Spinal root arteriovenous malformations and same-segment cord cavernous malformation in familial cerebral cavernous malformation

Spinal vascular malformations are uncommon lesions, and controversy persists regarding optimal investigation, classification, and treatment strategies. The authors report on a patient with a spinal root arteriovenous malformation (AVM) associated with a parenchymal cavernous malformation (CM) in the same spinal cord segment and describe a complete familial and molecular investigation. This 35-year-old woman presented with symptoms of progressive clinical spastic paraparesis. Magnetic resonance imaging results were suggestive of a spinal cord cavernoma associated with cerebral CMs. Her family history included 2 sisters treated for epilepsy. At surgery an intraspinal root AVM was found at the same level of the cord CM, and both lesions were completely removed. Cerebral gradient echo MR imaging disclosed multiple cavernomas in her relatives, which prompted the molecular diagnosis. On sequence analysis, a novel mutation on the cerebral CM1 (CCM1) gene (c796insA) was found. The authors report on a unique case of familial cerebral CM in which a spinal root AVM was situated next to a cord CM, and discuss the concomitant occurrence of altered nervous system angiogenesis and vasculogenesis.

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Anterior thoracic intradural arachnoid cysts

Neurosurgical FOCUS ◽

10.3171/foc.1999.6.5.11 ◽

1999 ◽

Vol 6 (5) ◽

pp. E10 ◽

Cited By ~ 2

Author(s):

Gerardo Caruso ◽

Antonino Germanò ◽

Mariella Caffo ◽

Massimo Belvedere ◽

Giovanni La Rosa ◽

...

Keyword(s):

Spinal Cord ◽

Treatment Options ◽

Relevant Literature ◽

Spastic Paraparesis ◽

Treatment Strategies ◽

Arachnoid Cysts ◽

Complete Excision ◽

Medline Search ◽

Excellent Outcome ◽

Intradural Arachnoid Cysts

Anterior thoracic intradural arachnoid cysts (ATIACs) are a rare cause of spinal cord and nerve root compression, for which different treatment strategies have been proposed. Although ATIAC represents a well-known clinical entity, the choice of surgical method has not been uniform, and no study has been specifically designed to compare the results of the different treatment options adopted. The authors report the case of a 40-year old man with a 1-year history of dorsal pain, weakness in the lower extremities, gait disturbance, and mild sexual and urinary dysfunction. On neurological examination spastic paraparesis, lower-extremity hypertonia, and hypesthesia below T-2 were demonstrated. Magnetic resonance imaging revealed the presence of an ATIAC at the T-2 level. The patient underwent complete microsurgical removal of the cyst. The authors conducted a Medline search of the relevant literature from 1966 to 1998 and also obtained data on other cases in which patients underwent surgical treatment of ATIAC. The literature search yielded five such cases. Treatment strategies were complete excision and fenestration followed by placement of a shunt. In addition, one case was characterized by intraoperative cyst rupture during retraction of the spinal cord. Correct preoperative workup coupled with microneurosurgical technique allow for successful removal of the lesion and excellent outcome. Based on the literature review and the results in our case, the complete excision of ATIAC is associated with an excellent outcome, which is different from results achieved using other surgical strategies.

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Brainstem cavernous malformation

Arquivos Brasileiros de Neurocirurgia Brazilian Neurosurgery ◽

10.1055/s-0038-1625933 ◽

2013 ◽

Vol 32 (01) ◽

pp. 31-36

Author(s):

Ariel Roberto Estramiana ◽

Diana Lara Pinto de Santana ◽

Eberval Gadelha Figueiredo ◽

Manoel Jacobsen Teixeira

Keyword(s):

Magnetic Resonance Imaging ◽

Spinal Cord ◽

Central Nervous System ◽

Clinical Presentation ◽

Cavernous Malformation ◽

Vascular Malformations ◽

Resonance Imaging ◽

The Central Nervous System ◽

Magnetic Resonance Imaging Mri ◽

Brainstem Cavernous Malformation

AbstractCavernous malformation (CM) of the central nervous system (CNS) are acquired or developmental vascular malformations that represent the 5% to 15% of all vascular malformations of the CNS. Eighty to ninety percent of CM are supratentorial, 15% infratentorial, and 5% occur in the spinal cord. The subset of brainstem malformation presents as a very difficult paradigm for treating clinicians. The widespread use of magnetic resonance imaging (MRI) has increased the recognition of this disease. Clinical presentation, pathophysiology and treatment are discussed in this article.

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Motor Recovery after Chronic Spinal Cord Transection in Rats: A Proof-of-Concept Study Evaluating a Combined Strategy

CNS & Neurological Disorders - Drug Targets ◽

10.2174/1871527317666181105101756 ◽

2019 ◽

Vol 18 (1) ◽

pp. 52-62 ◽

Cited By ~ 1

Author(s):

Antonio Ibarra ◽

Erika Mendieta-Arbesú ◽

Paola Suarez-Meade ◽

Elisa García-Vences ◽

Susana Martiñón ◽

...

Keyword(s):

Spinal Cord ◽

Treatment Strategies ◽

Chronic Phase ◽

Motor Recovery ◽

Neural Regeneration ◽

Histological Evaluation ◽

Control Group ◽

Proof Of Concept ◽

Sprague Dawley ◽

Concept Study

Background: The chronic phase of Spinal Cord (SC) injury is characterized by the presence of a hostile microenvironment that causes low activity and a progressive decline in neurological function; this phase is non-compatible with regeneration. Several treatment strategies have been investigated in chronic SC injury with no satisfactory results. OBJECTIVE- In this proof-of-concept study, we designed a combination therapy (Comb Tx) consisting of surgical glial scar removal plus scar inhibition, accompanied with implantation of mesenchymal stem cells (MSC), and immunization with neural-derived peptides (INDP). Methods: This study was divided into three subsets, all in which Sprague Dawley rats were subjected to a complete SC transection. Sixty days after injury, animals were randomly allocated into two groups for therapeutic intervention: control group and animals receiving the Comb-Tx. Sixty-three days after treatment we carried out experiments analyzing motor recovery, presence of somatosensory evoked potentials, neural regeneration-related genes, and histological evaluation of serotoninergic fibers. Results: Comb-Tx induced a significant locomotor and electrophysiological recovery. An increase in the expression of regeneration-associated genes and the percentage of 5-HT+ fibers was noted at the caudal stump of the SC of animals receiving the Comb-Tx. There was a significant correlation of locomotor recovery with positive electrophysiological activity, expression of GAP43, and percentage of 5-HT+ fibers. Conclusion: Comb-Tx promotes motor and electrophysiological recovery in the chronic phase of SC injury subsequent to a complete transection. Likewise, it is capable of inducing the permissive microenvironment to promote axonal regeneration.

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Changes of Dorsal Root Ganglion Volume in Dogs with Clinical Signs of Degenerative Myelopathy Detected by Water-Excitation Magnetic Resonance Imaging

Animals ◽

10.3390/ani11061702 ◽

2021 ◽

Vol 11 (6) ◽

pp. 1702

Author(s):

Eiji Naito ◽

Kohei Nakata ◽

Yukiko Nakano ◽

Yuta Nozue ◽

Shintaro Kimura ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Spinal Cord ◽

Magnetic Resonance ◽

Clinical Signs ◽

Intervertebral Disc Herniation ◽

Resonance Imaging ◽

Diagnostic Potential ◽

Water Excitation ◽

Spinal Cord Segment ◽

Degenerative Myelopathy

Canine degenerative myelopathy (DM) is a progressive and fatal neurodegenerative disease. However, a definitive diagnosis of DM can only be achieved by postmortem histopathological examination of the spinal cord. The purpose of this study was to investigate whether the volumetry of DRG using the ability of water-excitation magnetic resonance imaging (MRI) to visualize the DRG in dogs has premortem diagnostic value for DM. Eight dogs with DM, twenty-four dogs with intervertebral disc herniation (IVDH), and eight control dogs were scanned using a 3.0-tesla MRI system, and water-excitation images were obtained to visualize and measure the volume of DRG, normalized by body surface area. The normalized mean DRG volume between each spinal cord segment and mean volume of all DRG between T8 and L2 in the DM group was significantly lower than that in the control and the IVDH groups (P = 0.011, P = 0.002, respectively). There were no correlations within the normalized mean DRG volume between DM stage 1 and stage 4 (rs = 0.312, P = 0.128, respectively). In conclusion, DRG volumetry by the water-excitation MRI provides a non-invasive and quantitative assessment of neurodegeneration in DRG and may have diagnostic potential for DM.

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The Histopathology of Severe Graded Compression in Lower Thoracic Spinal Cord Segment of Rat, Evaluated at Late Post-injury Phase

Cellular and Molecular Neurobiology ◽

10.1007/s10571-021-01139-7 ◽

2021 ◽

Author(s):

Fedorova Jana ◽

Kellerova Erika ◽

Bimbova Katarina ◽

Pavel Jaroslav

Keyword(s):

Spinal Cord ◽

Spontaneous Recovery ◽

Traumatic Injury ◽

Blue Dye ◽

Post Injury ◽

Thoracic Spinal Cord ◽

Thoracic Spinal ◽

Severe Traumatic Injury ◽

Spinal Cord Segment ◽

Cord Injury

AbstractSpontaneous recovery of lost motor functions is relative fast in rodent models after inducing a very mild/moderate spinal cord injury (SCI), and this may complicate a reliable evaluation of the effectiveness of potential therapy. Therefore, a severe graded (30 g, 40 g and 50 g) weight-compression SCI at the Th9 spinal segment, involving an acute mechanical impact followed by 15 min of persistent compression, was studied in adult female Wistar rats. Functional parameters, such as spontaneous recovery of motor hind limb and bladder emptying function, and the presence of hematuria were evaluated within 28 days of the post-traumatic period. The disruption of the blood-spinal cord barrier, measured by extravasated Evans Blue dye, was examined 24 h after the SCI, when maximum permeability occurs. At the end of the survival period, the degradation of gray and white matter associated with the formation of cystic cavities, and quantitative changes of glial structural proteins, such as GFAP, and integral components of axonal architecture, such as neurofilaments and myelin basic protein, were evaluated in the lesioned area of the spinal cord. Based on these functional and histological parameters, and taking the animal’s welfare into account, the 40 g weight can be considered as an upper limit for severe traumatic injury in this compression model.

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Treatment options and long-term outcomes in pediatric spinal cord vascular malformations: a case report and review of the literature

Child s Nervous System ◽

10.1007/s00381-020-04624-4 ◽

2020 ◽

Vol 36 (12) ◽

pp. 3147-3152

Author(s):

Helen J. Zhang ◽

Nicole Silva ◽

Elena Solli ◽

Amanda C. Ayala ◽

Luke Tomycz ◽

...

Keyword(s):

Spinal Cord ◽

Case Report ◽

Vascular Malformations ◽

Treatment Options ◽

Review Of The Literature ◽

Long Term Outcomes

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Evaluation of deformable image registration (DIR) methods for dose accumulation in nasopharyngeal cancer patients during radiotherapy

Radiology and Oncology ◽

10.1515/raon-2017-0033 ◽

2017 ◽

Vol 51 (4) ◽

pp. 438-446 ◽

Cited By ~ 5

Author(s):

Wannapha Nobnop ◽

Imjai Chitapanarux ◽

Hudsaleark Neamin ◽

Somsak Wanwilairat ◽

Vicharn Lorvidhaya ◽

...

Keyword(s):

Spinal Cord ◽

Image Registration ◽

Treatment Plan ◽

Nasopharyngeal Cancer ◽

Treatment Strategies ◽

Deformable Image Registration ◽

Organ Dose ◽

Target Dose ◽

Dose Accumulation ◽

The Impact

Abstract Introduction Deformable image registration (DIR) is used to modify structures according to anatomical changes for observing the dosimetric effect. In this study, megavoltage computed tomography (MVCT) images were used to generate cumulative doses for nasopharyngeal cancer (NPC) patients by various DIR methods. The performance of the multiple DIR methods was analysed, and the impact of dose accumulation was assessed. Patients and methods The study consisted of five NPC patients treated with a helical tomotherapy unit. The weekly MVCT images at the 1st, 6th, 11th, 16th, 21st, 26th, and 31st fractions were used to assess the dose accumulation by the four DIR methods. The cumulative dose deviations from the initial treatment plan were analysed, and correlations of these variations with the anatomic changes and DIR methods were explored. Results The target dose received a slightly different result from the initial plan at the end of the treatment. The organ dose differences increased as the treatment progressed to 6.8% (range: 2.2 to 10.9%), 15.2% (range: -1.7 to 36.3%), and 6.4% (range: -1.6 to 13.2%) for the right parotid, the left parotid, and the spinal cord, respectively. The mean uncertainty values to estimate the accumulated doses for all the DIR methods were 0.21 ± 0.11 Gy (target dose), 1.99 ± 0.76 Gy (right parotid), 1.19 ± 0.24 Gy (left parotid), and 0.41 ± 0.04 Gy (spinal cord). Conclusions Accuracy of the DIR methods affects the estimation of dose accumulation on both the target dose and the organ dose. The DIR methods provide an adequate dose estimation technique for observation as a result of inter-fractional anatomic changes and are beneficial for adaptive treatment strategies.

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Cervical Perimedullary Arteriovenous Shunts

Neurosurgery ◽

10.1227/neu.0000000000000401 ◽

2014 ◽

Vol 75 (3) ◽

pp. 238-249 ◽

Cited By ~ 14

Author(s):

Toshiki Endo ◽

Hiroaki Shimizu ◽

Kenichi Sato ◽

Kuniyasu Niizuma ◽

Ryushi Kondo ◽

...

Keyword(s):

Spinal Cord ◽

Anterior Approach ◽

Open Surgery ◽

Treatment Strategies ◽

Good Recovery ◽

Intraoperative Angiography ◽

Indocyanine Green Videoangiography ◽

Arteriovenous Shunts ◽

Complete Obliteration

Abstract BACKGROUND: Reports of cervical perimedullary arteriovenous shunt (PMAVS) are limited, and treatment strategies have not been established. OBJECTIVE: To describe angioarchitecture and optimal treatment strategies for cervical PMAVS. METHODS: We treated 22 patients with cervical PMAVS between 2000 and 2012 (8 women and 14 men; age, 9-80 years). According to the classification, our patients included type IVa (4 patients), type IVb (16 patients), and type IVc (2 patients). Seventeen patients presented with subarachnoid hemorrhage. RESULTS: A total of 41 shunting points were localized in 22 patients, of which 34 points were located ventral or ventrolateral to the spinal cord. The anterior spinal artery (ASA) contributed to the shunts in 16 patients. Aneurysm formation was identified in 8 patients. Endovascular treatment was attempted in 3 patients, resulting in complete obliteration in 1 patient (type IVc). Overall, 21 patients underwent open surgery. An anterior approach with corpectomy was elected for 2 patients; the other 19 patients underwent the posterior approaches using indocyanine green videoangiography, intraoperative angiography, endoscopy (8 patients), and neuromonitoring. Twenty patients were rated as having a good recovery at 6 months after surgery. No recurrence was observed in any patients during the follow-up (mean, 59.7 months). CONCLUSION: Shunting points of the cervical PMAVS were predominantly located ventral or ventrolateral to the spinal cord and were often fed by the ASA. Even for ventral lesions, posterior exposure assisted with neuromonitoring and endoscopy, and intraoperative angiography provided a view sufficient to understand the relationships between the shunts and the ASA and contributed to good surgical outcomes.

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