ventricle was normal. During the course of the disease, the patient had a cough, a small amount of white sticky sputum, no hemoptysis, headache, no dizziness, convulsions, no diarrhea, no diet, and normal bowel movements.
He has previously denied high blood pressure, diabetes history, surgical trauma and blood transfusion history, food drug allergy history, hepatitis tuberculosis and other infectious diseases history, and special bad habits.
Admission examination: T36.2℃ P70 beats/minute R19 beats/minute BP110mmHg/70mmHg Shenqing, no yellowing of the skin and mucous membranes, no swelling of superficial lymph nodes. Both pupils are equal to 3 mm in diameter, reflect light, lips are not cyanotic, tonsils are not enlarged, neck is soft, trachea is in the middle, and thyroid is not enlarged. Breath sounds in both lungs were clear, no wet rales were heard, the heart rate was uniform, and no pathological murmurs were heard in the auscultation area of each valve. The abdomen is flat and soft, the liver and spleen are not under the ribs, and there is no edema in both lower limbs. The bilateral pathological signs were negative. Specialist examination: lack of fluency in speech. The pupils on both sides are erect and equi-circular, and are sensitive to light reflection. Forehead lines and nasolabial folds are symmetrical on both sides, and the tongue is centered. Muscle strength of both lower limbs was level 2 and both upper limbs were level 3. The tendon reflexes were active in the extremities, the bilateral Pap sign was positive, and the depth of sensation below C6 was absent. Urinary retention.
Auxiliary examination: head CT: dilation of the whole ventricle system, changes after interventional embolization of anterior communicating aneurysm. Cervical MRI showed: (1) Cervical 2-3, 3-4, 4-5, 5-6 intervertebral disc protruding backward or both sides, cervical 3-4, 4-5, 5-6 intervertebral disc horizontal spinal stenosis, The spinal cord is compressed and deformed. (2) Abnormal signals of the spinal cord at the level of the medulla oblongata-cervical 6 vertebral body. Consider exudation and edema. Please exclude myelitis in combination with clinical practice.
Preliminary diagnosis: (1) numbness and weakness of the extremities. Acute myelitis? (2) Communication hydrocephalus; (3) After aneurysm
- Diagnosis and treatment process:
The patient’s admission diagnosis considers the possibility of acute myelitis. He was given intravenous human immunoglobulin immunomodulation and methylprednisolone sodium succinate pulse therapy. The patient’s clinical symptoms did not improve significantly, and headache, nausea, and vomiting appeared gradually. Fundus examination showed bilateral papilledema. Considering the patient’s high intracranial pressure, he was transferred to brain surgery for lateral ventricle puncture and external drainage. The postoperative headache, nausea, vomiting and other symptoms of intracranial hypertension were quickly relieved. 4 days after the operation, the muscle strength of the lower limbs of the patient recovered to 4+, and the muscle strength of the upper limbs recovered to 5, and the head CT showed that the ventricle shape was better than before. The patient’s family refused to perform another neck MRI examination. Combined with the patient’s medical history, imaging findings and treatment effects, the revised diagnosis was made as: communicating hydrocephalus communicating syringomyelia; after interventional aneurysm.
The main manifestations of this patient were unstable walking, blurred vision, and numbness and weakness of the limbs. Combined with physical examination and analysis, the muscle strength of both lower limbs was level 2, the muscle strength of both upper limbs was level 3, and the muscle tension of the limbs was low. Active tendon reflexes in limbs. Positive bilateral Pap sign is located in the corticospinal tracts of the bilateral corticospinal tract; blurred vision in both eyes, can be located in the optic nerve, optic chiasm, optic tract, optic radiation, etc.; bilateral deep and shallow sensory loss below C6, consider bilateral spinal thalamus The fascicles, thin fascicles, and fascicles are affected; urine retention is located in the autonomic nervous system. The patient’s acute onset is mainly manifested by involvement of the motor system, sensory system, and autonomic nervous system, accompanied by a clear sensory plane, combined with the patient’s cervical MRI performance, so myelitis has become the first consideration for diagnosis. The patient has blurred vision in both eyes, except for neuromyelitis optica. Admission to the hospital to complete related auxiliary examinations, and to give intravenous human immunoglobulin immune conditioning and methylprednisolone sodium succinate therapy. The clinical symptoms did not improve significantly, and headache, nausea, and vomiting gradually appeared. The ophthalmological consultation found bilateral papilledema. Increased internal pressure, transferred to brain surgery for lateral ventricle puncture and external drainage. Postoperative headache, nausea, vomiting and other symptoms of intracranial hypertension were relieved quickly. 4 days after the operation, the muscle strength of both lower limbs recovered to grade 4+, and the muscle strength of both upper limbs recovered to grade 5. Reexamination of the head CT showed that the ventricle shape was better than before. Finally, lateral ventricle-intra-abdominal drainage was performed, and the prognosis was good.
Final diagnosis: communicating hydrocephalus communicating syringomyelia; after aneurysm intervention
Syringomyelia mainly affects the motor system, sensory system, and autonomic nervous system. Its clinical manifestations include: sensory symptoms, characterized by segmental dissociative sensory disturbances, pain, temperature sensation decreased or disappeared, and deep sensation exists; motor symptoms, upper limbs Lower motor neuron paralysis, upper motor neuron paralysis, dizziness, gait instability and nystagmus, etc.; autonomic nervous system, Horner syndrome, abnormal sweat secretion on the skin of the limbs and trunk, urinary disorders and recurrent urinary system Infection etc. The severity of symptoms is closely related to the speed and time of cavity development. Early symptoms can be relatively limited and mild, and late symptoms can be severe or even paraplegia. MRI is relatively specific for the diagnosis of syringomyelia. Typical MRI manifestations are: abnormal tubular signal in the center of the spinal cord, low signal on T1 (equal to the signal of cerebrospinal fluid in the adjacent subarachnoid space), high signal on T2, and most of the spinal cord in the hollow area. Thickened, the spinal cord is thin. On the cross-sectional image, most of the cavities are round or oblate, and a few with glial hyperplasia may have a double cavity shape.
The low incidence of traffic syringomyelia, the lack of vigilance of clinicians, and the lack of systematic understanding of the MRI manifestations of syringomyelitis and acute myelitis are the main reasons for misdiagnosis. (1) MRI manifestations of acute myelitis myelitis: ①The lesion is long, usually more than 5 vertebral body segments, and the upper boundary of the lesion is often higher than the clinical sensory level; ②The spinal cord is uniformly swollen and thickened; ③The lesion is fragmentary Long T1, long T2 signal, high signal in the lesion area T2, sagittal image can clearly show the lesion area. The cross-sectional T2 signal is uniform or uneven; ④The lesions on enhanced scan are generally not enhanced, or show patchy light enhancement. The patient had an acute onset, and the motor, sensory, and autonomic nervous systems were all involved with a clear sensory level. Myelitis seemed acceptable for the first diagnosis. (2) The clinical manifestations and onset forms of patients are different from most patients with syringomyelia. Syringomyelia is more chronic onset and progresses slowly. Acute disease with clear sensory level and paraplegia are relatively rare. The patient had an acute onset with paraplegia. Cervical spinal MRI showed that the central canal of the cervical spinal cord was dilated and exuded significantly. It is considered to be related to acute or subacute obstruction of the fourth ventricle, and belongs to a patient with relatively specific clinical manifestations of syringomyelia. (3) The patient was admitted without optic nerve and fundus examination, which became another reason for delayed diagnosis. During treatment, the patient’s lumbar puncture pressure was normal but the imaging showed hydrocephalus, and the diagnosis of normal intracranial hydrocephalus was made. As everyone knows, the patient had a history of SAH, subarachnoid adhesion, cerebrospinal fluid circulation path is not smooth, lumbar puncture pressure It does not reflect the true level of the patient’s intracranial pressure, which excludes the diagnosis of communicating hydrocephalus and communicating syringomyelia.
Therefore, for patients with hydrocephalus and abnormal intramedullary signals, especially patients with a history of intracranial infection or subarachnoid hemorrhage, it is necessary to be alert to the possibility of syringomyelia due to cerebrospinal fluid circulation disorders and include them in the differential diagnosis. Early detection, early diagnosis, reasonable treatment, and timely removal of spinal cord compression can significantly improve the prognosis of patients with communicative syringomyelia and reduce the occurrence of sequelae.