Comparison of Communication Ability Stage with Adverse Clinical Signs in Patients with Amyotrophic Sclerosis (ALS) on Tracheostomy Invasive Ventilatio Yuki Nakayama1, Chiharu Matsuda1, Toshio Shimizu², Yoko Mochizuki3,4, Kentaro Hayashii², Mas Akihiro Kawata², Kiyomitsu Oyanagi5, Imaharu Nakano² 1. Laboratory of Nursing Research for Intractable Disease, Tokyo Metropolitan Institute of Medical Science,  2. Department of Neurology, Tokyo Metropiolitan Neurofogical Has 3. Department of Pathology,Tokyo Metropolitan Neurological Hospital,  4. Department of Neurology, Tokyo Metropolitan Kita Medical and Rehabilitation Center forthe Disabled 5. Department of Brain Disease Research, Shinshu University School of Medicine Patients with amyotrophic lateral sclerosis (ALS) on tracheostomy invasive ventilation (TIV) : Lives long time 1) Have adverse clinical signs Develop communication impairment with disease progression  2) The appropriate care and to maintain communication ability become important a classification of advanced ALS focusing on the patient’s communication ability. 3) Number of ALS patients and ventilator users in Tokyo Duration of Ventilation becomes long Stage of communication ability Stage I II III IV V
1)  Tokyo Metropolitan specific resarch for Intractible disease ,2013    2)  Nakayama Y, Ogura A, Matsuda C. J. J. Intrac. Ill. Nurs 2010;14:179-93.  Hayashi et al.2013 3)  Hayashi K, et al. Rinsho Shinkeigaku 2013; 53:98-103. 2. Objective To clarify the relationship between the stages of communication ability and adverse clinical signs in ALS patients on TIV. Our multi-disciplinary research team 3). Methods Subject : 46 patients with ALS on TIV at home from 2005 to 2013. Collected Data : sex, age of onset, duration of disease, duration of TIV, and time from onset to start of TIV.  Incidence of adverse clinical signs Data analysis : Classified subjects into three groups by stage of communication ability at the last observation point. Comparison of three groups by the the Kruskal-Wallis test  performed using SPSS, version 21. p-values ≤0.05 were considered significant. Voluntary movement • fatigability of eye movement • dry eye •  drooling or dry mouth • megaloglossia Infections • otitis media • Pneumonia •  urinary tract infections Autonomic dysfunction •  unstable blood pressure •  disturbance of thermoregulation • dysuria Others • tracheal granuloma • Cholelithiasis •  urinary stones • Decubitus • Nausea • unstable blood glucose 4. Results
Voluntary movement 100% Time from onset to start TIV * Autonomic dysfunction stage I stage II-IV stage V P:  ×  < 0.05 ** ＜0.01 *** ＜ 0.001 We found that the patients with ALS in advanced stages showed an early requirement for TIV had more adverse signs. Clinicians should give careful attention to the adverse signs of ALS patients on TIV to prevent communication impairment. Others This work supported by JSPS KAKENHI Grant Number 25293449. 5. Discussion Several type of ALS patients Who maintain stage I Ventilator needs slowly Had not many adverse clinical signs Who progress stage II~IV or V Rapid disease progression Ventilator needs  early phase Had various adverse clinical signs Number of signs *** Infections Relationship between Adverse Clinical Signs and progress Communication Impairment in Patients with Amyotropfiic Sclerosis (ALS) on Tracheostomy Invasive Ventilation (g Y Nakayama¹, T Shimizu², C Matsuda¹,  M Haraguchi¹, Y Mochizuki³.4,  K Hayashi², T Hirai², M Nagao², A Kawata², K Oyanagi5 1. Laboratory of Nursing Research for Intractable Disease, Tokyo Metropolitan Institute of Medical Science,  2. Department of Neurology, Tokyo Metropolitan Neurology:QLHöspi 3. Department of Pathology,Tokyo Metropolitan Neurological Hospital, 4. Department of Neurology, Tokyo Metropolitan Kita Medical and Renabilitation Center forthe firsthe fir 5. Department of Brain Disease Research, Shinshu University School of Medicine Summary of this study We found that the patients stages showed an early appearance of opthalmoplegia and multisystem neurodegeneration. Clinicians should give careful attention to the adverse signs of ALS patients on TIV to prevent communication impairment. 3 . Methods Subject : 60 patients with ALS on TIV at home from 2005 to 2014. Stage of communication ability
Impossible Havashi K, Mochizuki Y, Nakayama Y, et al. Communication disorder in amyotrophic lateral sclerosis after ventilation —— a proposal of staging and a study of predictive factor. Rinsho Shinkeigaku 2013 2. Objective Purpose of this study, to clarify which adverse clinical signs are affected communication stage  progression in ALS patients on TIV. Collected Data : sex, age of onset, duration of disease, duration of TIV, and time from onset to start of TIV. Incidence of adverse clinical signs. Voluntary movement • fatigability of eye movement • dry eye •  drooling or dry mouth • megaloglossia 16 adverse clinical signs Autonomic dysfunction • unstable blood pressure • disturbance of thermoregulation • dysuria Others Infections • otitis media • pneumonia •  urinary tract infections • tracheal granuloma • cholelithiasis •  urinary stones • decubitus • nausea • unstable blood glucose 4. Results 19(31.7%)  of the patients progressed stages IV-V. Their duration from onset to opthalmoplegia was early and had more adverse clinical signs. Patients Characteristics
Disease duration and communication stage of 60 patients Stage Ill Stage IV → Stage I → Stage Il
Data analysis : Classified subjects into two groups(Stages I-III and IV-V) by stage of communication ability at the end point. Cox proportional hazard model was used to compare between two groups. Data analysis was performed using SPSS, version21. p-values ≤0.05 were considered signifi- cant. Incidence of Adverse Clinical Signs 100 (%)件< 0.001 Univariate  analyses for Stage!V by Cox proportional hazarad model Vicious circle of Adverse Clinical signs and Communication ability Voluntary movement disorder by progression of ALS it’s own. Adverse clinical signs Eye signs Autonomic dysfunctions Getting worse eye movement disorder Can’t tell own idea to others Relation to Communication Instability of General conditions Need for nursing care to maintain genaral conditions Several types of ALS patients Who progress stages IV –V had more adverse clinical signs unstable blood pressure disturbance of   thermoregulation dysuria This suggested that stages IV or V shows multisystem neurodegeneration involving the oculomotor nuclei and limbic system. Future study is required to related ALS-plus. * < 0.001 Study Supported by: JSPS KAKENHI (Grant-in-Aid for Scientific Research (B)) Grant Number 25293449 No potential Conflict of Interest (COI) to disclose
Multivariate analyses for Stage!V by Cox proportional hazarad model P: with disease progression The adverse clinical signs affect to maintain communication ability. We proposed  a classification of advanced ALS focusing on the patient’s communication ability. 1 . Background Patients with ALS on TIV : ı Stage V Respirator-aided long-term survival cases of amyotrophic lateral sclerosis (ALS) with communication abilities, motoneuron system-confined degeneration, and scanty TDP-43 aggregation ---a subgroup of ALS? Mochizuki Y¹, 6, Hayashi K², Nakayama Y³, Shimizu T², Kamide M², Ogino M³, Komori T¹, Isozaki E², Nakano I² Department of 'Pathology and ?Neurology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan, 3Laboratory of Nursing Research for Intractable Disease, Tokyo, Japan, *Emergency Department of Atsugi City Hospital, Kanagawa, Japan, *Department of Neurology, Kitasato University School of Medicine, Kanagawa, Japan, §Department of Neurology, Tokyo Metropolitan Kita Medical and Rehabilitation Center for the Disabled, Tokyo, Japan Not all the patients with ALS on long-term mechanical ventilation (LTMV) develop into a totally locked-in state Totally-locked in state (Communicaion stage V) About 13% of cases on LTMV Very rapid progression ♪ Lost of all voluntary movements → Can not communicate by any means >  Severe degeneration of motor neuron system .> Multiple system degeneration beyond motor neuron system Maintain communication abilities What type of ALS? an approximately 30-year survival and good communication abilities were clinicopathologically investigated. Comparison with cerebral, brain stem and spinal cord findings of stage V Several types of long-duration ALS On TIV
Mean disease duration of ALS on TIV (61 cases) was 8 year 5 months in our 134 autopsied ALS ✓ Rapid progress into a totally locked-in state/ stage V [Hayashi H, Kato S. J Neurol Sci 93:19, 1989] ✓ Lower motor-predominant and illness ranged from10 to 20 without TIV [Kato et al. Brain Nerve 45:267-272, 1993, Nishihara et al Acta Neuropathol 117:45-53, 2009] All three cases could communicate in entences via eye movements until death mplete tetraplegia 10 Disease duration (years) mal-looking motor neurons remained. Neither hypertrophic astrocytes nor macrophages were observed. There were no TDP-43-immunoreactive inclusions in the lower motor neurons and occasional ones in non-motor neurons in case 3. There wore no Bunina bodies. These findings are different from findings of classical ALS. A distinct subgroup of ALS?
Neuropathologocally ♦Motor neuron system-confined degeneration ♦Remaining good-shaped motor neurons ♦A few TDP-43 immunoreactive inclusions This work supported by JSPS KAKENHI Grant Number 25293449