Amyotrophic Lateral Sclerosis - Anesthesia

1. Medicine (Baltimore). 2021 Oct 29;100(43):e27621.

Transversus abdominis plane block provides effective and safe anesthesia in the cesarean section for
an amyotrophic lateral sclerosis parturient: A case report.
Wang Y(1), Zhang Y, Li S, Chen L, Jiang J.
RATIONALE: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease
with the fatal course of muscle weakness. The published experience of anesthesia
management in the cesarean section with ALS parturient is scant. PATIENT CONCERNS: A 34-
year-old woman was admitted to our center complaining of obvious dysphagia together with
atrophy and weakness of quadriceps at 24 weeks of her pregnancy. Cesarean was planned at
36 weeks' gestation due to the rapid deterioration of the mother. DIAGNOSES: The results of
neurological examination, electromyography and spinal magnetic resonance imaging suggested
ALS according to the EI Escorial World Federation of Neurology criteria. INTERVENTIONS:
Ultrasound-guided transversus abdominis plane block with 0.6 minimum alveolar
concentration sevoflurane was used in this ALS parturient during her cesarean section
procedure. OUTCOMES: This anesthesia strategy successfully met the demands of the surgery,
helped avoid prolonged ventilation and prevent maternal respiratory complications. LESSONS:
Transversus abdominis plane block with subanesthetic concentrations of sevoflurane can
provide effective and safe anesthesia in the cesarean section for a patient with ALS.

2. Muscle Nerve. 2020 Jul;62(1):70-75.

Paravertebral block for radiologically inserted gastrostomy tube placement in amyotrophic lateral
sclerosis.
Porter SB(1), McClain RL(1), Robards CB(1), Paz-Fumagalli R(2), Clendenen SR(1), Logvinov II(1), Hex
KO(1), Palmucci C(3), Oskarsson BE(3).
INTRODUCTION: Radiologically inserted gastrostomy (RIG) placement in patients with
amyotrophic lateral sclerosis (ALS) carries risks related to periprocedural sedation and
analgesia. To minimize these risks, we used a paravertebral block (PVB) technique for RIG
placement. METHODS: We retrospectively reviewed patients with ALS undergoing RIG
placement under PVB between 2013 and 2017. RESULTS: Ninety-nine patients with ALS
underwent RIG placement under PVB. Median (range) age was 66 (28 to 86) years, ALS
Functional Rating Scale-Revised score was 27 (6 to 45), and forced vital capacity was 47% (8%-
79%) at time of RIG placement. Eighty-five (85.9%) patients underwent RIG placement as
outpatients, with a mean postanesthesia care unit stay of 2.3 hours. The readmission rate was
4% at both 1 and 30 days postprocedure. DISCUSSION: PVB for RIG placement has a low rate of
adverse events and provides effective periprocedural analgesia in patients with ALS, the
majority of whom can be treated as outpatients.

3. Chin Med J (Engl). 2020 Jun 5;133(11):1371-1372.

Anesthetic management of a parturient with amyotrophic lateral sclerosis undergoing cesarean
section.
Li J(1), Zeng H, Li M, Wang J.

4. Korean J Anesthesiol. 2020 Jun;73(3):257-258.

Transverse abdominis plane block for surgical anesthesia in a patient with flail arm syndrome.
Mishra P(1), Govil N(1), Parameswaran P(1), Gupta B(1).

5. Rev Esp Anestesiol Reanim (Engl Ed). 2019 Dec;66(10):497-505.

Anesthetic management of the diaphragmatic pacemaker placement surgery. Our experience in the
Institut Guttmann.[Article in English, Spanish]
Alegret N(1), Serra P(2), Pessas JP(2), Curià X(3), Vidal J(4), Valero R(5).
BACKGROUND: The diaphragm pacemaker (DP) has proven its utility in replacing mechanical
ventilation in patients with chronic spinal cord injury (SCI) and amyotrophic lateral sclerosis
(ALS). The DP improves patient quality of life and reduces morbidity and mortality and their
associated health care costs. The anesthetic management of these patients and the
 particularities of the surgical procedure are challenging. The aim of our study is to analyze
anesthetic management and intraoperative complications in patients undergoing DP
placement in our hospital. METHODS: We performed a chart review of patients treated
between December 2007 and July 2017, recording the patients' preoperative status, anesthetic
management and intraoperative complications. RESULTS: The study included 16 patients (5
pediatric) undergoing DP implantation for chronic SCI (63%), ALS (25%) and other neurologic
conditions (12%). Abdominal laparoscopy was performed under general anesthesia, with
intravenous (87%) or inhalational (13%) induction and maintenance using total intravenous
(50%) or balanced (50%) anesthesia. Rocuronium was administered in one case to permit
airway management. Complications included: hypotension (50%), difficulties in mechanical
ventilation during laparoscopy (31%), pneumothorax (12.5%) and autonomic dysreflexia (6%).
CONCLUSIONS: DP placement under general anesthesia is a safe intervention in both adult and
pediatric patients. Complications derived from both the underlying disease and the surgical
technique may appear, and must be rapidly identified and treated to obtain a satisfactory
surgical outcome.

6. J Anesth. 2019 Apr;33(2):257-265.

Amyotrophic lateral sclerosis and anesthesia: a case series and review of the literature.
Hoeper AM(1), Barbara DW(1), Watson JC(2), Sprung J(1), Weingarten TN(3).
PURPOSE: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that
leads to death due to respiratory failure. This report describes the perioperative characteristics
of ALS patients who underwent procedures with anesthesia at our institution. METHODS: We
reviewed perioperative records of ALS patients who underwent procedures with anesthesia
from January 1, 2014, through December 31, 2015. RESULTS: Seventy-eight patients
underwent 89 procedures (71 procedures with monitored anesthesia care and 18 with general
anesthesia), including 45 gastrostomy tube placements and 18 bone marrow biopsies. Three
patients had prolonged duration of postoperative intubation related to preexisting respiratory
muscle weakness, and one patient with bilateral pneumothorax required tracheal reintubation
for respiratory distress. Four patients had prolonged duration of hospitalization. Three patients
were hospitalized for ALS-related complications, and one patient was hospitalized for
respiratory distress when pneumoperitoneum developed after gastrostomy tube placement.
Three of these patients died of complications attributable to ALS within 30 days of the
procedure. Twenty-nine (32.6%) procedures required minimal sedation (e.g., bone marrow
biopsy, cataract surgery) and were performed on an ambulatory basis. CONCLUSION: When
caring for patients with ALS, the perioperative team must be prepared to treat potentially
complex medical conditions that may not be directly related to the procedure and anesthetic
management. However, minor procedures performed with minimal sedation may be safely
performed on an ambulatory basis.

7. Int J Obstet Anesth. 2018 Nov;36:108-110.

Combined spinal-epidural anesthesia with non-invasive ventilation during cesarean delivery of a
woman with a recent diagnosis of amyotrophic lateral sclerosis.
Kock-Cordeiro DBM(1), Brusse E(2), van den Biggelaar RJM(3), Eggink AJ(4), van der Marel CD(5).
Amyotrophic lateral sclerosis is the most common neurodegenerative upper and lower motor
neuron disease in adults but is not common in women of child-bearing age. We present a case
of a pregnant woman who was diagnosed with amyotrophic lateral sclerosis and developed
respiratory distress at 32 weeks-of-gestation. She underwent a cesarean delivery under
combined spinal-epidural anesthesia with non-invasive ventilation. This resulted in a successful
outcome for both the mother and the baby.

8. Minerva Anestesiol. 2017 Nov;83(11):1216-1217.

Anesthesia and postoperative analgesia for a patient with amyotrophic lateral sclerosis.
Gu J(1)(2), Lin X(3)(2).
9. J Clin Anesth. 2017 Feb;36:107-109.
Total intravenous anesthesia without muscle relaxant in a parturient with amyotrophic lateral sclerosis
undergoing cesarean section: A case report.
Xiao W(1), Zhao L(2), Wang F(3), Sun H(4), Wang T(5), Zhao G(6).
Cases of amyotrophic lateral sclerosis with pregnancy are quite rare. The aim of this case
report is to present the successful use of total intravenous anesthesia without muscle relaxant
for cesarean section in a parturient with amyotrophic lateral sclerosis at 35 weeks' gestation.
The parturient presented with impaired neuromuscular function. Titrated general anesthesia
with short-acting anesthetics was applied. During the procedure, no muscle relaxant was used.
This strategy helped avoid prolonged ventilation and prevent maternal respiratory
complications.

10. Rev Esp Anestesiol Reanim. 2014 Jun-Jul;61(6):354-5.

[Comments to the article "thoracic paravertebral block plus pectoral nerve block for mastectomy in a
patient with advanced amyotrophic lateral sclerosis"].[Article in Spanish]
Fajardo Pérez M(1), Alfaro de la Torre P(2), López Álvarez S(3), Diéguez García P(3).

11. Rev Esp Anestesiol Reanim. 2014 Mar;61(3):172-3.

[Thoracic paravertebral block plus pectoral nerve block for mastectomy in a patient with advanced
amyotrophic lateral sclerosis].[Article in Spanish]
Díaz A(1), Ledesma B(2), Gilsanz F(2).

12. Minerva Anestesiol. 2013 Jul;79(7):822-3.

Thoracic paravertebral block for breast surgery in a patient with amyotrophic lateral sclerosis.
Agnoletti V, Corso R, Cattano D, Novi A, Piraccini E, Folli S, Gambale G.

13. Med Princ Pract. 2013;22(3):304-6.

Use of sugammadex in a patient with amyotrophic lateral sclerosis.
Kelsaka E(1), Karakaya D, Zengin EC.
OBJECTIVE: To report on general anesthesia management in amyotrophic lateral sclerosis.
CASE PRESENTATION AND INTERVENTION: A 47-year-old man presented with fracture of the
humerus. The patient was diagnosed with amyotrophic lateral sclerosis. General anesthesia
was induced with propofol, rocuronium and remifentanil. After uneventful surgical repair, TOF
(train-of-four) ratio reached >0.90 at the end of operation. However, muscle strength and tidal
volume were inadequate. After sugammadex 2 mg kg(-1) i.v. was given, the patient was
extubated 120 s later. CONCLUSION: This case highlights that rocuronium and sugammadex
can be used safely in patients with amyotrophic lateral sclerosis undergoing surgery with
general anesthesia.

14. J Anesth. 2012 Apr;26(2):314-5.

Peripheral nerve block in a patient with amyotrophic lateral sclerosis.
Sertöz N, Karaman S.

15. J Clin Anesth. 2010 Nov;22(7):549-52.

Laparoscopic diaphragmatic pacer placement--a potential new treatment for ALS patients: a brief
description of the device and anesthetic issues.
Schmiesing CA(1), Lee J, Morton JM, Brock-Utne JG.
The Diaphragm Pacing Stimulator (DPS) has been used to treat ventilatory insufficiency in
quadriplegic patients. The FDA approved a trial using the DPS in patients with amyotrophic
lateral sclerosis (ALS). Three patients with advanced ALS, who underwent laparoscopic
diaphragmatic pacer placement, and their general anesthetic management, are presented.

16. Rev Bras Anestesiol. 2009 Mar-Apr;59(2):206-9.

Combined spinal-epidural block in a patient with amyotrophic lateral sclerosis: case report.
Hobaika AB(1), Neves BS.
 BACKGROUND AND OBJECTIVES: Amyotrophic lateral sclerosis starts between the fifth and
sixth decades of life, causing degeneration and death of upper and lower motor neurons.
When the muscles responsible for ventilation are affected, the patient dies of respiratory
failure within a few years. CASE REPORT: This is a 63 years old female with amyotrophic lateral
sclerosis who underwent surgical treatment of a transtrochanteric fracture of the femur. The
patient presented weakness of upper and lower limbs and dysarthria, and she was awake and
oriented. Respiratory function: ineffective cough, decreased strength of the intercostal muscles
and diaphragm, and reduction of the breath sounds in both lung bases. Initially, the L3/L4
epidural space was punctured and a silicon catheter was introduced to 5 cm. This was followed
by a spinal puncture in the L4/L5 space and the administration of 7.5 mg of hyperbaric
bupivacaine. This was followed by the administration of 37 mg of 0.37% ropivacaine through
the epidural catheter for a sensitive blockade up to T10. The procedure evolved without
complications and the patient was discharged from the hospital after three days.
CONCLUSIONS: The evidence has demonstrated that neuroaxis blocks can be safely performed
in patients with amyotrophic lateral sclerosis since it avoids manipulation of the airways and
respiratory complications.

17. Am J Surg. 2009 Mar;197(3):386-90.

Amyotrophic lateral sclerosis: the Midwestern surgical experience with the diaphragm pacing
stimulation system shows that general anesthesia can be safely performed.
Onders RP(1), Carlin AM, Elmo M, Sivashankaran S, Katirji B, Schilz R.
BACKGROUND: There is a paucity of literature concerning general anesthesia and surgery in
patients with amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease). This report
summarizes the largest series of surgical cases in ALS during multicenter prospective trials of
the laparoscopic diaphragm pacing system (DPS) to delay respiratory failure. METHOD: The
overall strategy outlined includes the use of rapidly reversible short-acting analgesic and
amnestic agents with no neuromuscular relaxants. RESULTS: Fifty-one patients were implanted
from March 2005 to March 2008 at 2 sites. Age at implantation ranged from 42 to 73 years and
the percent predicted forced vital capacity (FVC) ranged from 20% to 87%. On preoperative
blood gases, Pco(2) was as high as 60. Using this protocol, there were no failures to extubate or
30-day mortalities. The DPS system increases the respiratory system compliance by decreasing
posterior lobe atelectasis and can stimulate respirations at the end of each case.
CONCLUSIONS: Laparoscopic surgery with general anesthesia can be safely performed in
patients with ALS undergoing DPS.

18. Rev Esp Anestesiol Reanim. 2008 Feb;55(2):124-5.

[Paravertebral block for major breast surgery in a patient with amyotrophic lateral sclerosis].[Article in
Spanish]
Gómez Fernández M, Mateos Arribas T, Cuello Azcárate J, Vega Cruz S.

19. J Anesth. 2008;22(4):443-5.

Total intravenous anesthesia without muscle relaxant in a patient with amyotrophic lateral sclerosis.
Lee D(1), Lee KC, Kim JY, Park YS, Chang YJ.
A 63-year-old woman with amyotrophic lateral sclerosis (ALS) was scheduled for open
reduction and internal fixation of the right tibia. Total intravenous anesthesia using propofol
and remifentanil without muscle relaxant was selected as the anesthetic method, in order to
avoid the possible occurrence of ventilatory depression due to abnormal responses to muscle
relaxants and exacerbation of the motor neuron disease. After standard and neuromuscular
monitoring devices were applied, anesthesia was induced and maintained with target
controlled infusion of propofol and remifentanil in the range of 2.5-5.0 microg x ml(-1) and 2.5-
5.0 ng x ml(-1), respectively. To avoid delayed neuromuscular recovery, we did not use any
muscle relaxant at all. Intubation was successful and there were no remarkable events during
anesthesia, except for three brief hypotensive events; there was no exacerbation of ALS itself
during or after the anesthesia. She was discharged on postoperative day 3, without any
discomfort.
20. J Anesth. 2006;20(2):109-12.
Effects of lumbar sympathetic ganglion block for a patient with amyotrophic lateral sclerosis (ALS).
Kitoh T(1), Kobayashi K, Ina H, Ofusa Y, Otagiri T, Tanaka S, Ono K.
A 59-year-old man with amyotrophic lateral sclerosis (ALS) received lumbar epidural and
sympathetic ganglion blocks to increase regional blood flow and improve his clinical symptoms.
After a lumbar epidural block (0.5% mepivacaine), the skin temperature of his affected lower
extremities rose by 7.0 degrees C and became close to that of the intact side, and the distance
he was able to walk with his cane increased from 2 to 8 m. The clinical effects produced by the
lumbar sympathetic ganglion block (99.5% alcohol) were sustained for approximately 8 weeks
after the first block and for approximately 6 weeks after the second block. There were no
particular adverse effects or complications associated with these nerve block procedures.
Epidural and sympathetic ganglion blocks for an ALS patient, albeit their effects are of a
transient nature, may improve related clinical symptoms, and were thought to play a
contributory role in improving our patient's quality of life.

21. Eur J Anaesthesiol. 2004 Nov;21(11):921-3.

General anaesthesia in a patient with motor neuron disease.
Moser B, Lirk P, Lechner M, Gottardis M.

22. Anesth Analg. 1996 Oct;83(4):878-9.

Epidural anesthesia and pulmonary function in a patient with amyotrophic lateral sclerosis.
Hara K(1), Sakura S, Saito Y, Maeda M, Kosaka Y.

23. Minerva Anestesiol. 1991 Sep;57(9):747-8.

[Subarachnoid anesthesia in a case of cesarean section in a 27-year old patient with amyotrophic
lateral sclerosis, in the 39th week of pregnancy].[Article in Italian]
Moret JE(1), Di Gioia M, Montaruli V.

24. Anesth Analg. 1989 Mar;68(3):410-2.

Epidural anesthesia for patients with amyotrophic lateral sclerosis.
Kochi T(1), Oka T, Mizuguchi T.

25. Anesth Analg (Paris). 1979 May-Jun;36(5-6):235-8.

[Aggravation following anaesthesia in a case of unknown lateral amyotrophic sclerosis (author's
transl)].[Article in French]
Nicolas F, Sollet JP, Mathé JF.
A 66 year old man whose activity was almost normal underwent a lombar sympathectomy
under general anesthesia (thiopental, gallamine, N2O). Immediately after recovery an acute
respiratory distress became evident. It was due to a respiratory paralysis on which neostigmine
had no effect. Neurologic examination showed the symptoms of an amyotrophic lateral
sclerosis which had remained unknown so far. The respiratory paralysis persisted and one year
later artificial ventilation is still necessary almost continuously. Two aspects of this very rare
observation are discussed: first the cause and mecanisms of the aggravation of the neurologic
disease following anesthesia, secondly the rather unusually important part played by the
respiratory paralysis in this case of lateral amyotrophic sclerosis.

26. Anesth Analg. 1971 May-Jun;50(3):431-7.

Circulatory collapse following succinylcholine: report of a patient with diffuse lower motor neuron
disease.
Beach TP, Stone WA, Hamelberg W.