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Complete Locked-in Syndrome

CLIS


Presentation

  • 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]
  • 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]
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]
  • 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]
  • Darren Leith, a painter from Cumbria, England, suffered the stroke in April 2017.[msn.com]
  • 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]
  • He is the son of painter Alice Lok Cahana. [5] Lynsey Cribbin [ edit ] Lynsey Cribbin, from Cavan, Ireland, woke up with several headaches in January 2012.[en.wikipedia.org]
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]
Internal Bleeding
  • Finally, those who did pass away had various complications including heart disease, multisystem failure, pneumonia, respiratory failure, internal bleeding and aspiration [5].[physio-pedia.com]
Respiratory Insufficiency
  • Respiratory insufficiency requires the use of mechanical ventilation. The most common cause of brainstem stroke is a thrombosis of the basilar artery. Other causes include but trauma or cardiocirculatory failure.[radiopaedia.org]
Vomiting
  • 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]
  • It was found after unexplained spells of severe headaches and vomiting. The aneurysm was treated with a balloon occlusion and the prognosis was good.[newsarchive.heart.org]
  • The brain stem handles basic functions like breathing, swallowing, heart rate, blood pressure, sleeping and vomiting. The brain stem does not play a part in higher cognitive functions.[strokeconnection.strokeassociation.org]
Abdominal Pain
  • Or abdominal pain? Or earache? There is no communication system existing in India for locked-in-syndrome. Though we tell our selves God has a purpose, it is extreme torture, pathetic and heart breaking.[bmj.com]
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]
Vascular Disease
  • Disease and Quality of Care Outcomes in Research Interdisciplinary Working Groups: the American Academy of Neurology affirms the value of this guideline as an educational tool for neurologists.[medscape.com]
Osteoporosis
  • As age advances, osteoporosis sets in and multiple fractures of vertebra of her thoracic and lumbar spine occured. It requires exposure to sunlight and hormone treatment. She has a tracheostomy for 27 years.[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]
Headache
  • But early the next morning, the headache returned with pain so intense, his parents took him to the local hospital. It was another brain aneurysm.[newsarchive.heart.org]
  • Only five hours after Daniela gave birth, she started having headaches and went into a coma. 'I immediately panicked,' remembers Luigi, 'because I had no idea what was happening and the doctors didn't either.[eurordis.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]
  • He is the son of painter Alice Lok Cahana. [5] Lynsey Cribbin [ edit ] Lynsey Cribbin, from Cavan, Ireland, woke up with several headaches in January 2012.[en.wikipedia.org]
  • "I had a particularly bad headache at work one day," says Lorna, communicating via a special computer. "The ache was centred round the lower part of my head, at the back, and my neck. I also couldn't speak.[news.bbc.co.uk]
Gait Ataxia
  • Patient 2: One week after influenza vaccination followed by diarrhoea, a 78 year old woman suddenly experienced double vision, difficulty in swallowing and gait ataxia. Laboratory investigations were negative.[bmj.com]
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]
  • 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]
  • Other authors, based in their experiences, do not recommend this treatment.[naric.com]

Prognosis

  • The aneurysm was treated with a balloon occlusion and the prognosis was good. Patrick was told to avoid contact sports, but encouraged to participate in cardiovascular activity to help his body develop new blood vessels.[newsarchive.heart.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]
  • 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

  • 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]
  • 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]
  • No statistical relationships of QoL with disease duration, LIS type, or LIS etiology were found.[ojrd.biomedcentral.com]
  • : traumatic or other non-traumatic etiology.[biomedical-engineering-online.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]
Sex distribution
Age distribution

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]
  • 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]
  • 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]

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]
  • […] horror condition THE horrific condition prevents sufferers from communicating or moving, but there is hope thanks to a recent breakthrough 12th July 2018, 10:20 am Updated: 12th July 2018, 10:23 am LOCKED-IN syndrome hit the headlines in 2o17, after new[thesun.co.uk]

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