Posterior spinal artery infarct.

Posterior spinal artery syndrome has a variable presentation and often poses a clinical challenge. We describe an acute posterior spinal artery syndrome in a man in his 60s with vascular risk factors, who presented with altered sensation in the left arm and left side of his torso but with normal tone, strength and deep tendon reflexes. MR imaging showed a left paracentral T2 hyperintense area affecting the posterior spinal cord at the level of C1. Diffusion-weighted MRI (DWI) showed high signal intensity in the same location. He was medically managed as having ischaemic stroke and made a good recovery. Three-month MRI follow-up showed a persisting T2 lesion but the DWI changes had resolved, consistent with the time course for infarction. Posterior spinal artery stroke has a variable presentation and is probably under-recognised clinically, requiring careful attention to MR imaging for its diagnosis.

Anti-neutrophil cytoplasmic antibody associated central nervous system vasculitis with brain and spinal cord subarachnoid hemorrhage: A rare case report and review of the literature.

This case report describes a 48-year old female who presented with altered mental status, lower extremity weakness, low back pain and a recent history of subjective fevers and night sweats found to have posterior parieto-occipital and spinal subarachnoid hemorrhage on imaging. Further work-up revealed vasculitic changes in the intracranial vasculature and the external carotid artery on angiography. She also demonstrated positivity for perinuclear anti-neutrophil cytoplasmic (p-ANCA) antibodies overall consistent with ANCA associated central nervous system vasculitis (AAV). The present case describes a rare and new presentation of AAV that caused both a cerebral and spinal subarachnoid hemorrhage. There has been no documentation of spinal subarachnoid hemorrhage associated with primary or secondary vasculitis in the literature. Ultimately, this case demonstrates the important finding that AAV can have spinal cord manifestations and cervical vasculature involvement along with the more classic intra-cranial vasculitis findings.

[The history of surgery for brain and spinal cord vascular pathology at the Burdenko Neurosurgical Institute (to the 50th anniversary of foundation of the First Neurovascular Department)]. / Istoriya khirurgii sosudistoi patologii golovnogo i spinnogo mozga v Institute neirokhirurgii im. akad. N.N. Burdenko (k 50-letiyu osnovaniya pervogo neirososudistogo otdeleniya).

The Vascular Department of the Burdenko Neurosurgical Institute is one of the country's first dedicated departments engaged in treatment of patients with cerebrovascular diseases. The modern vascular service of the Institute is represented by several departments and groups: the Department of Microsurgical Treatment of Vascular Diseases, a group of Reconstructive Brachiocephalic Surgery, and the Department of Endovascular Surgery and Neurodiagnosis that is also engaged in intra-arterial chemotherapy and angiographic diagnosis. The neurovascular service of the Institute is a rightful leader of Russia in the number operations and their complexity: patients with the most serious and unusual pathologies are referred to the Institute from across the country. The achievements of the service are based on science and clinical practice that underlie progressive improvement in the diagnosis, surgical methodology, and recovery of neurovascular patients. On November 02, 2016, the Vascular Department of the Burdenko Neurosurgical Institute will celebrate the 50th anniversary of its foundation.

Suspected primary hematomyelia in 3 dogs.

Primary hematomyelia refers to hemorrhage occurring within the spinal cord without an identifiable etiology. Clinical signs, magnetic resonance imaging characteristics, and histopathological findings are described. Diagnosis was made through histological analysis and rule-outs for underlying factors. Following removal of the hematoma, neurologic deficits improved, although some residual deficits persisted.

Hématomyélie primaire suspectée chez 3 chiens. L'hématomyélie primaire fait référence à l'hémorragie qui se produit dans la moelle épinière sans une étiologie identifiable. Les signes cliniques, les caractéristiques de l'imagerie par résonance magnétique et les résultats de l'histopathologie sont décrits. Le diagnostic a été posé à l'aide d'une analyse histologique et de l'élimination des facteurs sous-jacents. Après l'enlèvement de l'hématome, le déficit neurologique s'est amélioré, même si des déficits résiduels ont persisté.(Traduit par Isabelle Vallières).

[A case of atraumatic hematomyelia extending from the C1 to T11 segments of the spinal cord].

Intramedullary spinal cord hemorrhage (hematomyelia) is rare and usually related to trauma. Spinal vascular malformations such as intramedullary cavernomas and intradural arteriovenous malformations are the most common cause of atraumatic hematomyelia. Other considerations include warfarin or heparin anticoagulation, bleeding disorders, spinal cord tumors, and delayed complication of spinal radiation. We report the case of 48-year-old man receiving warfarin and aspirin therapy, who showed upper limb pain and dysesthesia from left axilla to left femur. Paraplegia, sensory disturbance, bladder and rectal disturbance developed gradually over two weeks, accompanied by severe back and neck pain. MRI showed hematomyelia extending from the C1 to T11 segments of the spinal cord. The hemorrhage was located mainly in the left side of the posterior column. Few cases of hematomyelia extending over 18 segments of the spinal cord have been reported. Past literature reports of hematomyelia tend to extend longitudinally above and below the area of initial hemorrhage. We thought that the shape of this hematomyelia extending longitudinally over several segments was formed by a similar pathogenesis to pencil-shaped softening of the spinal cord.

Clinical features and treatment outcomes of the spinal arteriovenous fistulas and malformation: clinical article.

OBJECT: Spinal vascular diseases, such as spinal dural arteriovenous fistulas (DAVFs), perimedullary arteriovenous fistulas (AVFs), and spinal arteriovenous malformations (AVMs), are very rare. The authors analyzed the features and treatment outcomes of these conditions. METHODS: Data from 64 patients were retrospectively reviewed. There were 33 spinal DAVFs (1 patient had 2 lesions), 20 perimedullary AVFs, and 12 spinal AVMs. Clinical features, radiological findings, treatment results, and clinical outcomes were evaluated according to the diseases, subtypes, and treatment modalities. The median duration of follow-up was 20, 42, and 56 months for spinal DAVFs, perimedullary AVFs, and spinal AVMs, respectively. RESULTS: Spinal DAVFs showed faster progression of symptoms (median 5, 12, and 36 months for spinal DAVFs, perimedullary AVFs, and spinal AVMs, respectively) and worse neurological status at diagnosis (poor neurological status in 56%, 65%, and 33%, respectively). On MRI, signal voids were demonstrated in all except 1 spinal DAVF. At the last follow-up, 94% of spinal DAVFs, 68% of perimedullary AVFs, and 50% of spinal AVMs were completely obliterated. Favorable clinical outcomes were achieved in 91%, 95%, and 58%, respectively. In detail, the majority (78%) of spinal DAVFs were embolized, resulting in complete obliteration in 92% and favorable clinical outcomes in 92%. Most Type IVa and IVb perimedullary AVFs were surgically treated (71% and 88%), with complete obliterations of 86% and 71%, and favorable clinical outcomes in 100% and 86%, respectively. All Type IVc lesions were embolized with a low cure rate of 40%; however, clinical outcomes were satisfactory. Spinal AVMs were generally embolized (67%), and only glomus-type lesions attained a satisfactory cure rate (80%) and clinical outcome (100%). CONCLUSIONS: Embolization produced satisfactory outcomes in spinal DAVFs and glomus-type spinal AVMs. Surgery is advantageous in Type IVa and IVb perimedullary AVFs. Palliative embolization can be effective in Type IVc perimedullary AVFs and juvenile spinal AVMs.

Posterior spinal artery syndrome showing marked swelling of the spinal cord: a clinico-pathological study.

OBJECTIVE: To describe a rare autopsy case of posterior spinal artery syndrome with marked swelling of the spinal cord, an unusually subacute onset and short clinical course. METHODS: Case report. FINDINGS: An 84-year-old Japanese woman presented with bilateral muscle weakness of the lower legs and sensory disturbance 1 week after head contusion. Neurological findings worsened gradually. She developed phrenic nerve paralysis and died of respiratory failure 6 weeks after the onset of neurological symptoms. On pathological examination, the spinal cord was markedly swollen in the cervical and upper thoracic segments. Microscopically, there was loss of myelin sheath in the bilateral posterior columns and neuronal loss of the posterior horns in all of the spinal segments. However, findings were unremarkable in the bilateral anterior columns and bilateral anterior horns in most of the spinal segments. Posterior spinal arteries had no stenosis, occlusion, or thrombosis. We considered that pathogenesis was infarction associated with head injury. CONCLUSION: To our knowledge, this is the first report of a case of posterior spinal artery syndrome with a markedly swollen spinal cord and poor prognosis.

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.

Hemorragia intramedular (Hematomielia)no traumática: revisión / Non traumatic intramedullary hemorrhage (hematomyelia): a review

La hemorragia intramedular (hematomielia) no traumática es el sangrado producido en el interior del tejido medular en ausencia de trauma, su primera descripción clínica aparece reportada por Tellegen en 1850, se le considera la más infrecuente de las hemorragias intraespinales y rara si la comparamos con la frecuencia en que ocurren las hemorragias intracerebrales. La incidencia es alta en los varones (relación hombre-mujer 1,5:1) y muy relacionada con la entidad patológica condicionante. Esta obedece a múltiples causas: malformaciones arteriovenosas, cavernomas, tumores, cuagulopatias, inflamatoria, etc. La disfunción aguda del cordón espinal junto a las imágenes por resonancia magnética son elementos decisivos para el diagnóstico. El tratamiento aun sujeto de controversias, médico o quirúrgico (precoz o tardío), dependerá de la etiología del sangrado y del estatus neurológico en el momento del diagnóstico, hallándose los resultados más favorables en aquellos pacientes a los cuales se les realizó un pronto diagnóstico seguidos de una cirugía temprana.

Non traumatic intramedular hemorrhage (hematomyelia) is the blood found in the medullar tissues in absence of trauma, its first clinical description was reported by Tellegen in 1850.Is considered to be the most infrequent of intraespinales hemorrhage. The incidence is high in men and is related with conditionate pathological entity. Its do to multiples causes: Arteriovenos malformation, cavernomas, tumors, coagulopathies, inflammations, etc. The acute dysfunction of the spinal cord and magnetic resonance imaging are decisive in the diagnosis. The treatment though subject to controversies, medical or surgery (early or late), will depend on the bleeding etiology and the neurological status in the moment of the diagnosis, finding the most favorable result in those patients that were diagnosed earlier following an early surgery.

Takayasu's arteritis complicated with subarachnoid hemorrhage and hematomyelia--case report.

A 44-year-old woman presented with severe headache, drowsy mentality, and right hemiparesis. Brain computed tomography and magnetic resonance angiography revealed non-aneurysmal subarachnoid hemorrhage (SAH). Thoraco-abdominal and pelvic computed tomography angiography showed multiple steno-occlusive lesions involving the aorta and its large branches suggesting Takayasu's arteritis. Spine magnetic resonance imaging was taken because of prominent right hand muscle atrophy on the 14th hospital day, which showed subacute stage of hematomyelia in the cervical cord and conus medullaris. Aneurysmal or non-aneurysmal SAH is rare in patients with Takayasu's arteritis but SAH with coincidental hematomyelia is even more unusual. This case emphasizes the rarity of the coincidental spinal hematomyelia and its importance in the differential diagnosis.

Infantile cervical intramedullary cavernous angioma manifesting as hematomyelia. Case report.

A 2-month-old infant presented with a cervical intramedullary spinal cord cavernous angioma manifesting as left hemiparesis caused by hematomyelia. Osteoplastic laminotomy of the cervical spine was carried out with subsequent microsurgical excision of the intramedullary spinal cord cavernous angioma. Magnetic resonance imaging revealed no residual tumor. At 25 months after the surgery, she presented no neurological deficit without recurrence and cervical deformity. This case of infantile intramedullary spinal cord cavernous angioma presenting with cervical hematomyelia shows osteoplastic laminotomy of the cervical spine might be helpful to prevent consequent cervical deformity in pediatric cases.

Subarachnoid hemorrhage following posterior spinal artery aneurysm. A case report and review of the literature.

Isolated posterior spinal artery aneurysms are rare vascular lesions. We describe the case of a 43-year-old man presenting with spinal subarachnoid hemorrhage after a minor trauma who was found to have a dissecting aneurysm of a posterior spinal artery originating from the right T4 level. Endovascular treatment was not contemplated because of the small size of the feeding artery, whereas surgical resection was deemed more appropriate because of the posterolateral perimedullary location that was well appreciated on CT angiography. After surgical resection of the aneurysm the patient had a complete neurological recovery. In comparison to anterior spinal artery aneurysms whose pathogenesis is diverse, posterior spinal aneurysms are most often secondary to a dissection and represent false or spurious aneurysms. Although the definite diagnosis still requires spinal angiography, MRI and CT may better delineate the relationship of the aneurysm to the spinal cord in order to determine the best treatment method. Prompt treatment is recommended as they have high rebleeding and mortality rates.

Intramedullary cavernoma presenting with hematomyelia: report of two girls.

INTRODUCTION: Less than 20 children with intramedullary cavernoma (ImC) have been reported in the English literature; however, cases with an unfavorable outcome may be underreported. Whereas these are predominantly boys, we report two girls who presented with hematomyelia (one cervical, one thoracic) and an acute, severe neurological deficit. CASE MATERIAL: A 10-year-old girl complaining about lower thoracic pain for several days suddenly developed lower body dysesthesias and paraparesis. Magnetic resonance (MR) demonstrated hematomyelia (T8-T11), intramedullary edema (T6-L1), and an ImC at T9-T10. Within an hour, she progressed to paraplegia and was therefore operated immediately. She slowly recovered regaining independent ambulation and continence. MR after 2 years shows no recurrence. A 7-year-old girl suddenly developed cervicalgia and paresis of her left arm and leg. MR demonstrated hematomyelia and an ImC at C4-C6. She gradually recovered with minimal residual deficit at 3 months and was subsequently operated uneventfully. Multiple cerebral cavernomas and a familial autosomal cavernous malformation syndrome were diagnosed. The following 1.5 years, she complained of intermittent cervicalgia and left brachial dysesthesias, with MR suggesting active residual cavernoma. Interestingly, her complaints gradually disappeared, and she is currently asymptomatic. MR after 3.5 years shows minimal cord swelling no longer suggesting active residual cavernoma. CONCLUSION: With adequate surgical treatment either in the acute phase in case of dramatic deterioration or after clinical recuperation, prognosis of symptomatic ImC may be surprisingly good. However, subtotally resected lesions and/or syndromal cases may recur, requiring further treatment. Definitive answers await more cases with longer follow-up.

Episodic encephalopathy due to an occult spinal vascular malformation complicated by superficial siderosis.

Superficial siderosis (SS) of the central nervous system is a rare condition caused by chronic subarachnoid hemorrhage. Clinical manifestations typically include sensorineural hearing loss and cerebellar ataxia. Recurrent episodic encephalopathy in the setting of SS has not been reported. We describe a unique case of SS in a 67-year-old man with an 8-year history of episodic encephalopathy associated with headache and vomiting. The patient also had a history of progressive dementia, ataxia, and myelopathy. A diagnosis of superficial siderosis was made after magnetic resonance gradient-echo images showed diffuse hemosiderin staining over the cerebellum and cerebral convexities. No intracerebral source of hemorrhage was identified. The patient therefore underwent gadolinium-enhanced spinal MRI which suggested a possible vascular malformation. A therapeutic laminectomy subsequently confirmed an arteriovenous fistula which was resected. In SS, there are often long delays between symptom onset and definitive diagnosis. Early identification is facilitated by magnetic resonance imaging with gradient-echo sequences. When no source of hemorrhage is identified intracranially, then total spinal cord imaging is indicated to assess for an occult source of hemorrhage as occurred in our case.

[Spinal cord hemorrhage complicating diagnostic lumbar puncture]. / Complicación espinal hemorrágica secundaria a una punción lumbar diagnóstica.

INTRODUCTION: Spinal cord hematoma is a serious and feared complication of lumbar puncture. We here describe two patients who developed a spinal cord hematoma following diagnostic lumbar punctures. CASE REPORTS: CASE 1: a 22-year-old male with a syndrome of cerebrospinal fluid hypotension, with normal coagulation parameters, underwent a traumatic, diagnostic lumbar puncture followed, a few hours later, by back pain irradiated to the legs. MRI showed the presence of a subdural hematoma from the lower dorsal region to the sacral region. A conservative approach, without surgery, was decided and he showed a complete recovery. CASE 2: a 69-year-old woman underwent a diagnostic lumbar puncture for the study of recent-onset headache with tinnitus and unstable gait. Puncture was traumatic and cerebrospinal fluid was normal. A few hours later, she complained of back pain and sciatica, and examination revealed a bilateral Lassegue sign. She required urinary catheterization. MRI showed an epidural hematoma from the T10 vertebra to the sacral area. Her outcome with a conservative approach was also excellent. CONCLUSIONS: Spinal cord hematomas can occur after a traumatic lumbar puncture in people without coagulation disorders or any other predisposing factor. Although surgery has been traditionally advocated in these cases, a conservative approach is an option when symptoms are mild and a close follow-up is possible.