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Chronic Neurological Problems – Chapter 61 (Ouellette, 2019; 2023)
Multiple Sclerosis (MS)
http://mssociety.ca/
Define Chronic, progressive, degenerative
disorder of the CNS
Characterized by disseminated
demyelination of nerve fibres of the
brain, spinal cord and the optic nerves
MS was first identified and described by
French neurologist Jean-Martin Charcot
in 1868.
Incidence/ Prevalence Over 77, 000 Canadians (20 or older)-
highest rates in the world
1/500-1000
Age of onset: 20-50 years; often
symptom onset 30-35 years;
75% women
Etiology Cause unknown*
May be related to: environmental &
infectious (viral) factors; dietary
deficiency (vit. D); immunological &
genetic factors; intrinsic factors (e.g.
faulty immunoregulation)
Susceptibility may be inherited (multiple
genes)
Possible precipitating factors: infection;
Parkinson’s Disease (PD)
http://www.parkinson.ca
Disease of the basal ganglia
Characterized by a slowing-down in the
initiation and the execution of movement
(bradykinesia), increased muscle tone
(rigidity), tremor at rest, & impaired
postural reflexes.
Named after James Parkinson who wrote
classic essay on “shaking palsy” in 1817
~84, 000 Canadians
Age of onset usually around 60, but can
occur as young as 20;
85% 65 or older;
3 men: 2 women
Well-established genetic component
Parkinsonism also can be caused by
intoxication with carbon monoxide &
manganese, drug induced , encephalitis,
meningitis, vascular, multiple system,
dementia with Lewy bodies
Amyotrophic Lateral Sclerosis (ALS)
http://www.als.ca/
A rare progressive fatal neurological
disorder; characterized by loss of motor
neurons and by weakness and atrophy of
the muscles of the hands, the forearms,
and the legs, spreading to involve most of
the body and the face.
People living with the disease become
progressively paralyzed due to
degeneration of the upper and lower
motor neurons in the brain and spinal
cord
Usually leads to death within 2-6 years
after diagnosis due to inability to breath
or swallow
Also known as Lou Gehrig’s disease
(1940’s famous baseball player who had
the disease)
4 to 7 per 100, 000 worldwide
~3000 Canadians currently have ALS; 2-3
die each day, usually within 2- years of
diagnosis
Exact cause unknown

trauma; emotional stress; excessive
fatigue; pregnancy; state of poor health
Pathophysiology Chronic inflammation, demyelination,
and gliosis (scarring) in the CNS
Autoimmune disease related to
autoreactive T cells
Virus may trigger process
Clinical manifestations Onset often insidious
Vague symptoms occur intermittently
over months or years
Chronic, progressive deterioration in
some vs remission and exacerbation in
others
Overall trend is progressive deterioration
in neurological function
motor: weakness or paralysis of limbs,
trunk or head; diplopia; scanning speech;
muscle spasticity
sensory: numbness & tingling;
paresthesias; patchy blindness; blurred
vision; vertigo; tinnitus; decreased
hearing; chronic neuropathic pain;
Lhermitte’s sign;
cerebellar signs-nystagmus, ataxia;
dysarthria; dysphagia;
severe fatigue present in many;
urinary problems: spastic bladder-
urgency, frequency, dribbling or
incontinence; flaccid bladder
bowel dysfunction: usually constipation
sexual dysfunction;
emotional stability may be affected;
cognitive functioning generally remains
Degeneration of dopamine –producing
neurons in the substania nigra of the
midbrain with Lewy bodies present in the
residual dopaminergic neurons
Normal balance between dopamine (DA)
and acetylcholine (Ach) in the basal
ganglia is disrupted
Normal functioning of the extrapyramidal
motor system (posture control, support
and voluntary movement) is affected
Symptoms appear when 80% of neurons
in substania nigra are lost
Onset is insidious, with a gradual
progression and a prolonged course
At beginning-mild tremor, slight limp,
decreased arm swing
Later-shuffling, propulsive gait with arms
flexed
Classic triad: tremor (70-100%); rigidity
(90%); bradykinesia (80-100%)
Hand tremor-“pin rolling”
Cogwheel rigidity
Loss of automatic movements-blinking,
swinging arms when walking, swallowing
saliva, self-expression with facial and
hand movements, lack of spontaneous
activity (stooped posture, masklike face)
drooling, shuffling gait (festination);
difficulty initiating movement
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Motor neurons in the brainstem and
spinal cord gradually degenerate
Electrical and chemical signals originating
in the brain don’t reach the muscles
Typical: weakness in upper extremities,
dysarthria, and dysphagia
Weakness may also begin in the legs
Muscle wasting and fasciculations result
from the deinervation of the muscles and
lack of stimulation and use
Death usually results from respiratory
infection secondary to the compromised
respiratory function
Devastating as client can remain
cognitively intact while wasting away;
Can cause some cognitive (30-50%) and
behavioral changes in some people;
20-25% can acquire severe cognitive
impairment;
Uncontrolled laughing/crying
While pain is not commonly reported by
people living with ALS, some people
experience physical pain, joint discomfort
or cramping. Movement exercises and
keeping warm can help alleviate the
feelings of pain. Medication may also be
needed in some cases.
https://www.als.ca/about-als/what-is-
als/symptoms/

intact
Life Expectancy 25 yrs after onset of symptoms
Complications Death usually related to complications
(e.g. pneumonia) of immobility or
because of unrelated disease
Diagnostic Studies No definitive test
Dx based on hx and clinical
manifestations & presences of multiple
plaques over time as measured by MRI
CSF may show ↑oligoclonal
immunoglobulin G &↑lymphocytes and
monocytes
Evoked potentials are delayed
Characteristic white-matter lesions
through brain or spinal cord (MRI)
To be diagnosed: 2 lesions in 2 CNS
locations; damage occurring at different
times; all other dx ruled out
Collaborative No cure
The life expectancy is lower than for
people who do not have the disease
Depression, anxiety, apathy, pain
Motor symptoms (e.g. dyskinesias or
involuntary movements); weakness;
akinesia; dementia (40%); depression;
hallucinations; psychosis; dysphagia;
malnutrition or aspiration; sleep
disorders;
No specific diagnostic tests
Dx based on hx and clinical
manifestations
Firm dx: requires 2 of the 3 characteristic
signs of the classic triad
No cure
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Usually leads to death within 2-6 years
after diagnosis
Spasticity, cramps, constipation, drooling,
uncontrolled laughing/crying, pain,
depression, anxiety, fatigue
Respiratory failure and death
Pseudobulbar affect, or emotional
lability, is characterized by uncontrolled
and inappropriate crying or laughing. It is
thought to occur as a result of lost
inhibition of the limbic motor neurons in
the brain which control muscles involved
in primitive vocalization. Emotional
lability is not a mood disorder, but
abnormal affective display. Physicians
must be alert to pseudobulbar affect as it
is often not recognized as part of ALS: it
can be confused with depression; and
patients often do not report symptoms,
yet as many as 50% of ALS patients
experience pseudobulbar affect
Research on protein biomarkers
The presence of upper and lower motor
neuron signs in a single limb is strongly
suggestive. However, an ALS diagnosis is
based primarily on the signs and
symptoms that appear and on a series of
tests that rule out other diseases.
Electromyography (EMG); Nerve
conduction velocity (NCV); Magnetic
resonance imaging (MRI); Blood and
urine tests; Muscle biopsy.
ALS has no known cure or effective

or Care aimed at treating disease process
Interprofessional Care and symptom relief
Drug Therapy Adrenocorticotropic hormone,
methylprednisone, prednisone (↓edema
& acute inflammation
Immunosuppressive drugs (e.g. Imuran)
benefit some patients with progressive-
relapsing, secondary-progressive, and
primary-progressive, but serious side-
effects
Immunomodulator drugs modify disease
process
Antineoplastic drug –Novantron-cardiac
toxicity
Antispasmodics (spasms), CNS stimulants
(fatigue); anticholinergics (bladder
symptoms); TCAs (chronic pain);
cannaboids (pain, spasticity)
(see table 61-15 or 61-16, for list of
drugs)
Medication Alert: Interferon Beta Meds
(Avonex)-rotate injection sites; assess for
depression/suicidal ideation; sun
protection; anticipate flu-like symptoms
Surgical Therapy Neurectomy-resection of all or part of a
nerve
Rhizotomy -nerve root section to relieve
pain or reduce spasticity
Cordotomy-spinal cord section of lateral
pathways to relieve pain
Thalamotomy-destruction of part of the
thalamus-to treat intractable pain or
relieve spasticity
Care aimed at treating disease process
and symptom relief
Drug therapy is aimed at enhancing the
release or supply of DA or blocking
effects of cholinergic neurons
Sinemet (levidopa with carbidopa often
1st drug-effectiveness may wear off after
a few years
(see table 61-19 for list of drugs)
Three categories:
1.Ablation (destruction);
2. deep brain stimulation (DBS);
3. transplantation: fetal neural tissue into
brain (experimental, ethical dilemma);
stem cell transplantation
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treatment yet.
Multidisciplinary care team at ALS centre
can help people live longer with ALS (PT,
OT,SLP, dietician, RT, SW) e.g. London
ON; Hamilton ON
Radiation may help control drooling
Riluzole-modestly slows rate at which
disease worsens; prolongs survival by 2-3
(some sources day 3-6 ) months; safe but
expensive
Radicava (edaravone) approved in US
2017 and in Canada 2018;
Slows progression by 33%; administered
IV daily for 10 days every month; thought
to reduce build-up of antioxidants
Dextromethorphan and quinidine may ↓
episodes of uncontrolled laughing/crying

Other Therapies Dorsal column electrical stimulation
Intrathecal baclofen (CSF space)
Deep brain stimulation
Physical & speech therapy
Exercise improves daily functioning
Water exercise-gives buoyancy
Nutritional therapy; megavitamin(B12, vit
C) supplemental vit D; diets low in fat,
gluten free, raw vegetables (insufficient
evidence but nutritious well-balanced
diet is important e.g. high protein, high
roughage with vitamin supplements and
adjust for chewing and swallowing status;
Other: heat, massage, acupuncture,
aromatherapy, apitherapy (bee stings)
(more efficacy research required)
Stem cell therapy: new research suggests
reduction in relapses
Note: CCSVI-no supporting research; see
footnote below*
Nursing Considerations Client admitted for diagnostic workup
and treatment of an acute exacerbation
Prevent complications of immobility
Maximize function & independence;
manage fatigue; optimize mental health
and well-being; identify and reduce
triggers for exacerbation
Health Teaching Build up resistance to illness
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Nutritional therapy to combat
malnutrition and constipation;
Appetizing foods that are easy to chew &
swallow with fibre and fruit (reduce
constipation); 6 small meals ? less tiring;
bite sized pieces;
Ingestion of protein and vitamin B6 can
limit absorption of levodopa-limiting
protein ingestion to evening meal may be
advisable; consult team re supplements
or cereals that contain B6
Genome Therapy: experimental
Promoting physical mobility Challenge: guide client in use of
Developing communication to meet moderate-intensity endurance types of
individual needs exercises for the trunk and the limbs, as
Dysphagia precautions may help with spasticity
Problems secondary to bradykinesia: Support cognitive and emotional
freezing-teach client to think about functions
stepping over imaginary or real lines on Facilitate communication
the floor, drop rice kernels and step over Reduce risk of aspiration
them, rock side to side, lift the toes when Pain management
stepping, take one step backward and Falls prevention
two steps forward Patient/family emotional support as per
Assess for levodopa overdose when wishes/concerns
freezing occurs
Facilitate getting out of the chair by using
upright chair with arms and place the
back legs on small blocks (2 inches)
Promotion exercise and a well-balanced See ASL MD/Interprofessional Care
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Avoid fatigue, extreme Temp., & diet Guidelines

exposure to infection
Balance exercise rest
Eat well-balanced meals
Avoid hazards of immobility (e.g.
pressure ulcers)
Bladder interventions (e.g. self-cath.)
Bowel (prevention constipation)
MS Society of Canada resources
Family counseling may be necessary
High incidence of marriages ending
Parkinson’s Society of Canada resources https://www.als.ca/about-als/resources/
Consultation with PT and OT as necessary for-physicians/
Remove fall hazards from environment
Encourage slip on footwear and avoid Living with ALS
clothing with challenging buttons or https://www.als.ca/about-als/resources/
hooks living-with-als/
Elevated toilet seat
Work with client/family to maximize
independence as per personal
preferences/concerns

References

Amyotrophic Lateral Sclerosis Society of Canada. (2017). A guide to ALS for primary care physicians.
https://als.ca/wp-content/uploads/2017/02/A-Guide-to-ALS-Patient-Care-For-Primary-Care-Physicians-English.pdf

Ouellette, D. (2019). Nursing management: Chronic neurological problems. In M. A. Barry, J. L. Lok, J. Tyerman, & S. Goldsworthy (Eds.), Medical-surgical nursing in Canada:
Assessment and management of clinical problems (4th ed., pp. 1534-1564). Elsevier.

Ouellette, D. (2023). Stroke. J. Tyerman, & S. L. Cobbett (Eds.), Medical-surgical nursing in Canada: Assessment and management of clinical problems (5th ed., pp. 1511-1540).
Elsevier.

Shoesmith, C., Abrahao, A., Benstead, T., Chum, M., Dupre, N., Izenberg, A., Johnston, W., Kalra, S., Leddin, D., O’Connell, C., Schellenberg, K., Tandon, A., & Zinman, L. (2020).
Canadian best practice recommendations for the management of amyotrophic lateral sclerosis. Canadian Medical Association Journal, 192, E1454-1468. doi:
10.1503/cmaj.191721 https://www.cmaj.ca/content/cmaj/192/46/E1453.full.pdf

Footnotes:

Scanning speech: that in which syllables of words are separated by noticeable pauses.

Lhermitte’s sign: a transient sensory symptom described as an electric shock radiating down the spine or into the limbs with flexion of the neck.
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Nystagmus: an unintentional jittery movement of the eyes. Nystagmus usually involves both eyes and is often exaggerated by looking in a particular direction.

*Paolo Zamboni, a professor of medicine at the University of Ferrara in Italy proposes new theory: the underlying cause of MS is a condition he has dubbed “chronic
cerebrospinal venous insufficiency.” If you tackle CCSVI by repairing the drainage problems from the brain, you can successfully treat, or better still prevent, the disease; theory
has been disproven https://www.statnews.com/2017/11/28/multiple-sclerosis-paolo-zamboni/