Severe Mimic of Transverse Myelitis - Spontaneous Non-Traumatic Intramedullary Hematomyelia: A Case Report.

Spinal cord hemorrhage remains an uncommon vascular pathology with no standard approach to treatment. Intramedullary hematomyelia is the rarest subtype and has only been described in case reports, making it a diagnosis easier to miss. A 55-year-old male with history of polysubstance abuse presented for abrupt onset non-traumatic back pain that progressed over several days evolving into paraplegia, sensory loss up to the T3 dermatome, and incontinence of bowel and bladder. His MRI imaging of the cervical and thoracic spine was concerning for a T1 and T2 hyperintense lesion extending from the internal medulla to the upper thoracic spine causing edema. The initial differential diagnosis, based on history and progressive neurologic decline, included a longitudinally extensive transverse mellitus of unclear etiology. He received high dose steroids and plasma exchange without any clinical improvement. An extensive autoimmune, inflammatory, infectious, and demyelinating workup via serum and CSF analysis was unremarkable. He was transferred to a tertiary facility, where repeat MRI spinal imaging included GRE sequences that was significant for increased susceptibility. MRA imaging of the spine was completed showing no vascular malformation. Overall, the diagnosis of hematomyelia was felt to be most likely. Despite aggressive inpatient rehabilitation, the patient remains paraplegic and requires long term management consistent with severe spinal cord injury. This case highlights the importance of recognizing vascular syndromes as a cause of acute myelopathy, understanding the imaging findings of blood breakdown products on MRI, and the desperate need for successful therapeutic strategies to combat a potentially devastating neurologic process.

Case report: Traumatic hemorrhagic cervical myelopathy in a dog.

A 1.5-year-old female entire French bulldog was referred for neurological evaluation, further diagnostic tests, and treatment 24 h after a road traffic accident. Initial emergency treatment, diagnostic tests, and stabilization had been performed by the referring veterinarian. Neurological examination revealed severe spastic non-ambulatory tetraparesis and was consistent with a C1-5 myelopathy. A magnetic resonance imaging (MRI) study revealed an irregular to elongated ovoid intramedullary lesion centered over the body of C2. The lesion showed marked signal heterogeneity with a central T2W and T2* hyperintense region, surrounded by a hypointense rim on both sequences. The lesion appeared heterogeneously T1W hypointense. The lesion was asymmetric (right-sided), affecting both white and gray matter. The C2-3 intervertebral disk appeared moderately degenerate with a Pfirrmann grade of 3. No evidence of vertebral fracture or luxation was found on radiographs or MRI of the vertebral column. Additional soft tissue abnormalities in the area of the right brachial plexus were suggestive of brachial plexus and muscle injury. A diagnosis of traumatic hemorrhagic myelopathy at the level of C2 and concurrent brachial plexus injury was formed. Conservative treatment was elected and consisted of physiotherapy, bladder care with an indwelling urinary catheter, repeated IV methadone based on pain scoring (0.2 mg/kg), oral meloxicam 0.1 mg/kg q24h, and oral gabapentin 10 mg/kg q8h. The dog was discharged after 4 days, with an indwelling urinary catheter and oral medication as described. The catheter was replaced two times by the referring veterinarian and finally removed after 10 days. Thereafter, voluntary urination was seen. During the 2 months after the road traffic accident, slow recovery of motor function was seen. The right thoracic limb recovery progressed more slowly than the left limb, also showing some lower motor neuron signs during follow-up. This was judged to be consistent with a right-sided brachial plexus injury. The dog was reported ambulatory with mild residual ataxia and residual monoparesis of the right thoracic limb at the last follow-up 3 months post-injury. This case report highlights the MRI-based diagnosis of traumatic hemorrhagic myelopathy in a dog. A fair short-term outcome was achieved with conservative treatment in this case.

A Zebra Diagnosis: A Case of Spontaneous Hematomyelia Associated With COVID-19.

Spontaneous non-traumatic spinal hematomyelia, characterized by intramedullary spinal hematoma, is a rare neurological emergency. Bleeding arteriovenous malformation, coagulopathies, and neoplasms are reported causes of this rare diagnosis. The authors present a case of a previously healthy man who presented with acute paraplegia and was found to have a spontaneous hematomyelia in association with covid infection. He underwent laminectomy and hematoma evacuation but did not recover any neurological function.

Hematomyelia Presenting with Neurogenic Stunned Myocardium.

Various neurological disorders and emotional stress may cause left ventricular dysfunction, known as a neurogenic stunned myocardium. A previously healthy 71-year-old woman collapsed immediately after experiencing left arm numbness and pain. Thereafter, the patient complained of anterior chest pain and became comatose. An electrocardiogram showed ST-segment elevation of I, aVL, and V2-3 and depression of II, III, and aVF. Echocardiography revealed anteroseptal hypokinesis of the left ventricle. Emergency coronary angiography revealed no significant stenosis in the coronary arteries; however, left ventriculography revealed obvious anteroseptal hypokinesis. When the patient regained consciousness the following day, tetraplegia was observed. Spinal computed tomography and magnetic resonance imaging revealed an intramedullary spinal cord hemorrhage from the medulla to the conus. The cardiac function recovered, but the patient remained tetraplegic with poor spontaneous respiration. Although its incidence is extremely rare, hematomyelia should be recognized as a potential cause of neurogenic stunned myocardium.

Non-traumatic hemorrhagic myelopathy in dogs.

BACKGROUND: Non-traumatic spinal cord hemorrhage (NTSH) is an uncommon cause of myelopathy in dogs. OBJECTIVES: Describe the clinical characteristics, concurrent medical conditions and underlying causes, magnetic resonance imaging (MRI) findings and outcome in dogs with NTSH. ANIMALS: Dogs diagnosed with NTSH using gradient echo T2-weighted (GRE) sequences with or without histopathological confirmation of hemorrhage were included. Dogs with a traumatic cause were excluded, including those with compressive intervertebral disc extrusion. METHODS: Retrospective descriptive study; the databases of 2 referral hospitals were searched between 2013 and 2021. RESULTS: Twenty-three dogs met inclusion criteria. The onset of signs was acute and progressive in 70% of cases; spinal hyperesthesia was variable (48%). Hemorrhage was identified in the thoracolumbar spinal segments in 65% of dogs. An underlying cause was identified in 65% of cases. Angiostrongylus vasorum represented 18% of the total cohort, followed by steroid-responsive meningitis arteritis (SRMA; 13%). Overall, 64% of dogs had a good or excellent outcome, regardless of cause; which was increased to 100% for SRMA, 75% for A. vasorum and 75% for idiopathic NTSH. Outcome was not associated with neurological severity. Recovery rate was 67% and 50% for nociception-intact and nociception-negative dogs, respectively. CONCLUSIONS: Larger prospective studies would be required to define prognostic factors for dogs with NTSH, but outcome appeared to be most influenced by the underlying cause, as opposed to neurological severity at presentation.

Spontaneous intramedullary hematoma following COVID-19 vaccination: A case report.

A 67-year-old female was hospitalized due to right-sided hemiparesis and neck pain with rapid deterioration to a deep coma. She had received the Sinopharm vaccine 2 days earlier. MRI showed extensive cervicothoracic hematomyelia. She received intensive medical care for 2 months and was discharged. An 18-month follow-up showed significant neurological recovery.

Spontaneous intramedullary hematoma in a patient with COVID-19 infection: A case report.

A 73-year-old man experienced four limb paresthesia and weakness following severe COVID-19 pneumonia. EMG-NCS showed inflammatory demyelinating polyneuropathy pattern while cervicothoracic imaging showed hematomyelia. The patient underwent laminectomy and hematoma evacuation. Neurological status improved to ASIA score C, postoperatively.

[A case of central nervous system primary lymphomatoid granulomatosis with multiple vascular lesions in the spinal cord and brain].

A 71-year-old man was hospitalized because of low back pain and weakness in both lower limbs. He presented with fever and stiff neck, and his cerebrospinal fluid sample contained blood. MRI revealed intramedullary and epidural hemorrhages in the spinal cord. Microhemorrhages occurred frequently in the central nervous system over a short period. A brain biopsy was performed. The diagnosis was primary lymphomatoid granulomatosis (LYG) of the central nervous system (grade 2). As a result of lymphocytic infiltration to the vascular walls in LYG, hemorrhages occurred in multiple sites in the central nervous system. The biopsy of samples from the sites of microhemorrhages proved useful for diagnosis even in the absence of mass lesions.

Acute Myelopathy: Vascular and Infectious Diseases.

Vascular and infectious causes are rare but important causes of spinal cord injury. High suspicion for these processes is necessary, as symptoms may progress over hours to days, resulting in delayed presentation and diagnosis and worse outcomes. History and clinical examination findings can assist with localization of the affected vascular territory and spinal level, which will assist with focusing spinal imaging. Open and/or endovascular surgical management depends on the associated vascular abnormality. Infectious myelopathy treatment consists of targeted antimicrobial therapy when possible, infectious source control, and again, close monitoring for systemic complications.

Unique presentation and management of Gowers intrasyringal hemorrhage.

Context: Intrasyringal hemorrhage was first described in literature in the renowned Lectures on Diseases of the Nervous System by Sir William Richard Gowers [Gowers W. A lecture on syringal haemorrhage into the spinal cord. Lancet [Internet]; 162(4180):993-997. [cited 2019 August 19]. Available from https://www.sciencedirect.com/science/article/pii/S0140673601362785]. The pathophysiology of this disease is a hemorrhage within a preexisting intramedullary fluid-filled cavity or hydrosyringomyelia in the spinal cord. Most common symptoms of this disease are numbness, weakness, paresthesia in the extremities and gait disturbances. Since first noted in 1903, there have been just fourteen confirmed cases of this disease reported in literature, making it extremely rare and difficult to study.Findings: Here we discuss a unique presentation of Gowers intrasyringal hemorrhage. The patient is a male in his late teens with no prior established neurological history. He presented at our institution four days post suspected injury with persistent mild left upper extremity numbness and weakness. The patient was admitted and underwent an MRI of the cervical spine without intravenous contrast. The imaging findings confirm subacute on chronic Gowers intrasyringal hemorrhage in the setting of a Chiari 1 malformation. This patient underwent neurosurgical intervention four months post initial presentation and had complete postoperative resolution of his interval non-progressive but persistent symptoms.Clinical relevance: This unique case suggests immediate surgical intervention may not always be necessary for treating Gowers intrasyringal hemorrhage.

Intramedullary Hemorrhage Caused by Lumbosacral Epidural Arteriovenous Fistula with Dual Retrograde Perimedullary Venous Draining Routes: A Case Report and Review of the Literature.

BACKGROUND: Spinal epidural arteriovenous fistulas (SEAVFs) are rare lesions with a low risk of hemorrhage. Most patients with lumbosacral SEAVFs with hemorrhagic events will develop a spinal epidural hematoma from epidural venous pouches. To the best of our knowledge, we have reported the first case of a lumbosacral SEAVF presenting with remote intramedullary hemorrhage in the conus medullaris. CASE DESCRIPTION: A 56-year-old man presented with sudden-onset severe paraparesis and bowel/bladder dysfunction. Magnetic resonance imaging of the thoracic and lumbosacral spine showed acute intramedullary hemorrhage in the conus medullaris surrounded by spinal cord congestion extending to T8, with perimedullary flow voids along the ventral and dorsal cord surfaces. Magnetic resonance angiography and spinal angiography confirmed the presence of a SEAVF with a large lumbosacral epidural venous lake supplied by dorsal somatic branches of bilateral L4 segmental arteries. Initially, the intradural venous drainage had been misinterpreted as a single route. The patient underwent surgical interruption of the dilated intradural draining vein after embolization of the feeding vessels. Another small intradural venous drainage route was found on the follow-up angiogram. The second operation with occlusion of the initial part of the epidural venous lake was sufficient to cure this lesion. CONCLUSIONS: With the significant improvement of our patient after treatment of the SEAVF, we speculated that the intramedullary hemorrhage, which had occurred in the area of spinal cord edema, might have resulted from severe venous hypertension, rather than hemorrhagic infarction of the spinal cord. Careful evaluation of the angioarchitecture of SEAVFs is imperative to determine the most effective treatment modality.

Primary spinal atypical teratoid/rhabdoid tumour presenting with hematomyelia and subarachnoid haemorrhage-a case report.

Atypical teratoid/rhabdoid tumours (AT/RTs) are highly aggressive and uncommon malignant tumours of the central nervous system (CNS) affecting children younger than 3 years of age. Primary spinal cord involvement is an extremely rare presentation. AT/RTs show necrosis and haemorrhages on histopathology frequently. However, spinal atypical teratoid/rhabdoid tumour (AT/RT) with hematomyelia and spinal subarachnoid haemorrhage (SAH), as seen in our case, has never been reported in the literature in the paediatric age group. We report a case of primary spinal AT/RT in a 3-year-old male child presenting acutely with hematomyelia and spinal SAH and try to elucidate its pathophysiological basis.

Multiple Spinal Chronic Subdural Hematomas Associated with Thoracic Hematomyelia: A Case Report and Literature Review.

BACKGROUND: Chronic subdural hematoma (CSDH) is uncommon in the spine. Most spinal CSDHs occur as solitary lesions in the lumbosacral region. We report a rare case of multiple spinal CSDHs associated with hematomyelia. The diagnostic and therapeutic management of these complex spinal CSDHs is reviewed as well as the pertinent literature. CASE DESCRIPTION: A 79-year-old woman on warfarin therapy presented with lower back pain and progressive lower extremity weakness that had developed in the previous 2 weeks. She subsequently developed paraplegia and urinary incontinence. Thoracolumbar magnetic resonance imaging showed a CSDH from T12-L3 compressing the cauda equina. Single-shot whole-spine magnetic resonance imaging showed another CSDH and hematomyelia at T2-3. She underwent L2-3 hemilaminectomy, which revealed a liquefied subdural hematoma. Delayed T2 laminectomy exposed an organized subdural hematoma and xanthochromic hematomyelia. After each surgery, the patient showed significant motor recovery. Finally, the patient could walk, and the urinary catheter was removed. CONCLUSIONS: Spinal CSDH may occur in multiple regions and may be associated with hematomyelia. Whole-spine magnetic resonance imaging is useful to examine the entire spine for CSDH accurately and thoroughly. Comprehensive surgical exploration of all symptomatic hematomas may restore neurologic functions even with delayed surgery.

Spinal Cord Hemorrhage.

BACKGROUND AND PURPOSE: Spinal cord hemorrhages are rare conditions that can be classified based on the primary location of bleeding into intramedullary (hematomyelia), subarachnoid hemorrhage (SAH), subdural hemorrhage, and epidural hemorrhage. We conducted a literature review to better understand the presenting symptoms, etiology, diagnosis, and treatment of spinal cord hemorrhages. METHODS: We performed a literature search using PubMed with the key words spinal hemorrhage, hematomyelia, spinal subarachnoid hemorrhage, spinal subdural hematoma, and spinal epidural hematoma RESULTS: Most commonly, spinal hematomas present with acute onset of pain and myelopathy but a more insidious course also may occur. Spinal SAH may be especially difficult as it may cause cerebral symptoms. The etiologies vary based on the type (location). The most common causes are trauma, iatrogenic causes, vascular malformations, and bleeding diatheses. Management is often aimed toward rapid surgical decompression and correction of the underlying etiology if possible. Conservative management, including administration of large doses of corticosteroids, reversal of anticoagulation, and close monitoring, has been used as bridging for surgical procedure or as the mainstay of treatment for patients with mild or improving symptoms. CONCLUSIONS: The variable and overlapping presentations of spinal cord hemorrhages make the diagnosis challenging. Maintaining high levels of clinical suspicion and utilizing magnetic resonance imaging may help in making the right diagnosis. Future studies should aim to create standardized outcome grading system and management guidelines for patients with spinal hemorrhage.

Idiopathic Cervical Hematomyelia in an Infant: Spinal Cord Injury without Radiographic Abnormality Caused by a Trivial Trauma? Case Report and Review of the Literature.

BACKGROUND: Spontaneous or idiopathic intramedullary bleeding is a very rare event in pediatric patients. This diagnosis requires an extended clinical, laboratory, and radiologic work-up to rule out all potential causes of hematomyelia. However, children may present with hematomyelia or spinal cord injury without radiographic abnormality even after a minor trauma. CASE DESCRIPTION: A 15-month-old girl presented with a 24-hour history of progressive neurologic deficits. A trivial trauma had occurred a few days before the clinical onset. Head computed tomography scan and craniospinal magnetic resonance imaging revealed an isolated hemorrhagic central medullary lesion extending from the obex to C3 level. No underlying causes of intramedullary bleeding were identified. In the absence of obvious vascular abnormalities, the patient underwent an urgent occipitocervical decompression with hematoma evacuation. Postoperatively, the patient's motor symptoms rapidly resolved, and she was discharged with cervical collar immobilization. CONCLUSIONS: We discuss the differential diagnosis of intramedullary bleeding in children, focusing on the diagnostic protocol and therapeutic options in this age group.

Idiopathic Spontaneous Intramedullary Hemorrhage: A Report of a Rare Case of Repeated Intramedullary Hemorrhage with Unknown Etiology.

A 48-year-old woman presented with acute voiding difficulty, numbness and weakness of both lower extremities. Magnetic resonance imaging (MRI) showed an intramedullary hemorrhagic mass that extended from T9 to T10. T8-T10 laminotomy and surgical removal of the hemorrhagic mass was performed. The pathological diagnosis was hematoma. Her neurological status remained the same after the operation. At 5 days post-operation, the patient suddenly became paraplegic, and MRI that was immediately performed revealed a recurrent intramedullary hemorrhage. Emergent surgical evacuation was performed. Again, histological examination showed only hematoma, without any evidence of abnormal vessels or a tumor. A postoperative MRI revealed no abnormal lesions other than those resulting from postoperative changes. At a 9-month follow up, the patient could walk a short distance with the aid of a walker. Because spontaneous intramedullary hemorrhage with unknown etiology is very rare, it is essential to perform a meticulous inspection of the hemorrhagic site to find the underlying cause. Repeated hemorrhage can occur; therefore, close observation of patients after surgery is important in cases without an apparent etiology. Urgent surgical evacuation is important to improve outcomes in these cases.

Idiopathic Spontaneous Intramedullary Hemorrhage: A Report of a Rare Case of Repeated Intramedullary Hemorrhage with Unknown Etiology

A 48-year-old woman presented with acute voiding difficulty, numbness and weakness of both lower extremities. Magnetic resonance imaging (MRI) showed an intramedullary hemorrhagic mass that extended from T9 to T10. T8-T10 laminotomy and surgical removal of the hemorrhagic mass was performed. The pathological diagnosis was hematoma. Her neurological status remained the same after the operation. At 5 days post-operation, the patient suddenly became paraplegic, and MRI that was immediately performed revealed a recurrent intramedullary hemorrhage. Emergent surgical evacuation was performed. Again, histological examination showed only hematoma, without any evidence of abnormal vessels or a tumor. A postoperative MRI revealed no abnormal lesions other than those resulting from postoperative changes. At a 9-month follow up, the patient could walk a short distance with the aid of a walker. Because spontaneous intramedullary hemorrhage with unknown etiology is very rare, it is essential to perform a meticulous inspection of the hemorrhagic site to find the underlying cause. Repeated hemorrhage can occur; therefore, close observation of patients after surgery is important in cases without an apparent etiology. Urgent surgical evacuation is important to improve outcomes in these cases.

Radiation-induced spinal cord hemorrhage (hematomyelia).

Intraspinal hemorrhage is very rare and intramedullary hemorrhage, also called hematomyelia, is the rarest form of intraspinal hemorrhage, usually related to trauma. Spinal vascular malformations such intradural arteriovenous malformations are the most common cause of atraumatic hematomyelia. Other considerations include warfarin or heparin anticoagulation, bleeding disorders, spinal cord tumors. Radiation-induced hematomyelia of the cord is exceedingly rare with only one case in literature to date. We report the case of an 8 year old girl with Ewing's sarcoma of the thoracic vertebra, under radiation therapy, presenting with hematomyelia. We describe the clinical course, the findings on imaging studies and the available information in the literature. Recognition of the clinical pattern of spinal cord injury should lead clinicians to perform imaging studies to evaluate for compressive etiologies.