Cervicomedullary Junction Research Articles

Scoliosis in patients with Chiari malformation type I.

The literature about the association between Chiari malformations (CMs) and scoliosis has been growing over the last three decades; yet, no consensus on the optimal management approach in this patient population has been reached. Spinal anomalies such as isolated syrinxes, isolated CM, and CM with a syrinx are relatively common among patients with presumed idiopathic scoliosis (IS), a rule that also applies to scoliosis among CM patients as well. In CM patients, scoliosis presents with atypical features such as early onset, left apical or kyphotic curvature, and neurological deficits. While spinal X-rays are essential to confirm the diagnosis of scoliosis among CM patients, a magnetic resonance imaging (MRI) is also recommended in IS patients with atypical presentations. Hypotheses attempting to explain the occurrence of scoliosis in CM patients include cerebellar tonsillar compression of the cervicomedullary junction and uneven expansion of a syrinx in the horizontal plane of the spinal cord. Early detection of scoliosis on routine spinal examination and close follow-up on curve stability and progression are essential initial steps in the management of scoliosis, especially in patients with CM, who may require full spine MRI to screen for associated neuro-axial anomalies; bracing and spinal fusion may be subsequently pursued in high-risk patients.

Clinical profile of patients with anomalies of the Craniovertebral junction

The Craniovertebral (or Craniocervical) junction (CVJ) is a collective term that refers to the occiput (posterior skull base), atlas, axis, and supporting ligaments. It encloses the soft tissue structures of the Cervicomedullary junction (medulla, spinal cord, and lower cranial nerves) and is visible in magnetic resonance (MR) imaging studies of the brain. The craniocervical junction is a complex articulation between occiput, atlas and axis is an important and often poorly understood anatomic area. It is a potential site of variety of radiologic diagnosis and misdiagnosis. Patients with a clinical suspicion of CVJ abnormalities will be evaluated by CT and Magnetic Resonance Imaging during the course of the study. No selection bias will be exercised in terms of patients' age, sex and pathology. Imaging characteristics of radiological modalities like CT and MRI will be recorded. Final diagnosis will also be noted. The results will be analyzed and studied. Combination of OA+AAD was seen in 20% patients. Combination of BI+OA was seen in 14.2% and BI +OA+AAD was seen in 7.1% patients and meant a localized congenital affection affecting both atlas and basiocciput. ACM I and Syringomyelia are the commonest neural anomalies associated with bony CVJ anomalies.

Clinical, neuroimaging and therapeutic response in AQP4-positive NMO patients from India

BackgroundNeuromyelitis Optica (NMO) is an autoimmune astrocytopathic disorder due to AQP4 antibodies. ObjectivesTo analyse clinical, neuroimaging features in NMO patients and assess the efficacy of various therapeutics. MethodsAQP4+ve NMO patients were diagnosed based on consensus diagnostic criteria. Results101 AQP4+ve NMO patients were seen with female (90) predominance. Adult population (71.3%) formed the larger group followed by pediatric (19.8%) and late-onset (8.9%). Myelopathy (36.2%) was most commonly seen followed by optic neuritis (19.1%), brainstem (17.1%), opticomyelopathy (16.1%), area postrema involvement (10.5%) and encephalopathy (1%). Encephalopathy and brainstem/cerebellar involvement were most common in pediatric population while opticomyelopathy was more common in late-onset patients. Hyperintensities of lower medulla was seen in 67.3% subjects and 49.5% had involvement of obex. Differential T2 hyperintensity of the long segment myelitis was found in 30.7%. Plasmapheresis was given in 71 subjects followed by maintenance therapy. Most of them showed significant improvement with EDSS score of 1 in 30.7%. ConclusionsClinical manifestations in AQP4+ve NMO patients may vary depending on the age at onset of illness. MRI features affecting cervicomedullary junction, obex, differential T2 hyperintensities of the spinal cord may form a useful diagnostic clue. Plasmapheresis is helpful in achieving remission along with immunomodulation.

Abstract TP475: A Case of Suspected Vertebral Giant Cell Arteritis Causing Cervico-medullary Ischemia

Objective: To present a rare case of cervico-medullary ischemia suspected to be from giant cell arteritis (GCA). Background: In patients with GCA, vertebral artery involvement occurs in 3-4% of ischemic events and manifests by disturbances, cranial nerve palsies, cerebellar signs, weakness and altered consciousness (1,2). Design: Case Report. Results: A 79-year-old man presented with 2-week history of vertigo right-sided weakness, right-sided neck pain and occipital headaches with magnetic resonance imaging (MRI) brain and cervical spine without contrast showing right distal medulla and cervico-medullary junction infarct and magnetic resonance angiogram (MRA) neck showing bilaterally diminutive/atherosclerotic vertebral arteries with absence of flow in right vertebral C2 segment. Relevant tests showed an elevated white cell count of 14 K/mm 3 , C-reactive protein (CRP) of 171 mg/L, erythrocyte sedimentation rate (ESR) of 67 mm/hour, antinuclear antibodies (ANA) of 1: 1280 and unrevealing Reichlin profile, temporal artery ultrasound, carotid duplex, PET/CT and cerebrospinal fluid infectious workup. After initiation of Prednisolone 60 mg PO daily, patient reported improvement in pain and levels of inflammatory markers decreased. As patient was on Clopidogrel, temporal artery biopsy (TAB) was delayed and performed 14 days after initiation of steroids and showed no signs of inflammation. Patient is being planned for gradual steroid taper and monitoring of response. Conclusion: Vertebral GCA is a high fatality condition. A combination of persistently elevated inflammatory markers, unusual stroke location, bilateral vertebral artery disease with multiple areas of stenosis, improvement in weakness and pain after initiation of steroids were all highly suggestive of GCA vasculitic process. High index of suspicion is crucial as specific and curative treatment exists. In select cases where TAB is delayed, treatment should not be withheld.

Cranio-vertebral junction anomaly –presenting as cervical myelopathy

Antlanto-axial dislocation with blocked and ankylosed C2-4 vertebrae causing severe canal stenosis with compression of cervico-medullary junction has been reported. The knowledge of such anomaly may be importance for radiologists, anaesthesiologists, orthopaedicians and neurosurgeons because cranio-cervical junction anomaly may result in sudden unexpected death. It can also result in dysphagia, dysarthria due to compression of cranial nerves and quadriparesis due to compression of spinal cord. We here report a rare case of cranio-vertebral junction anomaly with blocked and ankylosed C2,3,4vertebrae with evidence of atlanto-axial dislocation causing compression of cervico-medullary junction and leading to cervical myelopathy.International Journal of Human and Health Sciences Vol. 03 No. 01 January’19. Page : 40-42

Refinement of the Transoral Approach to Craniovertebral Junction Malformations.

The transoral approach provides the most direct surgical corridor for treatment of congenital bony abnormalities that exert irreducible ventral compression of the cervicomedullary junction. In this paper, based on our experience with the transoral approach over the past three decades, we briefly describe the surgical strategies and the operative nuances that allow effective decompression of the craniovertebral junction (CVJ) while minimizing postoperative morbidity. The surgical strategy is dictated by the type and severity of the malformation. Fibre-optic nasotracheal intubation obviates the necessity of preoperative tracheostomy, and avoidance of a soft-palate incision significantly reduces oropharyngeal morbidity. When feasible, the atlas-sparing technique minimizes postoperative instability. The transoral transatlas approach is generally required in patients with severe basilar invagination and allows wider exposure of the anterior CVJ at the price of a higher incidence of postoperative instability. The transoral approach is extremely effective in providing excellent decompression of the anterior cervicomedullary junction in patients with fixed malformations. Tailoring the approach to the peculiar anatomy of each malformation reduces iatrogenic instability and minimizes postoperative complications.

Traumatic Posterior Atlantoaxial Dislocation without Related Fractures of C1 - C2 in a Patient with Traumatic Brain Injury: Case Report and Literature Review

It is exceptionally rare to have posterior atlanto axial dislocation without an associated atlas or odontoid fracture and very few cases have been reported in literature. We present a case of a 50 year old female pedestrian who was hit by car while crossing a road. She presented with a depressed level of consciousness and the Glasgow coma score of 12/15, pupils were equal and reactive to light and all limbs were moving equally. She had an extensive abrasion on the forehead which extended into scalp. CT scan showed a posterior dislocation of the atlas with respect of axis with no associated fractures of C 1 and C2. She had traumatic subarachnoid hemorrhage in the basal cisterns and multiple brainstem contusions. Closed reduction of the dislocation was done successfully under fluoroscopy guidance. MRI was done post reduction because funds were not available initially and it showed increased signal in the tectorial membrane and transverse and alar ligaments with mild cord oedema at the cervicomedullary junction. However, the patient post reduction began to deteriorate and eventually passed away before definitive treatment of her cervical-spine injury. The case is reported in view of its rarity and to review literature on this uncommon condition.

Surgical, clinical, and radiological outcomes of occipitocervical fusion using the plate-screw-rod system with allograft in craniocervical instability.

Objective:We evaluated surgical, clinical, and radiological outcomes of posterior occipitocervical fusion (OCF) using plate–rod–screw construct supplemented with allograft in cases of occipitocervical instability.Study Design:This was a retrospective analysis of prospective collected data.Methods:Data of 52 patients who underwent posterior OCF using plate–screw–rod construct supplemented with allograft at a single institute from 2009 to 2014 were analyzed. Demographics, clinical parameters (Visual Analog Score [VAS], ODI, and mJOA score), functional status (McCormick scale), radiological parameters – mean atlantodens interval, posterior occipitocervical angle, occipitocervical 2 angle, and surgical parameters (operative time, blood loss, hospital stay, and fusion) with complications were evaluated.Results:The mean age of the patients was 54.56 ± 16.21 years with male: female was 28:24. The mean operative time was 142.2 min (90–185 min) and mean blood loss was 250.8 ml. The mean duration of hospital stay was 6.7 days and mean follow-up period was 65.17 ± 5.39 months. There was significant improvement in clinical parameters (modified JOA score, VAS, and Oswestry Disability Index values) postoperatively. Forty patients showed recovery in neurological status at least in Grade 1 in McCormick scale with no neurological deterioration in any patient. Furthermore, radiological parameters at cervicomedullary junction got into acceptable range. Implant-related complications noted in 1 patient and 1 patient had vertebral artery injury. We had dural tear in 3 patients and infection in 2 patients. Fusion was achieved in 46 cases with mean time for fusion was 11.039 months.Conclusion:Patients with occipitocervical instability can successfully undergo posterior OCF using plate–screw–rod construct supplemented with allograft with high fusion rate, good clinical and functional outcomes, and low complication rate.

Endoscopic single stage trans-oral decompression and anterior C1 lateral mass and C2 pedicle stabilization for atlanto-axial dislocation.

Although most of the cases of atlanto-axial dislocation (AAD) and basilar invasion can be managed by posterior approaches in the recent times, anterior decompression with stabilization is required in selected patients who persist with irreducible AAD even after manipulation of the C1-C2 facet joint under general anesthesia. A single stage endoscopic trans-oral decompression and stabilization can be used in such patients. It has not been described so far to the best of authors' knowledge. This is indicated in irreducible AAD with the mandibular angle lying below the C2-C3 disc space. It is not a proper choice when the mandibular angle is above the C2-C3 disc space, there is involvement of the facet joint by trauma or any other pathologies, and if a posterior compression at the cervicomedullary junction persists. All patients should undergo pre-operative radiographs, computed tomography (CT) scan and magnetic resonance imaging with angiogram of the cranio-vertebral region. Utilizing this technique, an intra-operative satisfactory reduction of the dislocation with C1-C2 stabilization could be achieved in 3 patients, and 7 required an additional odontoid excision. Post- operative plain radiographs should be performed to assess for C1- C2 alignment and fusion at 3 and 12 months after surgery. All 10 patients of our series had an irreducible AAD and two had an additional basilar invasion. All patients improved from the pre-operative Ranawat grade 3A (n = 8) and 3B (n = 2) to post-operative grade 1 (n = 9) and 2 (n = 1) at a 3-12- month follow-up assessment. The average duration of the procedure and blood loss was 145 minutes and 75 ml, respectively. Endoscopic trans-oral single stage decompression and stabilization seems to be an effective and safe alternative in selected patients with AAD and basilar invasion.

Full-endoscopic Uniportal Odontoidectomy and Decompression of the Anterior Cervicomedullary Junction Using the Retropharyngeal Approach.

A study of a series of consecutive full-endoscopic uniportal decompressions of the anterior craniocervical junction with retropharyngeal approach. The aim of this study was to evaluate the direct anterior decompression of the craniocervical junction in patients with bulbomedullary compression using a full-endoscopic uniportal technique via an anterolateral retropharyngeal approach. Acute or progressive myelopathy may necessitate direct anterior decompression of the craniocervical junction and odontoidectomy. Different techniques with individual advantages and disadvantages can be used. In addition to the gold standard - the transoral approach - there is increasing experience with the endoscopic transnasal technique. Other alternative procedures are also being developed. Between 2013 and 2016, eight patients with basilar impression, retrodental pannus, or retrodental infection were operated in the full-endoscopic uniportal technique with a retropharyngeal approach. Anterior decompression of the bulbomedullary junction with odontoidectomy was performed. All patients additionally underwent posterior stabilization. Imaging and clinical data were collected in follow-up examinations for 1 year. The bulbomedullary junction was adequately decompressed. No problems due to swelling of pharyngeal soft tissue occurred. One patient required revision due to secondary bleeding. No other complications were observed. All patients had a good clinical outcome with stable regression of the myelopathy symptoms and/or healing of the infection. The imaging follow-up showed sufficient decompression of bone and soft tissue in all cases. No evidence was found of increasing instability or failure of posterior fusion. In the operated patients, the full-endoscopic uniportal surgical technique with anterior retropharyngeal approach was found to be a sufficient and minimally invasive method with the known advantages of an endoscopic procedure under continuous irrigation. It should not be viewed only as competition for other surgical techniques - due to its individual technical parameters, it can also be considered to be an alternative or complementary procedure. 4.

Endoscopic Endonasal Approach to the Upper Cervical Spine for Decompression of the Cervicomedullary Junction Following Occipitocervical Fusion.

Basilar invagination is defined as abnormal upward and/or posterior displacement of the odontoid leading to ventral compression of the cervicomedullary junction. This condition leads to lower cranial neuropathies, sensorimotor deficits, and myelopathy. These symptoms can persist even after posterior decompression, which is an indication for ventral decompression. Transoral approaches to the upper cervical spine carry significant morbidity, limiting their utility. The endonasal approach to the upper cervical spine presents an alternative for patients with amenable anatomy. In this report, we present a case of a patient with type 1 Chiari malformation with persistent symptoms despite adequate posterior decompression through suboccipital craniectomy and C1 laminectomy. A retroflexed odontoid and dorsal clival bone lip contributed to persistent cervicomedullary compression. To address this, we performed a 2-stage procedure: an occiput-to-C4 fusion followed by endoscopic endonasal approach for dorsal clivusectomy, C1 anterior arch resection, and odontoidectomy. In the associated video, Supplemental Digital Content 1 (http://links.lww.com/CLINSPINE/A52), we demonstrate the step-by-step approach for this anterior approach including positioning, dissection through the nasopharyngeal fascia, identification of bony landmarks using an intraoperative CT scanner with 3-dimensional navigation guidance, and drilling/bony decompression of the dorsal clivus, C1, and C2. We also discuss key pearls, pitfalls, and preoperative/postoperative considerations critical to successful outcomes.

Place de la TMS pour évaluer l’activation volontaire corticale liée à l’exercice

While Transcranial magnetic stimulation (TMS) was initially used in upper limbs, the cortical representation of the lower limbs can be stimulated with a double-cone coil. It is thus possible to use TMS to study changes that occur within the central nervous system during all types of exercise. For instance, TMS can be used to localise the site of central fatigue (i.e., a decrease in voluntary activation, VA) as traditionally measured using the interpolated twitch technique even though calculating VATMS is more complicated than calculating VA with motor nerve stimulation [3] . A suboptimal cortical VA (VATMS) indicates that the output from the motor cortex is not driving the muscle maximally at the time of stimulation (e.g. [2] ). Yet, an exercise-induced decrease in VATMS does not mean that the origin is solely supraspinal i.e., occurring at or upstream of the motor cortex, and deficits may still occur at the spinal level. Single-pulse TMS of sufficient intensity stimulates the axons of both pyramidal neurons and (excitatory and inhibitory) cortical interneurons within the cortical area under the site of stimulation. The volley of excitation travels down the corticospinal pathway and can be recorded in a target muscle using surface EMG. The motor evoked potential (MEP) can be used as a quantitative marker for state-specific changes in corticospinal excitability, provided it is normalised to the maximal M-wave as exercise can change M-wave characteristics. In order to differentiate spinal and cortical changes, corticospinal axons can be stimulated at the level of the cervicomedullary junction to measure motoneuronal excitability [1] . These methods will be illustrated in the context of acute (e.g., fatigue) and chronic (training) changes due to exercise. The challenge of using TMS for dynamic whole-body exercise will also be discussed.

Safety of Dynamic Magnetic Resonance Imaging of the Cervical Spine in Children Performed without Neurosurgical Supervision

The need for neurosurgical supervision as well as the general safety and utility of dynamic magnetic resonance imaging (MRI) of the cervical spine in children remains controversial. We present the largest descriptive cohort study of cervical flexion-extension MRI scans in pediatric patients to help elucidate the safety and utility of this technique. We retrospectively reviewed all cervical spine MRI scans performed at Lucile Packard Children's Hospital at Stanford from 2009 to 2015. We identified 66 dynamic cervical MRI scans performed in 45 children and 2young adults for further study. General anesthesia was used in 43 scans. The neuroradiology team performed all scans with no direct supervision by the neurosurgery team. There were no adverse events. Dynamic MRI detected significant instability that was not clearly seen on dynamic radiographs (5patients) and cord compression not seen on static MRI (9 patients). One patient with asymptomatic instability found on flexion-extension radiographs had no cord compression with movement on MRI and was managed conservatively. Two neonates with significant congenital malformations of the cervical spine were cleared for operative positioning for cardiac procedures based on flexion-extension MRI. Dynamic MRI is a safe tool for evaluating the cervical spine and cervicomedullary junction in various pediatric populations and can be performed safely without direct neurosurgical supervision. We describe for the first time the use of flexion-extension MRI to clear neonates with severe congenital cervical spine abnormalities for complex operative positioning and further care in the intensive care unit.

Evaluation of Soft Tissue Hypertrophy at the Retro-Odontoid Space in Patients with Chiari Malformation Type I on Magnetic Resonance Imaging

Chiari malformation type I (CM-I) is a well-known hindbrain disorder in which the cerebellar tonsils protrude through the foramen magnum. The soft tissues, including the transverse ligament and the tectorial membrane at the retro-odontoid space, can compress the cervicomedullary junction if they become hypertrophic. Twenty-two symptomatic patients with CM-I (aged 5-19 years) were treated between 2007 and 2017 at our institute. The retro-odontoid soft tissue was evaluated using T2-weighted magnetic resonance imaging. Anteroposterior (AP) distances and craniocaudal distances of the soft tissue were measured in patients with CM-I and 48 normal control children. Modified clivoaxial angles were also evaluated as the index of ventral compression of the cervicomedullary junction. Of the 18 patients treated with foramen magnum decompression, 16 patients improved postoperatively, whereas the condition of 2 remained unchanged. The AP distances in the CM-I group (6.0 mm) were significantly larger than those in the control group (3.5 mm), whereas there were no apparent differences in the craniocaudal distances. Modified clivoaxial angles were obviously smaller in the CM-I group (131.5°) than in the control group (146.9°). Moreover, the AP distances were significantly reduced postoperatively (5.5 mm), although the other parameters did not change significantly. The retro-odontoid soft tissue in symptomatic patients with CM-I can be hypertrophic enough to compress the cervicomedullary junction ventrally even if there are no combined osseous anomalies. Foramen magnum decompression works to reduce the hypertrophic changes significantly, suggesting that downward tonsil movement might participate in hypertrophic soft tissue formation at the retro-odontoid space.

“Sandwich Deformity” in Klippel-Feil syndrome: A “Full-Spectrum” presentation of associated craniovertebral junction abnormalities

Klippel-Feil syndrome (KFS) is defined as congenital fusion of two or more cervical vertebrae resulting from a segmentation failure in the developing spine. According to Samartzis et al., the most commonly fused segments are found at C2/3 (74.1%) and C6/7 (70.4%). In patients with C2/3 fusion, especially when there is additional C1 occipitalization, several secondary anomalies including atlantoaxial dislocation (AAD), basilar invagination (BI), Chiari malformation, and syringomyelia can be identified.In this report, we present a case of a 12-year-old patient with C2/3 and occipitalization and a “Full-Spectrum” presentation of associated CVJ abnormalities including C0/1 fusion, AAD, BI, Chiari malformation, syringomyelia, myelopathy and cranial neuropathy received neurological decompression of the cervico-medullary junction by posterior reduction of the AAD and reconstruction of her CVJ using an unconventional hybrid construct due to a high-riding right vertebral artery in C2. To our knowledge, her “Full-Spectrum” presentation may include the most categories of concomitant abnormalities in the literature. In addition, She received neurological decompression of the cervico-medullary junction using an unconventional hybrid construct due to a high-riding vertebral artery in C2.Three months after the surgery, all of her symptoms recovered significantly. Neither Chiari malformation nor syringomyelia could be identified by MRI two years after the surgery. At the last follow-up (4 years), the patient became completely asymptomatic.

T119. Strength of peripheral nerve stimulation impacts on the magnitude of synaptic plasticity induced at spinal motoneurons

Paired-associative stimulation (PAS), repeated transcranial magnetic stimulation (TMS) pairing with electrical peripheral nerve stimulation (ES), is one method of synaptic plasticity induction. If these two stimuli are delivered at the timing that descending volleys overlap with peripheral inputs at the spinal motoneurons (SM), long-term potentiation (LTP) like plasticity is induced at the SM synapses (SM-PAS). Here we investigated influences of stimulus intensity of ES on the degree of plasticity. Eight right-handed healthy volunteers participated in this study. Electromyograms were recorded from right first dorsal interosseous muscle (FDI). Peripheral ES was delivered on right ulnar nerve at the wrist. TMS was applied over the left primary motor cortex (M1) hand area, and stimulus intensity was set at 120% resting motor threshold of the right FDI. We measured motor-evoked potentials (MEP) and F-wave latencies in each subject. As for SM-PAS, TMS and ES were delivered at the timing that antidromic volley derived by ES arrives at the postsynaptic terminals 1 ms before an initial descending volley evoked by TMS reaches the presynaptic terminals. A stimulus pairing of TMS with ES was given every 5 s (0.2 Hz) for 15 min (total of 180 pairs). Three different ES intensities were delivered at supramaximal intensity, at three times the sensory threshold (with muscle twitch), and at two times the sensory threshold (without muscle twitch). For evaluating SM synaptic excitability, single-pulse TMS was delivered at cervicomedullary junction level during brief contraction of the right FDI, and cervicomedullary MEP (CMEP) was recorded before and 1 min and every 10 min from 10 to 60 min after the SM-PAS intervention. CMEP increased after SM-PAS at supramaximal ES and at three times the sensory threshold, while SM-PAS at two times the sensory threshold induced no CMEP changes. SM-PAS with supramaximal ES was most effective. SM-PAS at three times the sensory threshold induces sensory-motor associative plasticity constituted of sensory afferent inputs with multiple corticospinal descending volleys. Supramaximal ES stimulates both sensory afferent fibers and motor neuron axons antidromically, and SM-PAS may induce both sensory afferent and motor antidromic input associative plasticity. Antidromic inputs driven by supramaximal ES may augment associative synaptic plasticity at spinal motoneurons.

Bell’s Cruciate paralysis in a patient victim of a gunshot wound in the cervical region

Bell’s cruciate paralysis is characterized by paralysis of the upper limbs associated with little or no deficit in lower limbs. Its diagnosis is often overlooked, given that its symptoms similarities to that occasioned by the centromedullary syndrome, whose incidence is considerably higher. The present case report describes the case of a 16-year-old male patient victim of a gunshot wound in the anterior cervical region that developed Bell’s cruciate paralysis. The Magnetic Resonance Imaging (MRI) of the cervical spine showed medullary signal change in the cervicomedullary junction, with anteromedial location. A somatotopy of the corticospinal tract was suggested by Bell (1970) at cervicomedullary transition, with the anatomical hypothesis that fibers responsible for the movement of the upper limbs would cross higher and more superficially that the fibers of the lower limbs. This case combines the MRI assessment with the clinical outcome and corroborates with the Bell’s anatomical theory.

Rare case of diffuse spinal arachnoiditis following a complicated vertebral artery dissection

Spinal arachnoiditis (SA) is an extremely rare and delayed complication of subarachnoid hemorrhage (SAH). Little is known about its underlying pathogenesis and subsequent clinical course. A middle-aged patient presented with the worst headache of her life and a grade 3 SAH of the basal-cisterns and posterior fossa was identified on Computed Tomography scans (CT). Angiography revealed a ruptured dissecting aneurysm of the left vertebral artery (VA-V4), as well as an unruptured left Anterior Cerebral Artery (ACA-A1) aneurysm. The VA aneurysm was treated with flow diversion. The patient re-ruptured the stented aneurysm, another telescoping pipeline was placed. The patient developed polymicrobial ventriculitis, and returned several months later complaining of paraparesis and left sided weakness. Magnetic Resonance Imaging (MRI) revealed diffuse thecal dural thickening from the cervicomedullary junction to the sacrum. Loculations, diffuse edema and cord compression were noticed along the inferior surface of the cerebellum, and the cervico-thoracic spine with a T4–T6 syrinx. The patient underwent a posterior (T4–T8) spinal fusion and (T5–T7) decompression with arachnoid-cyst fenestration and placement of a subarachnoid-pleural shunt. On latest follow-up, the patient is weaning off the thoraco-lumbosacral orthosis and ambulating with a cane. SA is often a complicated two-staged disease in which a “free interval phase” separates the initial inflammatory reaction (IIR) from the late adhesive phase. Posterior fossa bleeding, warranting prolonged surveillance, additional bleeding and ventriculitis might augment the risk and the severity of arachnoiditis.

MRI Features of Aquaporin-4 Antibody-Positive Longitudinally Extensive Transverse Myelitis: Insights into the Diagnosis of Neuromyelitis Optica Spectrum Disorders.

Longitudinally extensive transverse myelitis is a well-documented spinal manifestation of neuromyelitis optica spectrum disorders, however, other forms of nontumorous myelopathy can also manifest as longitudinally extensive transverse myelitis. Our aim was to evaluate the MR imaging features of aquaporin-4 antibody-positive longitudinally extensive transverse myelitis, which is strongly associated with neuromyelitis optica spectrum disorders. We evaluated cervicomedullary junction involvement, cord expansion ratios, bright spotty lesions, the number of involved segments, skipped lesions, enhancement patterns, and axial distribution patterns using spinal MR imaging of 41 patients with longitudinally extensive transverse myelitis who underwent aquaporin-4 antibody testing. Univariate logistic regression analysis was performed to identify factors associated with aquaporin-4 antibody seropositivity, which were then used to develop a scoring system for diagnosing aquaporin-4 antibody-positive longitudinally extensive transverse myelitis. Interrater reliability for cord expansion ratio measurement and bright spotty lesions was determined using intraclass correlation coefficients and κ values, respectively. Fifteen patients with longitudinally extensive transverse myelitis were aquaporin-4 antibody-positive. Sex (female), cervicomedullary junction involvement, a cord expansion ratio of >1.4, and bright spotty lesions were significantly associated with aquaporin-4 antibody seropositivity. The sensitivity and specificity of the scoring system were 73.3% and 96.2%, respectively. The interclass correlation value for the cord expansion ratio was 0.78, and the κ value for bright spotty lesions was 0.61. Our scoring system, based on cervicomedullary junction involvement, higher cord expansion ratio, bright spotty lesions, and female sex, can facilitate the timely diagnosis of neuromyelitis optica spectrum disorders.

Chiari malformation type 1 presenting as unilateral progressive foot drop: a case report and review of literature

BackgroundFoot drop is a disabling clinical condition with multiplicity of causes, which requires detailed evaluation to identify the exact aetiology. Here, we report an extremely rare cause of foot drop in a child, which if not recognized early, could lead to multiple complications.Case presentationA 6-year-old girl presented with difficulty in walking and left sided foot droop for1-month duration. On examination she had reduced muscle power in dorsiflexors and plantar flexors and diminished knee and absent ankle jerk in the left side. Sensory loss was noted in L4 and L5 dermatomes on the left side. Superficial abdominal reflex was absent on the left side while preserved in the right. Nerve conduction and electromyography revealed nerve root or spinal cord cause for the foot drop. These results prompted ordering MRI spine and brain which revealed Chiari malformation type-1 with holocord syrinx extending from the cervicomedullary junction to conus medullaris.ConclusionsThis case highlights the importance of considering broad differential diagnosis for foot drop and value of the complete neurological examination including superficial reflexes in arriving at a diagnosis. Prompt diagnosis helped to early neurosurgical referral and intervention which is an important prognostic factor.