Locked-in syndrome following elective cervical foraminotomy: a case report.

INTRODUCTION: There are no previously reported cases of locked-in syndrome occurring following cervical spinal surgery. We describe a case of locked-in syndrome following an elective cervical foraminotomy and discuss potential etiologies and contributing factors to our patient's presentation. CASE PRESENTATION: A 54-year-old male with a history of head and neck cancer and prior anterior cervical discectomy and fusion presented with neck pain following a motor vehicle accident. The patient underwent C4-C7 left-sided cervical posterior foraminotomy with no intraoperative complications. On postoperative day 1, the patient suddenly developed rapidly progressing weakness of the extremities and soon became non-verbal. CT angiography and near-infrared spectroscopy confirmed a basilar artery occlusion and left vertebral artery dissection. On MRI, infarcts involving the bilateral pons, left cerebral hemisphere, and left cerebellar infarct were identified. CONCLUSION: The etiology of locked-in syndrome in our patient remains unclear, but it is likely multifactorial. It is possible that the patient was predisposed to vascular injury from prior radiation therapy to the head and neck. In addition, intraoperative vascular insult may have occurred from vibrational shear stress, in turn leading to a vertebral artery dissection, basilar artery occlusion, and pontine infarct, ultimately resulting in our patient's locked-in state.

Locked-in syndrome responding to endovascular treatment.

BACKGROUND: Locked-in syndrome (LiS) is a rare and devastating condition in patients with acute basilar artery occlusion. However, the benefits of endovascular treatment (EVT) for LiS remain unclear. OBJECTIVE: To assess the outcomes associated with EVT and identify the factors associated with outcomes of LiS. METHODS: We used the data of the Endovascular Treatment for Acute Basilar Artery Occlusion Study Registry (BASILAR) from 47 tertiary stroke centers in China. The included patients had LiS and received EVT or standard medical treatment (SMT) alone. The primary outcome was improvement in the modified Rankin Scale (mRS) score at 90 days. RESULTS: Among the 120 patients with LiS, 92 (76.7%) received EVT and 28 (23.3%) received SMT. Compared with SMT, EVT was associated with improved mRS score (common OR (cOR)=2.68 (95% CI 1.16 to 6.20); p=0.02) and decreased mortality (aOR=0.35 (95% CI 0.13 to 0.90); p=0.03). Moreover, the benefit of EVT for LiS was sustained for at least 1 year (p=0.008). Higher baseline posterior circulation Alberta Stroke Prognosis Early CT Score (pc-ASPECTS, aOR=2.04 (95% CI 1.34 to 3.10); p<0.001) and absence of pneumonia (aOR=0.26 (95% CI 0.08 to 0.90); p=0.03) were significantly associated with favorable functional outcome at 90 days in patients who received EVT, while lower pc-ASPECTS (aOR=0.52 (95% CI 0.36 to 0.76); p<0.001) was associated with increased 90-day mortality. CONCLUSIONS: This study found that EVT was associated with favorable functional outcomes and decreased mortality among patients with LiS. Baseline pc-ASPECTS and pneumonia were independent predictors of outcomes.

Neurophysiological aspects of the completely locked-in syndrome in patients with advanced amyotrophic lateral sclerosis.

OBJECTIVE: Amyotrophic lateral sclerosis (ALS) patients in completely locked-in syndrome (CLIS) are incapable of expressing themselves, and their state of consciousness and awareness is difficult to evaluate. Due to the complete paralysis included paralysis of eye muscles, any assessment of the perceptual and psychophysiological state can only be implemented in passive experimental paradigms with neurophysiological recordings. METHODS: Four patients in CLIS were investigated in several experiments including resting state, visual stimulation (eyes open vs eyes closed), auditory stimulation (modified local-global paradigm), somatosensory stimulation (electrical stimulation of the median nerve), and during sleep. RESULTS: All patients showed altered neurophysiological metrics, but a unique and common pattern could not be found between patients. However, slowing of the electroencephalography (EEG) and attenuation or absence of alpha wave activity was common in all patients. In two of the four patients, a slow dominant frequency emerged at 4 Hz with synchronized EEG at all channels. In the other two patients slowing of EEG appears less synchronized. EEGs between eyes open and eyes closed were significantly different in all patients. The dominant slow frequency during the day changes during slow-wave sleep (supposedly sleep stage 3) to even slower frequencies below 2 Hz. Somatosensory evoked potentials (SEPs) were absent or significantly altered in comparison to healthy subjects, similarly for auditory evoked potentials (AEPs). CONCLUSIONS: The heterogeneity of the results underscores the fact that no single neurophysiological index is available to assess psychophysiological states in unresponsive ALS patients in CLIS. This caveat may also be valid for the assessment of cognitive processes; a functioning BCI can be the solution. SIGNIFICANCE: Most of the studies of the neurophysiology of ALS patients focused on the early stage of the disease, and there are very few studies on the late stage when patients are completely paralyzed with no means of communication (i.e., CLIS). This study provides quantitative metrics of different neurophysiological aspects of these patients.

Severe Locked-In-Like Guillain-Barré's Syndrome: Dilemmas in Diagnosis and Treatment.

Guillain-Barré's syndrome in childhood can follow an atypical course, increasing the challenges in diagnosis and decisions regarding immunomodulatory treatment. Here, we report the case of on a 13-year-old boy with acute onset Guillain-Barré's syndrome progressing over 40 days to a very severe, locked-in-like syndrome despite intensive immunomodulatory treatment. After a plateau phase lasting 3 months and characterized by fluctuating signs of ongoing inflammatory disease activity, we were prompted to perform repeated and maintenance immunomodulatory treatment, which resulted in a continuous and nearly complete recovery of function. Atypical features at disease onset, the severe "total" loss of all peripheral and cranial nerve function, and an apparent late response to treatment give rise to reviewing the dilemmas of diagnosis and treatment in such severe and protracted courses of Guillain-Barré syndrome.

Locked-In with COVID-19.

Coronavirus Disease 2019 (COVID-19) can be associated with various neurological manifestations including acute strokes. Hyper acute diagnosis and treatment are key factors which decrease mortality and morbidity in stroke patients. The COVID-19 pandemic has introduced a great strain on the healthcare system, and as a result clinicians are facing several barriers in diagnosing and treating strokes. Delayed presentation of strokes is a problem as some in the general population defer the decision to seek immediate medical attention fearing contracting the virus. Also playing a role is the paucity of healthcare professionals available during a pandemic. Recent literature demonstrates the association of acute strokes in young patients with COVID-19. Lack of clear pathophysiology of the neurological manifestations from COVID-19 intensifies the problem. A thorough examination of the intensive care unit patient has always been a challenge owing to several factors including use of sedatives, sepsis, uremia, and encephalopathy secondary to medications. Locked-In Syndrome (LIS) secondary to stroke is much more challenging to diagnose as patients are unable to communicate or elicit any motor functions apart from certain ocular movements. We present the case of a 25 year old patient with no known history of coagulopathy, but had developed COVID-19 cytokine storm which culminated in LIS secondary to pontine strokes.

Medullary Infarction Leading to Locked-In Syndrome Following Lumbar Puncture in a Patient with Basilar Invagination.

BACKGROUND: Lumbar puncture is a common procedure that can be safely performed in most patients. Certain populations may have increased risk for complications following lumbar puncture, but the significance of basilar invagination is often underappreciated. CASE DESCRIPTION: A 45-year-old woman with basilar invagination received multiple lumbar punctures in the workup of acute meningitis. Preprocedural computed tomography was obtained. Following lumbar puncture, the patient developed locked-in syndrome. Magnetic resonance imaging obtained several days later demonstrated severe compression and infarction of the medulla and inferior cerebellum by the odontoid process and ectopic cerebellar tonsils. The patient was transferred but at this point, surgical decompression was not possible. She did not regain significant neurologic function. CONCLUSIONS: Basilar invagination is a risk factor for devastating neurologic complications following lumbar puncture. Awareness of this complication and prompt recognition of its occurrence may prevent future morbidity of lumbar puncture in patients with basilar invagination.

Transient Incomplete Locked-In Syndrome Secondary to Supratentorial Gunshot Wound.

BACKGROUND: Locked-in syndrome (LIS) is a rare neurologic disorder characterized as quadriplegia with anarthria. The diagnosis of LIS is challenging and requires a high index of suspicion. The syndrome is typically caused by an infratentorial lesion to the ventral pons, regardless of etiology. LIS secondary to supratentorial injury is extremely rare, and to our knowledge, this is the first reported case. CASE DESCRIPTION: We report the case of a 26-year-old woman who sustained a gunshot to the left suboccipital area, with supratentorial extension. A diagnosis of incomplete LIS was made on the day of admission, with eye movement preservation. Imaging studies confirmed bilateral injury of the motor homunculus. The clinical course was that of progressive improvement, aided by intensive care unit (ICU) supportive care and early physiotherapy rehabilitation. Her condition improved, and she was discharged to a rehabilitation facility at the end of week 7 postadmission. CONCLUSIONS: This is a unique case of incomplete LIS after supratentorial injury. Initial ICU care and early rehabilitation likely played a major role in the full recovery of this patient. The influence of etiology and site of injury on outcome prognosis is also suggested. Although severe diffuse brain injury may occur in the face of an unremarkable computed tomography (CT) scan, the emerging role of magnetic resonance imaging in optimally evaluating traumatic brain injury with discordant clinical and CT information is highlighted and is useful in cases of LIS where prognosis prediction is important.