Presentation
Presented at Ignite Health 2.0. Portland, OR. [ohsu.edu]
Cardenas, Aparna Prabhu, Yan Zhang First published November 26, 2018, DOI: An 85-year-old man presented with 48 hours of recurrent vomiting and vertigo with rapidly progressive dysphagia and quadriplegia. [n.neurology.org]
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]
This paper presents results from 12 patients (3 CLIS and 9 LIS) and 3 healthy controls. [frontiersin.org]
At present, Ramsey is only able to communicate short and basic sounds. [en.wikipedia.org]
Entire Body System
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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]
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Painter
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]
Darren Leith, a painter from Cumbria, England, suffered the stroke in April 2017. [msn.com]
But in 2002, the painter was diagnosed with amyotrophic lateral sclerosis, also known as Lou Gehrig's disease. This debilitating neurological disorder progressively destroys a person's motor neurons. [news.virginia.edu]
He is the son of painter Alice Lok Cahana. Tony Nicklinson [ edit ] Tony Nicklinson, of Melksham, Wiltshire, England, was left paralysed after suffering a stroke in June 2005, [5] at age 51. [en.wikipedia.org]
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Pathologist
Neuroscience at Seton Hall University, South Orange, NJ, and Associate Director of Neuropsychology at JFK Johnson Rehabilitation Institute and the New Jersey Neuroscience Institute, JFK Medical Center, Edison, NJ Joseph T Giacino T Cullen is a Speech-Language Pathologist [nature.com]
Gastrointestinal
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Overeating
MARYLAND WOMAN SUFFERS STROKE AFTER TEARING ARTERY DURING YOGA Leith has been in a neurorehabilitation center over 100 miles away from his family. [foxnews.com]
Leith has been in a neurorehabilitation center over 100 miles away from his family. Shannon said the last time she went to go see him he spelled out “take me home,” using his eyes to point to letters on a board. [msn.com]
A less advanced form of the condition, simply known as locked-in syndrome, refers to patients who retain some small control over their eye movements. [mashable.com]
Despite being locked in, Bauby “dictated” his memoir to Claude Mendibil by the laborious method of blinking when the correct letter was reached by a person slowly reciting the alphabet over and over again. [medlink.com]
Characteristics: A Locked-in patient has no control over the cripple limbs ( quadriplegia or tetraplegia ) Often one does not have good control over the facial muscles. Swallowing is often impossible. Reduced facial expression. [braininjury-explanation.com]
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Hiccup
Catherine O'Leary 31-year-old Catherine O'Leary suffered from hiccups for three years before the cause was discovered to be a brain tumor. During surgery to remove the tumor, a series of strokes left her completely paralyzed. [mentalfloss.com]
Musculoskeletal
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Back Pain
“You have back pain.” (“No.”) “You are happy to be alive.” (“Yes.”) [extremetech.com]
Psychiatrical
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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]
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Type A Personality
Introduction The left panel of Figure 1 categorizes different types of persons based on their available cognitive and motor functions. [frontiersin.org]
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Hunger
You can feel pain, hunger, loneliness, and fear, but you can't react to those sensations. You are totally aware of your surroundings, but you can't communicate your feelings or desires, or even your basic needs. [mentalfloss.com]
Neurologic
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Slurred Speech
A brain stem stroke can also cause double vision, slurred speech and decreased level of consciousness. [strokeassociation.org]
Abstract Background A 53-year-old male with a history of hypertension, diabetes mellitus, and factor V deficiency presented to an emergency room with progressively increasing headache, slurred speech, and left upper extremity weakness. [nature.com]
Brainstem stroke can also cause diplopia, slurred speech and decreased level of consciousness. A more serious outcome is locked-in syndrome. [en.wikipedia.org]
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Babinski Sign
The following symptoms are detected on regaining consciousness: Paralysis of voluntary muscles Paralysis of all 4 limbs and torso ( quadriplegia ) spasticity, deep tendon reflexes, positive bilateral Babinski's sign Bulbar palsy : inability to speak ; [amboss.com]
Workup
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]
Treatment
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]
Prognosis
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
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
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. Sury, M. R. J., Worley, A., & Boyd, S. G. ( 2014 ). [biorxiv.org]
Pathophysiology
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]
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]
Rehabilitation starts with assessment of the functional impairment and the underlying pathophysiology by using all modern diagnostic tools. [naric.com]
Prevention
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]
The goals of this research are to find ways to prevent, treat, and cure these disorders. [childneurologyfoundation.org]
[…] nutrition Providing physical therapy to prevent limb contractures There is no specific treatment. [msdmanuals.com]