Spinal cord ischemia - from diagnosis to treatment.

Purpose: The aim of the article is to outline the frequency of the occurrence, course, diagnosis and possible treatment of spinal cord ischemia (SCI) on the basis of a literature overview and to raise awareness of this rare yet devastating condition. Views: SCI, when compared to cerebral stroke, is a relatively rare disease, being diagnosed 100 times less often. Our knowledge as to its root causes, proper treatment for it and long-term prognosis is still inconclusive. Magnetic resonance imaging with diffusion- weighted contrast is a main tool with which to confirm SCI and rule out a broad spectrum of possible alternative diagnoses. SCI is a condition in which prompt recognition, accurate diagnostic steps, and reperfusion therapy are essential to ensure a desirable functional outcome and reduce mortality and disability. Although there are no specific guidelines regarding treatment, the administration of rt-PA might be an effective therapy for acute ischemic stroke, preventing permanent spinal dysfunction. In surgical causes close cooperation between a neurologist and a neurosurgeon is necessary to provide combined appropriate management promptly. Conclusions: Due to the relative rarity of SCI, multi-center studies of ischemia of the spinal cord and its treatment would be advisable in neurological practice to enhance current knowledge. A rapid diagnosis is crucial for appropriate care and desirable long-term outcomes.

Outcome in paraplegic dogs with or without pain perception due to thoracolumbar fibrocartilaginous embolic myelopathy or acute non-compressive nucleus pulposus extrusion.

Background: Fibrocartilaginous embolic myelopathy (FCEM) and acute non-compressive nucleus pulposus extrusion (ANNPE) are common causes of acute spinal cord injury in dogs. Outcome among paraplegic deep pain positive (DPP) and deep pain negative (DPN) dogs with either condition and factors influencing recovery have not been clearly established. Methods: Dogs with thoracolumbar FCEM or ANNPE resulting in paraplegia presenting to university hospitals between 2012 and 2022 were retrospectively included. Diagnosis of FCEM or ANNPE was based on clinical and magnetic resonance imaging findings. Outcome was defined as successful (recovery of independent ambulation) or unsuccessful (non-ambulatory ≥3 months following diagnosis or at the time of death/euthanasia). Logistic regression analysis was performed to investigate associations between clinical or imaging variables and outcome. Results: Thirty-one dogs were included. In total, 14 dogs were initially paraplegic DPP (8 FCEM, 6 ANNPE) and 17 dogs were paraplegic DPN (11 FCEM, 6 ANNPE). Outcome was available for 26 dogs (14 DPP, 12 DPN) with a median follow-up time of 182 days (range 0-2,311) including 2 dogs euthanized at the time of diagnosis; 1 of 12 DPN dogs (8.3%) regained independent ambulation, whereas 9 of 14 DPP dogs (64.3%) regained independent ambulation. DPN dogs had a significantly higher risk of not regaining independent ambulation compared with DPP dogs (OR: 47.40, 95% CI: 2.09-1073.99). No other variables were associated with outcome. Conclusion: While recovery of ambulation was possible, these results confirm that the absence of pain perception is a useful negative prognostic indicator in dogs with severe thoracolumbar FCEM or ANNPE.

Clinical signs, causes, and outcome of central cord syndrome in 22 cats.

OBJECTIVE: To describe the signalment, clinical findings, presumptive or definitive diagnosis, and outcome in cats with central cord syndrome (CCS). ANIMALS: 22 cats. CLINICAL PRESENTATION: Cats evaluated for CCS at 7 referral hospitals between 2017 and 2021 were included. Information retrieved from medical records included signalment, physical and neurological examination findings, diagnostic investigations, definitive or presumptive diagnosis, treatment, and follow-up. RESULTS: Median age at presentation was 9 years. Two neuroanatomical localizations were associated with CCS: C1-C5 spinal cord segments in 17 (77.3%) cats and C6-T2 spinal cord segments in 5 (22.7%) cats. Neuroanatomical localization did not correlate with lesion location on MRI in 8 (36.3%) cats. The most common lesion location within the vertebral column was over the C2 and C4 vertebral bodies in 6 (27.2%) and 5 (22.7%) cats, respectively. Peracute clinical signs were observed in 11 (50%) cats, acute in 1 (4.5%), subacute in 4 (18%), and chronic and progressive signs were seen in 6 (40.9%) cats. The most common peracute condition was ischemic myelopathy in 8 (36.3%) cats, whereas neoplasia was the most frequently identified chronic etiology occurring in 5 (22.7%) cats. Outcome was poor in 13 (59%) cats, consisting of 4 of 11 (36.6%) of the peracute cases, 3 of 4 (75%) of the subacute cases, and 6 of 6 of the chronic cases. CLINICAL RELEVANCE: Central cord syndrome can occur in cats with lesions in the C1-C5 and C6-T2 spinal cord segments. Multiple etiologies can cause CCS, most commonly, ischemic myelopathy and neoplasia. Prognosis depends on the etiology and onset of clinical signs.

Suspected Cerebral Salt Wasting Syndrome with Cervical Spinal Lesion in a Domestic Shorthair Cat.

A 12-year-old spayed female domestic short cat was presented with tetraplegia. The cat also showed signs of hyponatremia and dehydration, which were rapidly corrected by intravenous fluid infusion. Based on thorough physical and neurological examinations, the patient was suspected of having an intracranial disease. MRI revealed a high-signal T2 image of the bilateral parietal cerebral cortical gray matter junction, which is associated with fast electrolyte calibration, and a high-signal T2 image of the C2 spinal cord ventral area, which is associated with ischemic myelopathy. The cat reappeared three days later due to anorexia. Laboratory examinations revealed that the cat was clinically dehydrated and exhibited hyponatremia. Other causes of hyponatremia were excluded through history-taking, laboratory examination, imaging, and therapeutic response to fluid therapy, except for cerebral salt-wasting syndrome (CSWS). The cat was discharged 3 days after the start of fludrocortisone therapy with electrolytes within the normal range. Magnetic resonance imaging (MRI) was performed again 1 month after hospitalization, and the cerebral lesion disappeared, but the spinal cord lesion worsened compared to the previous image. The patient was euthanized due to the progression of the spinal lesion, with a poor prognosis and poor quality of life. This is the first case of suspected CSWS with a cervical spinal lesion in a cat.

Spontaneous spinal cord infarction in Austria: a two-center comparative study.

Background: Spinal cord infarction (SCI) is a neurological emergency associated with high rates of persistent neurological deficits. Knowledge about this rare but potentially treatable condition needs to be expanded. Objective: To describe the characteristics of spontaneous SCI in a large retrospective series of patients treated at two tertiary care centers in Austria. Methods: We performed a descriptive and comparative analysis of spontaneous SCI treated at the University Hospitals of Salzburg and Graz between the years 2000 and 2020. The analysis included pre- and in-hospital procedures, clinical presentation, etiology, diagnostic certainty, reperfusion therapy, and functional outcome at discharge. Results: We identified 88 cases, 61% were ascertained in the second half of the study period. The median age was 65.5 years [interquartile range (IQR) = 56-74], 51.1% were women. Anterior spinal artery infarction was the predominant syndrome (82.9%). Demographics, vascular comorbidities, and clinical presentation did not differ between the centers. The most frequent etiology and level of diagnostic certainty were distinct, with atherosclerosis (50%) and definite SCI (42%), and unknown (52.5%) and probable SCI (60%) as front runners in Salzburg and Graz, respectively. Patients arrived after a median of 258.5 min (IQR = 110-528) at the emergency room. The first magnetic resonance imaging (MRI) of the spinal cord was performed after a median of 148 min (IQR = 90-312) from admission and was diagnostic for SCI in 45%. Two patients received intravenous thrombolysis (2.2%). The outcome was poor in 37/77 (48%). Conclusion: Demographics, clinical syndromes, and quality benchmarks for spontaneous SCI were consistent at two Austrian tertiary care centers. Our findings provide the foundation for establishing standards for pre- and in-hospital care to improve outcomes.

MRI negative myelopathy post mild SARS-CoV-2 infection: vasculopathy or inflammatory myelitis?

Since the onset of the COVID-19 pandemic, there have been rare reports of spinal cord pathology diagnosed as inflammatory myelopathy and suspected spinal cord ischemia after SARS-CoV-2 infection. Herein, we report five cases of clinical myelopathy and myeloradiculopathy in the setting of post-COVID-19 disease, which were all radiographically negative. Unlike prior reports which typically characterized hospitalized patients with severe COVID-19 disease and critical illness, these patients typically had asymptomatic or mild-moderate COVID-19 disease and lacked radiologic evidence of structural spinal cord abnormality. This case series highlights that COVID-19 associated myelopathy is not rare, requires a high degree of clinical suspicion as imaging markers may be negative, and raises several possible pathophysiologic mechanisms.

Fatal outcome of cervical myelopathy caused by fibrocartilaginous embolism. Rare cause of spinal vascular damage.

BACKGROUND AND PURPOSE: Fibrocartilaginous embolism is a rare cause of ischemic myelopathy. Authors report a case of a 39-year-old woman with progressive tetraparesis and severe autonomic dysfunction. Despite of the detailed examinations, the definite diagnosis was verified by autopsy. METHODS: The patient was admitted because of progressive pain and numbness of the upper extremities and tetraparesis. Hypotonic muscles of the lower extremities with mild tetraparesis were observed. Magnetic resonance imaging showed an intramedullary lesion at the level of the cervical V-VII vertebral. Patient's tetraparesis worsened gradually to plegia with urinary retention. Expansive, rapidly progressing multiple decubiti developed, which were resistant to therapy. In spite of the complex therapy, the patient died. RESULTS: No internal disease was found to explain the death by autopsy. Multiple subacute infarctions of the cervical myelon (involving the lateral columns as well) in the territory of the anterior spinal artery were verified by neuropathological examination. The occluded vessels were filled by a material containing cartilaginous cells, while signs of atherosclerosis or thrombosis were not present. CONCLUSION: Cartilaginous embolism of spinal arteries was diagnosed.

Atypical Spinal Cord Infarction: A Prolonged and Stuttering Course for Six Days.

PURPOSE: Spinal infarction is a rare condition and usually presents with a sudden or acute course. A prolonged course is rare and may mimic the presentation of inflammatory myelitis. Here we present a case of atypical spinal cord infarction with a stuttering course for six days.. CASE REPORT: A 47-year-old male presented initially with symptoms of sudden onset, limb pain. Sudden chest pain radiating to the back, occurred three days later. Sudden urinary retention and quadriparesis were presented after another three days. The diagnosis of spinal cord infarction was made through diffusion restriction in spinal magnetic resonance imaging. CONCLUSION: A prolonged course of spinal cord infarction is relatively uncommon but a stepwise and stuttering course may provide clues. Diffusion restriction in magnetic resonance imaging also may be helpful. The diagnosis of spinal cord infarction should always be kept in mind.

Convergence-Retraction Nystagmus in a Dog With Presumptive Ischemic Encephalopathy Following Acute Cervicothoracic Myelopathy.

A 6.5-year-old male neutered Trailhound was admitted for hyperacute, nonprogressive, left-sided hemiparesis. Physical and neurologic examination revealed nonpainful, left-sided poorly ambulatory hemiparesis, decreased left-sided postural reactions and thoracic limbs hyporeflexia. Neuroanatomic localisation was consistent with a left-sided C6-T2 myelopathy. Haematology and biochemistry revealed nonspecific abnormalities. Magnetic resonance imaging of the neck revealed a focal intramedullary lesion at the level of C6-C7 vertebrae compatible with acute hydrated noncompressive nucleus pulposus extrusion or ischemic myelopathy. During the second day of hospitalization, the dog developed convergence-retraction nystagmus, up-gaze palsy and eyelid retraction (Collier's sign) compatible with dorsal midbrain syndrome. Magnetic resonance imaging of the brain revealed a focal lesion compatible with dorsal midbrain ischemic infarct. Further clinicopathologic testing, thoracic and abdominal imaging were unremarkable. Ischemic encephalopathy of unknown etiology was additionally diagnosed. Physiotherapy was performed therapeutically. At 1-year follow-up the dog was normal. This is an unusual report of a dog with myelopathy followed by ischemic encephalopathy with manifestation of convergence-retraction nystagmus in the absence of vestibular signs. This saccadic intrusion is a characteristic clinical manifestation of a dorsal midbrain syndrome localization. The importance of a complete differential diagnoses list formation in a dog with ischemic encephalopathy which leads to a thorough diagnostic investigation plan is highlighted. Moreover, this report contributes to the enrichment of the clinical reasoning veterinary literature on convergence-retraction nystagmus. To the authors' knowledge, this is the second case report (fourth dog) to describe convergence-retraction nystagmus in dogs as a manifestation of dorsal midbrain syndrome.

Pediatric fibrocartilaginous embolism inducing paralysis.

BACKGROUND: Fibrocartilaginous embolism (FCE) is a rare cause of pediatric ischemic myelopathy. The pathology is thought to result from fragmentation with embolization into the microvasculature of the radicular artery often secondary to high axial force. While most cases arise in the setting of vigorous activity, our case reveals that FCE can also occur during relative physical inactivity. Additionally, while a majority of cases are associated with neck or back pain, our case also reveals that FCE can present without concurrent pain episodes. We describe a rare case of spinal cord infarction (SCI) likely due to FCE in a 14 year old male. Our patient was sitting with his feet elevated, playing a video game, when he developed sudden difficulty moving his arms. Initially presenting with a negative MRI scan and la belle indifference, our patient was suspected to exhibit functional quadraparesis secondary to psychosomatization/adjustment disorder. Repeat MR imaging 7 days later revealed typical findings for FCE with SCI (irregular, pencil-like T2 hyperintensity in the ventral cervical/upper thoracic cord and owl's eye pattern on axial images). Six months later, the diagnosis of FCE remains predominant. Our patient continues to improve with occupational and physical therapy. Ambulatory efforts and bladder function continue to progress. To improve functional gains, the patient is being considered for a chemodenervation procedure. CONCLUSION: Our case reveals that FCE can occur during physical inactivity and present without concurrent pain. Outcome regarding pediatric fibrocartilaginous embolism is highly variant; however, the two largest outcomes reported were either patient death or discharge.

Fibrocartilaginous Embolism of the Spinal Cord in Children: A Case Report and Review of Literature.

Fibrocartilaginous embolism is assumed to be caused by fibrocartilaginous nucleus pulposus component migration through retrograde embolization to the spinal cord artery. Fibrocartilaginous embolism is currently not well recognized among pediatricians because of its rarity. We describe a previously healthy 15-year-old soccer player who, after kicking a ball, developed progressive weakness in both legs and urinary retention the next day. Magnetic resonance imaging revealed T2 hyperintensity in the anterior horn of the spinal cord at the Th12/L1 level with Schmorl node at the level of L1/2. We also review the previous literature on fibrocartilaginous embolism of the spinal cord in children (less than18 years age); a total of 25 pediatric patients, including our patient, were identified. The median age was 14 years, and 64% of the reviewed patients were female. The most common trigger event was intense exercise or sports. The neurological symptoms started within one day in most cases, and the time to symptom peak varied from a few hours to two weeks. The most common initial neurological symptoms were weakness or plegia (100%), followed by paresthesia or numbness (48%). Affected areas of the spinal cord were distributed evenly from the cervical to thoracolumbar regions. Although steroids and anticoagulants were most commonly used, the prognosis was quite poor (mild to severe sequelae with three deaths). Although fibrocartilaginous embolism is a very rare condition, physicians should be aware of the characteristics and include fibrocartilaginous embolism of the spinal cord in their differential diagnosis, especially for physically active patients.

Acute ischemic myelopathy treated with intravenous thrombolysis: Four new cases and literature review.

BACKGROUND: Intravenous thrombolysis is a well-established treatment of ischemic stroke within 4.5 h. However, its effectiveness in acute ischemic myelopathy is unknown. PURPOSE: We describe a series of four acute ischemic myelopathy patients treated with intravenous thrombolysis within 4.5 h and review the current literature to explore this treatment feasibility, potential safety, and efficacy. METHODS: We reviewed all routinely collected clinical, radiological, and follow-up data of patients with a final acute ischemic myelopathy diagnosis who received acute intravenous thrombolysis in our stroke network. We also reviewed thrombolyzed acute ischemic myelopathy patients in the literature. RESULTS: Four patients (three women) aged 57 to 83 years presented with acute uni- or bilateral extremity paresis, considered initially as cerebral strokes in two of them. After excluding contraindications by brain imaging in three, spinal computed tomography in one and confirmation of acute ischemic myelopathy on spinal magnetic resonance imaging in one patient, intravenous thrombolysis was administered at 135, 190, 240, and 245 min accordingly. Subacute diffusion-weighted imaging-magnetic resonance imaging confirmed acute ischemic myelopathy in all but one patient. Favorable outcome was achieved in two patients rapidly and in three patients at three-month follow-up. We identified seven other thrombolyzed acute ischemic myelopathy patients in the literature, who showed variable recovery and no hemorrhagic complications. CONCLUSIONS: With appropriate acute imaging, intravenous thrombolysis after acute ischemic myelopathy is feasible and potentially safe within 4.5 h. Given the potential of benefit of thrombolysis in acute ischemic myelopathy, this treatment warrants further efficacy and safety studies.

Spinal cord infarction: Clinical and imaging insights from the periprocedural setting.

OBJECTIVE: Describe the range of procedures associated with spinal cord infarction (SCI) as a complication of a medical/surgical procedure and define clinical and imaging characteristics that could be applied to help diagnose spontaneous SCI, where the diagnosis is often less secure. METHODS: We used an institution-based search tool to identify patients evaluated at Mayo Clinic, Rochester, MN from 1997 to 2016 with a periprocedural SCI. We performed a descriptive analysis of clinical features, MRI and other laboratory findings, and outcome. RESULTS: Seventy-five patients were identified with SCI related to an invasive or non-invasive surgery including: aortic aneurysm repair (49%); other aortic surgery (15%); and a variety of other procedures (e.g., cardiac surgery, spinal decompression, epidural injection, angiography, nerve block, embolization, other vascular surgery, thoracic surgery) (36%). Deficits were severe (66% para/quadriplegia) and maximal at first post-procedural evaluation in 61 patients (81%). Impaired dorsal column function was common on initial examination. Imaging features included classic findings of owl eyes or anterior pencil sign on MRI (70%), but several other T2-hyperintensity patterns were also seen. Gadolinium enhancement of the SCI and/or cauda equina was also common when assessed. Six patients (10%) had an initial normal MRI despite a severe deficit. CONCLUSIONS: Procedures associated with SCI are many, and this complication does not exclusively occur following aortic surgery. The clinical and radiologic findings that we describe with periprocedural SCI may be used in future studies to help distinguish spontaneous SCI from alternate causes of acute myelopathy.

Ischemic myelopathy caused by fibrocartilaginous embolism in a horse / Mielopatia isquêmica associada a embolismo fibrocartilaginoso em um equino

ABSTRACT: This report described clinical, epidemiological, and pathological aspects of ischemic myelopathy caused by fibrocartilaginous embolism (FCE) in a 10-year-old, mixed breed gelding. Clinically, the horse presented acute hind limbs paralysis, with a clinical course of approximately 24 hours. At necropsy, no gross lesions were observed. Cross-sections of the spinal cord revealed focally extensive areas of malacia from the T10 to L4 segments. Focally extensive areas of liquefactive necrosis involving the gray matter and adjacent white matter were observed on histologic sections. The lumen of multiple blood vessels in the periphery of the necrotic areas was occluded by fibrocartilaginous emboli that strongly stained with alcian blue. Clinical signs, gross necropsy, and histological findings observed in this case were identical to those described in the literature for ischemic myelopathy caused by FCE in the horse and other species.

RESUMO: Este relato descreve os aspectos epidemiológicos, clínicos e anatomopatológicos de um caso de mielopatia isquêmica por embolismo fibrocartilaginoso (FCE) em um equino, macho castrado, sem raça definida, com 10 anos de idade. Clinicamente, o equino apresentou paralisia aguda dos membros pélvicos com evolução de aproximadamente 24 horas. Na necropsia não foram observadas alterações. Após serem realizados vários cortes transversais na medula espinhal, observou-se área focalmente extensa de malacia entre o segmento T10 até L4. Histologicamente haviam áreas focalmente extensas de necrose de liquefação envolvendo a substância cinzenta e a substância branca adjacente. Múltiplos vasos sanguíneos da periferia das áreas de necrose estavam ocluídos por êmbolos fibrocartilaginosos, que reagiram fortemente pela técnica de azul alciano. Os sinais clínicos, as lesões macroscópicas e os achados histológicos observados neste caso, são idênticos aos descritos na literatura para FCE em equinos e nas demais espécies.

Clinical characteristics of canine fibrocartilaginous embolic myelopathy (FCE): a systematic review of 393 cases (1973-2013).

Fibrocartilaginous embolic myelopathy (FCE) is common in dogs; however, there is conflicting information in the veterinary literature regarding clinical characteristics and data on recovery in severe cases is sparse. A systematic review of canine FCE was performed to delineate the natural history of this disease. 322 previously reported cases and 71 previously unreported cases were identified for inclusion. Source publications were identified via PubMed central search and by references from review articles. Previously unreported cases were identified via computerised medical records search at two veterinary institutions. FCE was most common in middle-aged large breed dogs (30 per cent); however, the miniature schnauzer was the most frequently reported individual breed and small breeds comprised 24 per cent of all reported cases. The most common neuroanatomical localisation was a T3-L3 myelopathy (33.1 per cent). Prognosis for recovery of ambulation was good to excellent with 85 per cent of cases regaining the ability to walk unassisted, most within 3 weeks. Persistent neurological deficits were common in patients that recovered ambulation (49.1 per cent). When nociception was absent in the affected limbs at initial presentation, rate of recovery was lower (10 per cent); however, this data is likely biased by limited follow-up in more severe cases. Future prospective studies should evaluate prognosis for more severely affected patients.

Spinal Cord Infarction.

Spinal cord infarction (SCI) is uncommon and, therefore, not extensively studied. Little is known about the epidemiology and individual risk factors for SCI. The prognosis for functional recovery after SCI from any cause is not well documented. Despite these deficiencies, useful clinical information regarding SCI following surgical procedures is available. Many nonsurgical causes of SCI are also recognized, including aortic pathology, systemic hypoperfusion, atherosclerotic emboli, or other embolic materials. An understanding of the patterns of blood supply to the spinal cord and the more common causes of SCI will aid in guiding future trials of neuroprotective medications, growth factors, and various perioperative techniques aimed at decreasing functional disability following SCI.