Presented at Ignite Health 2.0. Portland, OR. [ohsu.edu]

The present clinical commentary highlights an under-recognized and clinically highly relevant topic, exemplified by a lesser-known but stunning piece of literature from one of the most beloved contemporary novelist. [ncbi.nlm.nih.gov]

Clinical Presentation The neurobehavioral criteria presenting in those diagnosed with LIS consist of: Maintained eye opening Basic cognitive abilities are preserved Severe hyophonia or aphonia Quadriparesis or quadriplegia Communication through vertical [physio-pedia.com]

Cardenas, Aparna Prabhu, Yan Zhang First published November 26, 2018, DOI: https://doi.org/10.1212/WNL.0000000000006579 An 85-year-old man presented with 48 hours of recurrent vomiting and vertigo with rapidly progressive dysphagia and quadriplegia. [n.neurology.org]

This paper presents results from 12 patients (3 CLIS and 9 LIS) and 3 healthy controls. [frontiersin.org]

• Disability

  Stroke is the No. 4 cause of death in the United States, and the leading cause of disability. Locked-in syndrome is an extremely rare, but debilitating effect of stroke. [newsarchive.heart.org]

  This study aimed to better understand whether these treatment options would be deemed worthwhile by patients with severe disability. [alsnewstoday.com]

  Background: There are few studies where quality of life (QOL) and contributive factors are assessed in locked-in syndrome (LIS) patients with complete physical and functional disability and dependence on caregivers. [liebertpub.com]

  Yet we’ve made limited progress in using it for its original purpose: helping people with severe disabilities to communicate. Until now, that is. [sciencealert.com]

  The patient is still severely disabled in spite of physiotherapy. His private, social, and professional life has been shattered. We believe this is the only paper detailing such a long-term follow-up of a fulminant GBS case. [dovepress.com]

• Nightmare

  He was placed in a coma and when he woke, was in a hellish nightmare. [foxnews.com]

  It must be a living nightmare. It’s like being buried alive.” Leith has been in a neurorehabilitation center over 100 miles away from his family. [msn.com]

  It's many people's idea of a nightmare: Being so paralyzed that you can no longer communicate at all. [bigthink.com]

  For most of us: a nightmare world. “Are you happy?” But surprisingly, when the researchers asked the question, “Are you happy?,” the answer from all four was consistently “yes,” repeated over weeks of questioning. [kurzweilai.net]

  Here are the stories of eight people who have lived this nightmare. Julia Tavalaro One of the dangers of locked in syndrome is the possibility of misdiagnosis. [mentalfloss.com]

• Fatigue

  Typing is easier than writing longhand, and fatigue is always nearby: “I have exhaustion that comes over me. And I mean exhaustion.” It was years before she overcame the fear of having another stroke. [strokeconnection.strokeassociation.org]

  "Eye movements fatigue very rapidly. It gives a sense of pressure on the communicator to be clever about the questions. "It's the equivalent of a mobile phone with a dying battery." [news.bbc.co.uk]

  The decision of whether to attempt communication included considerations such as the physician's assessment of the patient's fatigue and available time; thus, clinical considerations were prioritized over research needs. [frontiersin.org]

  It was hypothesized that those two values would be negatively correlated if the patient was able to perceive the probability difference of target and non-target stimuli, that is a decrease in the accuracy from oddball-pre to oddball-post, e.g. due to fatigue [academic.oup.com]

• Cough

  Further motor phenomena like whining, moaning, groaning, chewing, and coughing were incidentally observed in LIS. [baillement.com]

  Many times when we lift her (though we are careful) the tracheostomy tube tilts, sometimes it causes violent cough. Even meticulous care and devotion cannot prevent such episodes. [bmj.com]

• Denial

  (SWNS) “I was in complete denial,” Shannon Leith told SWNS. “It was just awful to be told that. We were told it is incurable and dad will be like this forever. I struggled to cope to begin with. It was absolutely horrible. [foxnews.com]

  “I was in complete denial,” Shannon Leith told SWNS. “It was just awful to be told that. We were told it is incurable and dad will be like this forever. I struggled to cope to begin with. It was absolutely horrible. [msn.com]

  "Locked-in Lynsey's care woe: Parents anger at denial of specialist help". The Sun. Archived from the original on October 10, 2012. Retrieved November 22, 2018. ^ a b de Lange, Catherine (December 30, 2011). [en.wikipedia.org]

• Withdrawn

  Treatment can already be withdrawn if a person’s relatives think it is what they would have wanted. There are well established ways of deciding if someone is of sound mind – but analysing brain activity is not currently conducive to them. [newscientist.com]

  They are allowed to die after their life-sustaining therapy (positive-pressure ventilation) is withdrawn in accordance with their wishes. [scholarpedia.org]

• Stroke

  Brain-stem stroke Blink Once [newsarchive.heart.org]

  OKLAHOMA DAD SUFFERS STROKE AFTER CRACKING NECK Darren Leith, a painter from Cumbria, England, suffered the stroke in April 2017. He was placed in a coma and when he woke, was in a hellish nightmare. [foxnews.com]

  Like all strokes, brain stem strokes produce a wide spectrum of deficits and recovery. [strokeassociation.org]

  Acute stroke care in the US: results from 4 pilot prototypes of the Paul Coverdell National Acute Stroke Registry. Stroke. 2005 Jun. 36(6):1232-40. [Medline]. [Full Text]. Dirnagl U, Pulsinelli W. [medscape.com]

  A brainstem stroke syndrome falls under the broader category of stroke syndromes, or specific symptoms caused by vascular injury to an area of brain (for example, the lacunar syndromes ). [en.wikipedia.org]

• Quadriplegia

  Anatomy Bilateral ventral pons Vascular Basilar artery Signs & Symptoms Side Manifestation Comments Both Weakness – upper and lower extremity Quadriplegia: bilateral cortical spinal tracts Both Weakness – face – entire side Bilateral corticobulbar tracts [strokecenter.org]

  LIS is characterized by quadriplegia and bulbar palsy, caused by the interruption of the corticospinal and corticobulbar tracts in the pons. The only remaining voluntary muscle movements include vertical eye movement and blinking. [amboss.com]

  Cardenas, Aparna Prabhu, Yan Zhang First published November 26, 2018, DOI: https://doi.org/10.1212/WNL.0000000000006579 An 85-year-old man presented with 48 hours of recurrent vomiting and vertigo with rapidly progressive dysphagia and quadriplegia. [n.neurology.org]

  Locked-in syndrome usually results from a brainstem hemorrhage or infarct resulting in quadriplegia and disruption of the lower cranial nerves. [medicinenet.com]

  Locked-in syndrome typically results from a pontine hemorrhage or infarct that causes quadriplegia and disrupts and damages the lower cranial nerves and the centers that control horizontal gaze. [msdmanuals.com]

• Intracranial Hemorrhage

  Brain death is classically caused by a massive brain lesion, such as trauma, intracranial hemorrhage or anoxia. The EEG in brain death shows absent electrocortical activity with a sensitivity and specificity of around 90%. [scholarpedia.org]

Doctors at the hospital in Bari were baffled and Loredana had to take her father to a specialist in Marseille for a full neurological workup. At first, Anselmo took the diagnosis of ALS badly. [theguardian.com]

Communication may be possible with blinking eye movements Treatment There is no cure for locked-in syndrome, nor is there a standard course of treatment. [brainfacts.org]

Biased clinicians might provide less aggressive medical treatment and influence the family in inappropriate ways. [ncbi.nlm.nih.gov]

They aimed to better understand the effects of locked-in syndrome on health management and life enhancing/sustaining treatments. [alsnewstoday.com]

Patients should receive treatment as soon as possible for the best recovery. Like all strokes, brain stem strokes produce a wide spectrum of deficits and recovery. [strokeassociation.org]

This valuable tool offers detailed descriptions, facts on treatment and prognosis, and patient organization contact information for over 500 identified neurological disorders. Neurological Disorders and Stroke » [brainfacts.org]

In cases with other causes, particularly traumatic injury, caution should be observed with respect to making a prognosis on the basis of EEG alone. [tidsskriftet.no]

Prognosis While in rare cases some patients may regain certain functions, the chances for motor recovery are very limited. Research The NINDS supports research on neurological disorders that can cause locked-in syndrome. [childneurologyfoundation.org]

What is the prognosis with locked-in syndrome? The prognosis of patients with locked-in syndrome varies from fair to poor. [medicinenet.com]

Prognosis (outlook for recovery) According to the International Encyclopaedia of Rehabilitation and researchers, recovery of horizontal eye movements prior to the first four weeks is associated with a good neurological prognosis (Bauer et al., 1979). [brainfoundation.org.au]

Etiology Ventral pontine damage/injury Bilateral ventral pontine stroke (most common) Direct trauma to the ventral pons Pontine demyelination (e.g., multiple sclerosis affecting the ventral pons, central pontine myelinolysis ) Brain tumor or brain abscess [amboss.com]

In this article, the author discusses the presentation, etiology, prognosis, diagnostic evaluation, and management of locked-in syndrome. [medlink.com]

Setting/subjects: We have included 19 LIS patients, most of them with vascular etiologies or terminal course of ALS, and 20 healthy controls comparable with LIS patients for age; none of them was in a caregiving position. [liebertpub.com]

traumatic or other non-traumatic etiology. [biomedical-engineering-online.biomedcentral.com]

No statistical relationships of QoL with disease duration, LIS type, or LIS etiology were found. [ojrd.biomedcentral.com]

Epidemiology The precise prevalence rate of LIS is not specifically documented in the literature and the incidence rate is thought to be underestimated. [physio-pedia.com]

Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature. Neuroepidemiology 2013 ;41: 118 - 130 3. Laureys S, Pellas F, Van Eeckhout P, et al. [nejm.org]

Epidemiology of ambulatory anesthesia for children in the United States: 2006 and 1996. Anesthesia and Analgesia, 111 ( 4 ), 1011 – 1015. https://doi.org/10.1213/ANE.0b013e3181ee8479 ↵ Sury, M. R. J., Worley, A., & Boyd, S. G. ( 2014 ). [biorxiv.org]

Neuropathological studies of autopsied cases have helped to understand the pathophysiology of the syndrome [ 4, 5 ]. [journals.plos.org]

While the pathophysiology of CMD remains unclear, the majority of patients diagnosed so far had sustained a traumatic brain injury. [medscape.com]

A possible pathophysiologic role for anti SSA/Ro and anti SSB/La autoantibodies. Arthritis Rheum, 36 (1993), pp. 259-262 [3.] P.A. Gordon, E. Rosenthal, M.A. Kamashta, G.R.V. Huges. [reumatologiaclinica.org]

In the present article we seek to provide a principle understanding of electroencephalography (EEG), with an outline of the basic physiology and some simple pathophysiological observations. [tidsskriftet.no]

Experience in solving clinico-pathophysiological tasks in practical lessons on pathological physiology. [Opyt resheniia kliniko-patofiziologicheskikh zadach na prakticheskikh zaniatiiakh po patologicheskoǐ fiziologii.] [ojs.ukw.edu.pl]

Can locked-in syndrome be prevented? Prevention of trauma that leads to locked-in syndrome is possible, but difficult to predict. [medicinenet.com]

The goals of this research are to find ways to prevent, treat, and cure these disorders. View research on this topic. View studies being conducted about this condition. View NINDS publications on this topic. [brainfacts.org]

Prevent complications from immobility: passive limb mobilizations are a critical part of the physiotherapist’s role in the early stages to help prevent contractures and periarticular calcifications, as well as reduce the amount of spasticity that may [physio-pedia.com]

[…] nutrition Providing physical therapy to prevent limb contractures There is no specific treatment. [msdmanuals.com]

The goals of this research are to find ways to prevent, treat, and cure these disorders. [childneurologyfoundation.org]