Vertebral artery dissection as the underlying cause of ventral spinal epidural haematoma.

Spontaneous spine epidural haematoma is a rare occurrence, with an incidence of 0.1/100 000 inhabitants/year. The anterior location of the haematoma is very uncommon since the dural sac is firmly attached to the posterior longitudinal ligament. Vertebral artery dissection as its underlying cause is an exceptionally rare event, with only two documented cases.This article presents the case of young woman who arrived at the emergency room with a spinal ventral epidural haematoma extending from C2 to T10, caused by a non-traumatic dissecting aneurysm of the right vertebral artery at V2-V3 segment. Since the patient was tetraparetic, she underwent emergent laminectomy, and the vertebral artery dissection was subsequently treated endovascularly with stenting.Vertebral artery dissection with subsequent perivascular haemorrhage is a possible cause of spontaneous spine epidural haematoma, particularly when located ventrally in the cervical and/or high thoracic column. Hence the importance of a thorough investigation of the vertebral artery integrity.

Experiencing the unreal. Mapping patient journeys from injury towards rehabilitation for a life with tetraplegia.

When patients are brought to the emergency room with a traumatic injury to the spinal cord, the road to recovery is long and uncertain. In minutes, their lives have been irreversibly altered. Time will tell if the paralysis to the limbs is permanent or if some degree of mobility or sensation can be reacquired. To many patients, the first weeks feel almost unreal. They find themselves in a state of shock, and feelings of uncertainty and loss of control are dominating. Spinal cord injuries are treated in multiple specialized hospital units. Each unit is accountable for providing the best possible treatment within their area of expertise. While continuity in care has been a hot research topic for the past 20 years, few studies provide empirical data on patients' perspectives on long-term hospital treatments. This study examines how patients with tetraplegia experience continuity in care when they journey across multiple hospital units over the course of several months. This paper is based on a study of patient pathways for patients with tetraplegia caused by high spinal cord injuries. Semi-structured interviews were conducted with nine patients, seven next of kin and thirteen healthcare professionals in Copenhagen University Hospital. Data from the interviews were analyzed using patient journey mapping, to uncover variations in patients' experience during various stages of their admission. The study finds that patients are struggling to maintain a sense of control over their life as they continuously engage in negotiations of perceptions of their body, of the physical surroundings and of their perception of time and the future. The study concludes that health care professionals should be mindful of these key themes to support the patients' empowerment and active participation during recovery.

The effect of level of injury on diabetes incidence and mortality after spinal cord injury - a longitudinal cohort study.

STUDY DESIGN: Retrospective longitudinal cohort study of veterans with SCI. OBJECTIVES: Spinal cord injury (SCI) is associated with an increased risk of developing diabetes mellitus (DM), likely due to body composition alterations and autonomic nervous system dysfunction. These factors are more pronounced in persons with tetraplegia (TP) versus paraplegia (PP). However, the effect of level of injury (LOI) on DM incidence is largely unknown. Therefore, the objective is to examine the effect of LOI on DM incidence in persons with SCI. SETTING: South Texas Veterans Health Care System. METHODS: We obtained electronic record data on age, sex, race/ethnicity, LOI and HbA1c concentration from January 1st 2001 through December 31st 2021. Cox proportional hazard regression analyses were used to assess the association between LOI, DM and all-cause mortality. RESULTS: Among 728 non-diabetic veterans with SCI (350 TP/ 378 PP, 52 ± 15 years, 690 male/38 female) 243 developed DM, of which 116 with TP and 127 with PP. Despite chronological variations between TP and PP, DM risk over the entire follow-up did not differ between the groups (hazard ratio (HR): 1.06, 95% CI: 0.82-1.38). Mortality was higher in TP versus PP (HR: 1.40, 95% CI: 1.09-1.78). However, developing DM did not increase the risk of death, regardless of LOI (HR: 1.07, 95% CI: 0.83-1.37). CONCLUSION: Despite chronological variations between both groups, the level of injury had minimal effect on long-term DM development in this cohort of veterans with SCI. Sponsorship NIH (DK105379; MS), RR&D SPiRE (I21RX003724-01A1; MT and SH).

Neurological and neuromuscular manifestations in patients with West Nile neuroinvasive disease, Belgrade area, Serbia, season 2022.

INTRODUCTION: We aimed to describe neurological manifestations and functional outcome at discharge in patients with West Nile neuroinvasive disease. METHODS: This retrospective study enrolled inpatients treated in the University Clinic for Infectious and Tropical Diseases in Belgrade, Serbia, from 1 June until 31 October 2022. Functional outcome at discharge was assessed using modified Rankin scale. RESULTS: Among the 135 analyzed patients, encephalitis, meningitis and acute flaccid paralysis (AFP) were present in 114 (84.6%), 20 (14.8%), and 21 (15.6%), respectively. Quadriparesis/quadriplegia and monoparesis were the most frequent forms of AFP, present in 9 (6.7%) and 6 (4.4%) patients, respectively. Fourty-five (33.3%) patients had cerebellitis, 80 (59.3%) had rhombencephalitis, and 5 (3.7%) exhibited Parkinsonism. Ataxia and wide-based gait were present in 79 (58.5%) patients each. Fifty-one (37.8%) patients had tremor (41 (30.3%) had postural and/or kinetic tremor, 10 (7.4%) had resting tremor). Glasgow coma score (GCS) ≤ 8 and respiratory failure requiring mechanical ventilation developed in 39 (28.9%), and 33 (24.4%) patients, respectively. Quadriparesis was a risk factor for prolonged ventilator support (29.5 ± 16.8 vs. 12.4 ± 8.7 days, p = 0.001). At discharge, one patient with monoparesis recovered full muscle strength, whereas 8 patients with AFP were functionally dependent. Twenty-nine (21.5%) patients died. All of the succumbed had encephalitis, and 7 had quadriparesis. Ataxia, tremor and cognitive deficit persisted in 18 (16.9%), 15 (14.2%), and 22 (16.3%) patients at discharge, respectively. Age, malignancy, coronary disease, quadriparesis, mechanical ventilation, GCS ≤ 8 and healthcare-associated infections were risk factors for death (p = 0.001; p = 0.019; p = 0.004; p = 0.001; p < 0.001; p < 0.001, and p < 0.001, respectively).

Atypical Presentation of the Classical Form of Locked-In Syndrome in a Young Patient.

Locked-in syndrome (LIS) is an uncommon neurologic disorder that manifests in quadriplegia and anarthria with preserved cognition and self-awareness. Generally, patients with LIS may live for years with preserved quality of life and cognitive function, but with severe disability. There are 3 types of LIS: classic, partial, and total. The classical form is defined by total immobility, with preservation of the ability to perform vertical eye movements, blink, and maintain a normal level of consciousness. We present an unusual case of classical LIS in a 54-year-old man who presented initially with acute-onset left lower extremity weakness and dysarthria with radiographic evidence of extensive, acute right paramedian pontine infarction and high-grade vertebral artery stenosis. However, a week later, the patient developed sudden-onset aphonia, bilateral facial palsy, and quadriplegia with repeat magnetic resonance imaging of the brain showing expansion of right paramedian pontine stroke to also involve the left paramedian pons, without significant change to the vertebral stenosis and basilar artery patency.

Acute Porphyria: An Unusual Case Of Quadriparesis, Hypertension, Recurrent Severe Cyclic Abdominal Pain, And Seizures.

Porphyria refers to a rare group of genetically inherited or acquired disorders that arise due to reduced metabolic activity of any of the enzymes in the haem biosynthetic pathway. Defect in any enzyme causes the presentation of symptoms of porphyria. The epidemiology of Acute Intermittent Porphyria (AIP) is complicated because of its rarity and delay in diagnosis. We present the case of a seven-year-old girl who presented with multisystem involvement; her symptoms were quadriparesis, hypertension, recurrent severe cyclic abdominal pain, and seizures. These symptoms together were not explained by the differentials taken into account. She presented before puberty with no family history of such conditions, while being born of consanguineous marriage. Her symptoms along with urinary porphobilinogen positivity test helped to reach the diagnosis of AIP in the absence of cutaneous manifestations. This case highlights the variable presentation of porphyria and emphasises the importance of appropriate and timely diagnosis and management in these patients.

Test-retest reliability of short- and long-term heart rate variability in individuals with spinal cord injury.

STUDY DESIGN: Cross-sectional. OBJECTIVES: To investigate test-retest reliability of heart rate variability (HRV) metrics in SCI without restriction of activity over long (24-h) and shorter durations (5-min, 10-min, 1-h, 3-h and 6-h). SETTINGS: University hospital in Khon Kaen, Thailand. METHODS: Forty-five participants (11 with tetraplegia and 34 with paraplegia) underwent two 24-h recordings of RR-intervals to derive time and frequency HRV metrics. Relative reliability was assessed by intraclass correlation coefficient (ICC) and absolute reliability by coefficient of variation (CV) and Bland-Altman limits of agreement (LoA). RESULTS: For 5- and 10-min durations, eight of eleven HRV metrics had moderate to excellent reliability (ICC 0.40-0.76); the remaining three were poor (ICC < 0.4). HRV values from 1-h and 3-h durations showed moderate to excellent reliability (ICC of 0.46-0.81), except for 1-h reliability of ULF and TP (ICC of 0.06 and 0.30, respectively). Relative reliability was excellent (ICC of 0.77-0.92) for 6-h and 24-h durations in all HRV metrics. Absolute reliability improved as recording duration increased (lower CVs and narrower LoAs). Participants with high AD risk (SCI level at or above T6) showed lower test-retest reliability of HF and LF values than participants with low AD risk. CONCLUSION: Relative reliability of HRV was excellent for 6-h and 24-h. The best absolute reliability values were for 24-h duration. Time-domain outcomes were more reliable than frequency domain outcomes. Participants with high risk of AD, particularly those with tetraplegia, showed lower reliability, especially for HF and LF.

Demographic, Medical, and Clinical Characteristics of a Population-Based Sample of Patients With Long-lasting Locked-In Syndrome.

BACKGROUND AND OBJECTIVES: Patients with classic locked-in syndrome (LIS), typically caused by ventral pontine stroke, present with quadriplegia, mutism, intact consciousness, and communication skills limited to vertical gazing and/or blinking. Clinical presentations and definitions of LIS differ, especially regarding incomplete LIS. In our study, we explored the functional diversity of LIS, its outcomes, and the complexity of its course along with variations in the location of lesions and their potential significance for outcomes. METHODS: A national cohort of patients with vascular LIS who remained in the LIS state for at least 6 weeks according to a functional definition of LIS was studied. Demographic, medical, and follow-up data, collected between 2012 and 2022, were obtained from the quality register of the Norwegian National Unit for Rehabilitation of Locked-In Syndrome. Outcomes in verbal communication, motor function, and dependency were evaluated according to criteria for being in or not in the LIS state. The modified Rankin scale and LIS motor recovery scale were applied. Descriptive analysis was performed. The relationship between lesion location and functional outcome was investigated. RESULTS: The sample included 51 patients (median age: 55.7 years, 36 male individuals), 43 of whom had follow-up data. Ischemic stroke was the most common etiology (n = 35). Twenty-three patients had emerged from the LIS state, mostly within 2 years after onset. All but 1 patient achieved some motor improvement, whereas only 3 achieved full motor recovery, and 88% had a persistently high level of dependence. The 3-year survival rate was 87%. Five patients had an isolated pontine lesion, whereas 80% showed various lesions outside the brain stem. Patients who emerged from the LIS state had a significantly lower prevalence of lesions outside the brain stem than patients who remained in the LIS state did. DISCUSSION: Investigating an unselected population-based sample of patients with vascular LIS offers important insights into the functional diversity of LIS. Although most patients remained severely disabled, even small improvements in function can substantially increase the potential for activity and participation. Additional lesions outside the brain stem seem to be common in long-lasting LIS and might be prognostic for remaining in the LIS state.

Grasp and Release Test for Tetraplegic Hand Assessment: an update of the Grasp and Release Test.

The Grasp and Release Test (GRT) was originally developed to measure effectiveness of an implanted neuroprosthesis in people with tetraplegia. Its ease of use and lack of floor and ceiling effects culminated in recommendations for inclusion in a battery of tests to measure outcome following upper limb reconstructive surgery. However, the length of time taken to administer the GRT in a clinical setting, lack of instructions of accepted grasp patterns in the upper limb reconstructive surgery population and scoring procedures lead to differences in reporting outcomes using this measure. In order to ensure clinical utility for the upper limb reconstructive surgery population, revisions of the original test instructions have been made and are reported in this article. Further testing of the psychometric properties of the new measure are currently underway.

The Surgical Restoration of Arm and Hand Function in Tetraplegic Patients.

BACKGROUND: There are approximately 140 000 people in Germany with spinal cord injury, with approximately 2400 new patients each year. Cervical spinal cord injuries cause, to varying degrees, weakness and impairment of everyday activities of the limbs (tetraparesis, tetraplegia). METHODS: This review is based on relevant publications retrieved by a selective search of the literature. RESULTS: From among 330 initially screened publications, 40 were included and analyzed. Muscle and tendon transfers, tenodeses, and joint stabilizations yielded reliable functional improvement of the upper limb. Tendon transfers improved the strength of elbow extension from M0 to an average of M3.3 (BMRC) and grip strength to approximately 2 kg. In the long term, 17-20% of strength is lost after active tendon transfers and slightly more after passive ones. Nerve transfers improved strength to M3 or M4 in over 80% of cases, with the best results overall in patients under 25 years of age who underwent early surgery (within 6 months of the accident). Combined procedures in a single operation have been found to be advantageous compared to the traditional multistep approach. Nerve transfers from intact fascicles at segmental levels above that of the spinal cord lesion have been found to be a valuable addition to the established varieties of muscle and tendon transfer. The reported long-term patient satisfaction is generally high. CONCLUSION: Modern techniques of hand surgery can help suitably selected tetraparetic and tetraplegic patients regain the use of their upper limbs. Competent interdisciplinary counseling about these surgical options should be offered as early as possible to all affected persons as an integral part of their treatment plan.

Improved walking capacity after complementary ankle-foot surgery and gait training in a person with an incomplete tetraplegia; a case report.

INTRODUCTION: The population of people with a spinal cord injury (SCI) is changing to a diverse population with an increasing number of incomplete lesions. Often, these individuals have the capacity to walk, but experience disabling gait impairments. CASE PRESENTATION: The course of a 34-year-old male with a chronic incomplete traumatic cervical SCI who initially could walk no more than a few steps with supervision or a wheeled walker is described. He participated in a clinical trial with Targeted Epidural Spinal Stimulation (TESS). After this trial, he was able to walk with a wheeled walker and bilateral orthosis over a distance of 100 meters. Despite these improvements, his main complaints were (1) difficulty to correctly preposition the feet, and (2) pain in his toe and calf muscles. An interdisciplinary approach and the use of structured gait analysis formed the basis for shared decision-making with the team and the patient to perform ankle-foot surgery followed by 2-month gait training with a body weight support system. After this trajectory his walking distance increased to 250 meters, with a wheeled walker; but now without orthosis and with an increased walking speed compared to pre-surgery. Additionally, there was reduction of pain and he experienced no disturbances during sleeping, washing and clothing anymore. DISCUSSION: This case shows that surgical interventions can improve the gait capacity even in case of chronic incomplete SCI. Furthermore, training with a body weight support system after medical-technical interventions is useful to utilize the full potential of these interventions.

Cervical Instability and Quadriparesis Requiring Stabilization in Pediatric Patient Caused by a Mutation in COL2A1.

A 3-year-old male with no past medical history presented with flaccid plegia of his upper extremities and significant weakness in his lower extremities after wrestling with his brother. Cervical spine magnetic resonance imaging was consistent with cord edema and intraparenchymal hemorrhage at C1-C2. A nonossified tissue mass at the expected location of the upper dens created narrowing of the canal at the C1-2 level and mass effect on the cord. Head computed tomography showed periventricular leukomalacia. Initial findings favored dysplasia of the odontoid with associated soft tissue mass/pannus caused by a possible underlying genetic or metabolic bone dyscrasia. The patient underwent suboccipital craniotomy/C1 laminectomy and occiput to C4 fusion, for decompression and stabilization. Genetic testing showed a COL2A1 collagen disorder, with the child harboring a de novo mutation for c.3455 G>T (p.G1152V). The patient was discharged to inpatient acute rehabilitation, with gradual improvement in strength in all 4 extremities.

Quantifying Tenodesis Hand Function in Cervical Spinal Cord Injury: Implications for Function.

PURPOSE: Cervical spinal cord injury (SCI) has a profound effect on upper-extremity function. Individuals with stiffness and/or spasticity may have more, or less, useful tenodesis function. This study examined the variability present before any reconstructive surgery. METHODS: Tenodesis pinch and grasp were measured with the wrist in maximal active extension. Tenodesis pinch was the contact point of the thumb with the index finger proximal phalanx (T-IF:P1), middle phalanx (T-IF:P2), distal phalanx (T-IF:P3), or absent (T-IF:absent). Tenodesis grasp was the distance from the long finger to the distal palmar crease (LF-DPC). Activities of daily living function was assessed using the Spinal Cord Independence Measure (SCIM). RESULTS: The study included 27 individuals (4 females, 23 males; mean age 36 years, mean time since SCI 6.8 years). The mean International Classification for Surgery of the Hand in Tetraplegia (ICSHT) group classification was 3. In the dominant hand, individuals with a T-IF tenodesis pinch to P1 or P2 had significantly higher total SCIM scores (43.7 and 34.2, respectively) compared to those with absent T-IF tenodesis pinch (SCIM 17.8). Shorter LF-DPC distance with tenodesis grasp (improved finger closing) also correlated with improved SCIM mobility and total scores. No association was found between the ICSHT group and SCIM score or tenodesis measures. CONCLUSIONS: Quantifying tenodesis with pinch (T-IF) and grasp (LF-DPC) is a simple method to characterize hand movement in individuals with cervical SCI. Better tenodesis pinch and grasp were associated with improved activities of daily living performance. CLINICAL RELEVANCE: Differences in grasp function have implications for mobility, and differences in pinch function have implications for all functions, particularly self-care. These physical measurements could be used to assess movement changes after nonsurgical and surgical treatment in tetraplegia.

Walking naturally after spinal cord injury using a brain-spine interface.

A spinal cord injury interrupts the communication between the brain and the region of the spinal cord that produces walking, leading to paralysis1,2. Here, we restored this communication with a digital bridge between the brain and spinal cord that enabled an individual with chronic tetraplegia to stand and walk naturally in community settings. This brain-spine interface (BSI) consists of fully implanted recording and stimulation systems that establish a direct link between cortical signals3 and the analogue modulation of epidural electrical stimulation targeting the spinal cord regions involved in the production of walking4-6. A highly reliable BSI is calibrated within a few minutes. This reliability has remained stable over one year, including during independent use at home. The participant reports that the BSI enables natural control over the movements of his legs to stand, walk, climb stairs and even traverse complex terrains. Moreover, neurorehabilitation supported by the BSI improved neurological recovery. The participant regained the ability to walk with crutches overground even when the BSI was switched off. This digital bridge establishes a framework to restore natural control of movement after paralysis.

Intractable postural hypotension resulting from cervical pseudomeningocele after a posterior cervical spinal surgery: a case report.

INTRODUCTION: Postural hypotension (PH) is common in patients with spinal cord injury (SCI), especially those with tetraplegia. To effectively treat PH, identifying and eliminating treatable predisposing factors of PH are prerequisites before applying any interventions. CASE PRESENTATION: We report a patient with post-acute cervical SCI who suffered from intractable PH resulting from pseudomeningocele causing unfavorable rehabilitation outcomes. A previously healthy 34-year-old man with complete C6 SCI resulting from C6-C7 fracture dislocation developed PH in the first week of the rehabilitation program. No specific predisposing factors including anemia, hyponatremia, and dehydration were identified. Non-pharmacological interventions and pharmacological treatment were administered to the patient without satisfactory result, causing a delay in rehabilitation progression. In the fourth week of rehabilitation program, a mass at the surgical site was detected. A cervical MRI revealed a large fluid collection at the posterior aspect of cervical spines with a size of 7.9 × 6.8 × 5.0 cm. A diagnosis of pseudomeningocele was made and surgical site debridement with closing dura by grafting was immediately conducted. One day after surgery, PH disappeared, and the patient could progress in his rehabilitation program and achieve his short-term goal within three weeks. CONCLUSION: Pseudomeningocele could be one of the precipitating factors of PH in patients with tetraplegia. Healthcare providers should consider investigating pseudomeningocele in patients who have intractable and unexplainable PH.