Severe Unilateral Proprioceptive Loss in Medullary- Rostral Spinal Cord Infarction. A Posterior Spinal Artery Syndrome.

We draw attention to a unique presentation, severe unilateral loss of limb proprioception, in patients with medullary and rostral spinal cord infarction. Two patients developed acute severe proprioceptive loss in the limbs ipsilateral to infarcts that involved the caudal medulla and rostral spinal cord. They also had symptoms and signs often found in lateral medullary infarction. The proprioceptive loss is attributable to injury to the gracile and cuneate nuclei and/or their projections to the medial lemniscus. The infarct territory is supplied by the posterior spinal branches of the vertebral artery near its penetration into the posterior fossa. The presence of severe ipsilateral proprioceptive loss in a patient with features of lateral medullary infarction indicates involvement of the rostral spinal cord.

Cervical Posterior Spinal Artery Syndrome Caused By Spontaneous Vertebral Artery Dissection: Two Case Reports and Literature Review.

Herein, we described 2 patients with posterior spinal artery syndrome (PSAS) caused by vertebral artery dissection. The patients complained of sudden neck pain or walking instability. Neurological examination revealed sensory loss, muscle weakness, and sensory ataxia. Angiography showed double lumen sign or intimal flap in the vertebral artery. T2-weighted imaging and diffusion-weighted imaging of MRI showed a hyperintense lesion in the dorsal side of the cervical spinal cord at different times after onset. Both patients had good outcome after antiplatelet therapy and physiotherapy. A review of previously reported PSAS cases was also conducted in order to improve the understanding and awareness of this rare myelopathy.

New grading system for the clinical evaluation of patients with spinal vascular lesions.

PURPOSE: Neurointerventional approaches have improved myelopathy in patients with spinal vascular lesions by providing effective management, particularly when surgical approaches are difficult. However, there have been challenges in describing and comparing recovery status during the post-treatment period. METHODS: We evaluated 43 patients with venous congestive myelopathy (VCM) using Aminoff-Logue Disability Scale for gait (AL-G) and micturition (AL-M) scores. These results were compared with our new PSMS grading system that evaluates four categories (grades 0-3): pain, sensory symptoms, motor deficit, and sphincter change. Simple linear regression was used to identify the association or trend among the scales. We also calculated an overall area under the receiver operating characteristic curve to compare the predictive ability of the PSMS system with that of the previous grading system (AL-G and AL-M). RESULTS: Compared with other grading system, the PSMS system was more sensitively correlated with patient status and the results were easy to compare with previous clinical statuses during follow-up. The PSMS system also measured pain, which is commonly associated with spinal dural arteriovenous fistula and not precisely evaluated by other grading system. CONCLUSIONS: The new PSMS grading system for patients with VCM correlated well with the previously used systems and included pain evaluation. This new grading system is an easy tool for the evaluation and comparison of outcomes before and after endovascular treatment.

Cervical Posterior Spinal Artery Syndrome: A Case Report and Literature Review.

We report a case of left upper cervical posterior spinal artery (PSA) syndrome caused by atherosclerosis of the left vertebral artery. A 70-year-old female experienced sudden dizziness and paralysis of the left upper and lower limbs. Diffusion-weighted magnetic resonance imaging (DWI) of the brain showed high signal intensity at the vermis and lower left hemisphere of the cerebellum, and magnetic resonance angiography showed that the entire left vertebral artery was thin. The patient was treated with an intravenous infusion of tissue plasminogen activator 2 hours after symptom onset and made a full recovery. Repeat DWI, fluid-attenuated inversion recovery images, and T2-weighted images showed high signal intensity in the left upper cervical PSA area from the lower medulla oblongata to the C2 level in addition to the cerebellum. Previously reported cases of cervical posterior artery syndrome are reviewed.

Links among glaucoma, neurodegenerative, and vascular diseases of the central nervous system.

Glaucoma is the leading cause of irreversible blindness worldwide. Although the intraocular pressure (IOP) has been considered for long time the key point and the only treatable risk factor of the disease, there are cases in which glaucoma continues to progress despite normal IOP values. Vision loss in glaucoma is related to a selective decrease in the number of retinal ganglion cells by apoptosis that is associated to alterations of the central visual pathways. Interestingly, similar events have been also described in disorders of the central nervous system (CNS), such as Alzheimer's disease, Parkinson's disease, Leber's hereditary optic neuropathy, and cerebrovascular diseases. In this review, we discuss recent evidence supporting pathological links between glaucoma and disorders of the CNS.

A case of posterior spinal artery syndrome in the cervical cord: a review of the clinicoradiological literature.

We describe a patient with posterior spinal artery (PSA) syndrome due to vertebral artery (VA) dissection. A 63-year-old woman developed neck pain, bilateral shoulder and arm numbness, and paraparesis after prolonged neck extension during a dental procedure. Neurological examination revealed sensory deficits in the legs, paraparesis, cerebellar ataxia, urinary retention and constipation. Magnetic resonance imaging disclosed T2-hyperintense lesions in the posterolateral C4-C7 cord with partial enhancement. T1-hyperintensity and stenosis were found in the right VA at C3-C5. These clinicoradiological findings suggested bilateral PSA syndrome and unilateral VA dissection. This is the fourth report of VA dissection-induced PSA syndrome.

Complex arteriovenous fistulas at C1 causing hematomyelia through aneurysmal rupture of a feeder from the anterior spinal artery.

A 64-year-old woman presented with left occipital headache and right dissociated sensory loss due to hematomyelia on the left ventral side of C1 caused by rupture of an aneurysm on one of the feeders extending from the anterior spinal artery to complex epidural or dural and intradural arteriovenous fistulas (AVFs). Branches from the left occipital and ascending pharyngeal arteries and those from the left C2 radicular, left posterior spinal and anterior spinal arteries formed these multiple shunts, linking with a common venous drain flowing into the right petrosal vein. Surgical interception of all the shunts was achieved, making it unnecessary to directly treat the aneurysm in the spinal cord. The feeders, aneurysm and AVFs were not visualized on postoperative angiography, and the patient returned to a normal working life.

[Two cases of posterior spinal artery syndrome (PSAS)].

We have reported two patients with posterior spinal artery syndrome. Both of them had sudden onset back pain, paraparesis, loss of deep sensation and bladder-bowel disturbances. MRI disclosed spinal cord lesions positioned at its posterior part including the posterior column or posterior horn at thoracic levels. Spinal artery syndrome is a rare disorder, especially the posterior spinal artery syndrome (PSAS). In our department, only ten patients had spinal artery syndrome out of 2,064 patients admitted to our hospital these 20 years. All the other 8 patients had anterior spinal artery syndrome. It supports the notion that PSAS is rare. The detection rate of PSAS may increase after the routine use of spinal MRI in clinical practice. Our two patients had bilateral, symmetric symptoms. These symmetric signs and symptoms are usually seen in PSAS. The bilateral posterior spinal arteries connect with each other through many complex anastomoses. Moderate blood flow insufficiency may produce no clinical symptoms because of compensation by these anastomoses. When symptoms appear, these anastomoses do not compensate blood flow deficit and may produce bilateral symptoms.

The clinical features and surgical outcomes of patients with intramedullary spinal cord cavernous malformations.

BACKGROUND: Cavernous malformations (CMs) are not uncommon, but most of them are found to be located intracranially. Intramedullary CMs are rare, accounting for only 3-5% of identified total central nervous system lesions. The natural history of intramedullary CMs and their clinical features, including the risk of hemorrhage from a large series, still remains unclear and needs to be elucidated. We review our experience with surgically treated patients with intramedullary CMs and discuss the clinical features and surgical outcomes. METHODS: Between March 2004 and March 2010, a total of 21 patients with intramedullary spinal cord CMs were surgically treated in a single institution. Data from 21 patients were retrospectively analyzed. RESULTS: There were 13 females and 8 males ranging in age from 10 to 70 years (mean age 39.3 years). All patients harbored single symptomatic CM of the nervous system, and multiple lesions were not found. The annual retrospective hemorrhage rate was 2.18% per patient/year. All but one CM were completely resected, and the average follow-up period was 22.1 months (1-73 months). Ten of the 21 patients experienced an improvement in neurological state, 9 patients remained unchanged, and 2 patients experienced worsening of their conditions. CONCLUSIONS: Symptomatic intramedullary CMs should be surgically removed to avoid further neurological deterioration. Though there are some limitations due to the retrospective nature of this study and its small number of patients, the prognosis was found to be related to the preoperative neurological state and to the type of symptom presentation.

Intramedullary spinal cord hemorrhage (hematomyelia).

Intramedullary spinal cord hemorrhage (hematomyelia) is an uncommon cause of myelopathy and can present in an acute, subacute, stepwise, or chronic fashion. Spinal vascular malformations such as intramedullary cavernomas and intradural arteriovenous malformations are the most common cause of atraumatic intramedullary spinal cord hemorrhage based on the existing literature. Additional considerations include warfarin or heparin anticoagulation, hereditary or acquired bleeding disorders, primary spinal cord tumors, spinal cord metastases, Gowers' intrasyringal hemorrhage, or a delayed complication of spinal radiation. Prompt diagnosis of hematomyelia first requires recognition of a myelopathy syndrome (transverse, central, anterior, posterior, or hemi-cord) often accompanied by sudden, severe back or neck pain and sometimes radicular pain. MRI with and without gadolinium is the preferred imaging modality. There are no clinical trials to guide the management of acute intramedullary spinal cord hemorrhage, and subsequent treatment is usually directed toward the underlying cause.

Conus perimedullary arteriovenous fistula in a child: unusual angio-architectural features and pathophysiology.

INTRODUCTION: The perimedullary arteriovenous fistulas are located on the pial surface and are usually supplied by spinal medullary arteries, that is, either by the anterior or posterior spinal arteries, with no intervening nidus between the feeder arteries and the venous drainage. The clinical findings are, more commonly, caused by progressive radiculomedullary ischemic processes secondary to steal vascular mechanism. As the vascular supply to the spinal cord and to the arteriovenous fistulas (AVF) is not shared with one another, the vascular steal phenomenon cannot be implicated in this case's physiopathology. Most probably, the mass effect caused by the giant venous dilatation was the pathophysiological mechanism involved in this lesion. CASE REPORT: The authors describe the case of a 6-year-old girl with an intradural ventral arteriovenous fistula, with a giant venous dilatation, fed directly by L2 and L3 radiculomedullary arteries at the conus medullaris. There was no arterial supply to the fistula from the anterior or posterior spinal arteries. Selective spinal angiography showed an arteriovenous fistula supplied directly by two radiculomedullary arteries, with a large draining vein caudally. Interposing the arterial and venous vessels was a giant venous aneurysmal dilatation located ventral to the conus medullaris and extending from L3 to T6. The patient was successfully treated by a surgical approach through a laminotomy from L3 to T11. CONCLUSION: The type IV-C spinal arteriovenous malformations or perimedullary AVFs are rare lesions predominately described at the conus medullaris with various types of angio-architecture and controversial treatment.

Transient traumatic spinal venous hypertensive myelopathy.

We present a case of a reversible spinal venous hypertensive myelopathy that occurred following a traumatic mediastinal hematoma. The mediastinal hematoma caused compression of the brachiocephalic vein, resulting in elevation of the venous pressures that ultimately resulted in dilation of the epidural venous plexus and spinal cord edema. The secondary neurologic deficits were the culmination of venous outflow obstruction at the level of the spinal cord that resolved on the resolution of the mediastinal hematoma.

Spinal tanycytic ependymoma with hematomyelia--case report--.

A 58-year-old man presented with an extremely rare case of "pure type" spinal tanycytic ependymoma associated with hematomyelia manifesting as sensory disturbance of the bilateral hands and weakness of the right arm. Magnetic resonance imaging demonstrated a tumor in the spinal cord from C-2 to C-4 levels. The soft gelatinous tumor was subtotally resected and the adjacent chronic liquid hematoma was aspirated. The immunohistochemical and ultrastructural findings indicated a diagnosis of tanycytic ependymoma.

Direct spinal cord electrical stimulations during surgery of intramedullary tumoral and vascular lesions.

Despite the use of somatosensory evoked potentials during surgery for spinal cord tumors or vascular lesions, postoperative neurological disorders, particularly motor deficits, frequently occur after aggressive surgery with an attempt of gross total resection. We report the use of peroperative direct spinal cord electrical stimulation to decrease morbidity while improving the quality of resection. Three patients with intramedullary lesions (1 ependymoma and 2 cavernomas), initially revealed by pain and followed by neurological deficit, were operated at our institution using peroperative direct medullary electrical stimulations (60 Hz, biphasic square wave pulses with 1 ms/phase, 0.9 mA) under general anesthesia without curare. In all cases, gross total resection was performed until motor responses to stimulation, which indicated anterior and lateral boundaries between the lesion and the functional tissue, were obtained. There was no postoperative neurological worsening, but an immediate partial improvement of sensory and bladder disorders in the patient with ependymoma. Postoperative MRI confirmed total resection in the 3 patients. These cases demonstrate that direct medullary electrical stimulation is a safe, easy, precise and reliable method to reduce morbidity during spinal cord surgery.