Transcript Neurology and nutrition

Neurology and nutrition
Dr Anhar Hassan, Dr Brendon Boot
Senior Neurology Registrar
Royal North Shore Hospital
Objectives






be able to describe the neurological disorders which give
rise to dysphagia
be able to describe the assessment of dysphagia
be able to describe nutritionally relevant stroke risk factors
provide or use in the presentation medical terminology and
abbreviations for these disorders
biomedical parameters and diagnostic tests and how these
change with disease progression or improvement
medications and other treatments associated with the
management of the neurological disorders discussed
Neurological disorders
resulting in dysphagia

Neurology deals with many conditions, affecting
the peripheral or central nervous system:
 Acute, may improve
– Stroke

Acute on chronic
– Multiple sclerosis

Progressive
– Motor neurone disease
– Parkinsons disease
– Huntington’s disease
Case 1: Mrs J.V.

72 yo lady
 2 years progressive weakness initially of upper
then lower limbs, later bulbar and respiratory
muscles.
 Diagnosed with motor neurone disease- no
treatment to significantly alter disease progression
 Progressive difficulty walking- now transfers only
to and from wheelchair with assistance of her
husband. Needs assistance of husband for all
activities of daily living
Case 1: Mrs JV

Noticed soft speech
 Difficulty swallowing, ineffective cough triggered
by food and long periods of time taken for small
meals. Drooling a major inconvenience and some
“choking” episodes (unpredictable)
 Marked weight loss- 45 kg at presentation, 5-6 kg
loss in the 3 weeks prior to presentation after no
oral intake
 Presented with breathlessness and clinically
dehydrated, malnourished and in respiratory
failure
Mrs JV management





Speech therapist assessment - safe for thickened fluid and
puree diet, dietitian calculated that her intake would not equal
her nutritional requirements
Hydrated, receiving all nutritional requirements through
percutaneous endoscopic gastrostomy (PEG) feeding tube
Discharged with noninvasive ventilator with the aim to use it
at night when most desaturations occur and anytime when
breathless
Discussed end of life decisions again- particularly if unwell
Linked in to community palliative care service and motor
neurone clinic follow up service
Motor neurone disease





Degeneration of both upper and lower motor neurones
Weakness often asymmetric in legs, hands, proximal arms or
oropharynx. No sensory impairment. Usually mentally alert.
Respiration is usually affected late.
Weight loss occurs from a combination of wasting and dysphagia.
Cranial nerve nuclei affected: dysarthria, lingual wasting and
fasciculation and impaired movement of the tongue.
Dysarthria and dysphagia is caused by upper motor neurone
disease (pseudobulbar palsy). The uvula does not move well (or at
all) on phonation but a vigorous response is seen in the pharyngeal
or gag reflex.
MND management
1.
2.
3.
4.
Nutrition
Ventilation and protect against aspiration
Nursing care
Medications: No cure
– Riluzole (glutamate inhibitor)- prolongs life by
3 to 6 months with no improvement in function
or quality of life
– Botulinum toxin injections to treat siallorrhea
MND multi-disciplinary clinic

Assessed as a day stay patient by all members of
the team and then follow up arranged in the
outpatient clinic via a nursing coordinator
 Team members:
–
–
–
–
–
–
–
–
Neurologist
Respiratory physician
Respiratory clinical nursing consultant
Physiotherapist
Occupational therapist
Speech therapist
Dietitian
Social worker
MND prognosis

Mean duration of symptoms is 4 years.
– Bulbar onset MND is slower to progress

Death is usually from respiratory failure,
aspiration pneumonia or pulmonary
embolism after prolonged immobility.
Case 2: Mrs SC

82 year old lady
 Lives alone following the admission of her
husband to a nursing home after his stroke.
Supported by a friend.
 A slowly progressive dementia has been
noticed by her friend (aware of people but
often forgets recent events)
Mrs SC presentation
Sudden onset:
 Hemiparesis (unable to move right side)
 Global dysphasia- follows 1 step commands
unreliably and has fluent but nonsensical speech
(conversational “niceties” are reasonably
preserved)
 Noted to be in rapid atrial fibrillation with a small
acute myocardial infarction noted on tests.
CT brain: dense middle cerebral artery
CT brain: loss of grey white differentiation
MRI: DWI- L middle cerebral artery territory stroke
MRI: T1- L MCA stroke
MRI: T2 coronal
Mrs SC: progress

Aspiration pneumonia noted on day 3 of admission.
When first reviewed on arrival (Saturday morning)
in emergency she had been “trialled” on a puree diet
in the absence of speech therapists to formally assess
her till Monday. Food was pooling in her mouth
which she seemed unaware of.
 Cardiac failure ensued with recurrent runs of rapid
atrial fibrillation. Medical management was limited
by hypotension.
Mrs SC: management issues
Immediately life threatening
– Rapid atrial fibrillation due to hemodynamic
compromise and risk of further heart attack.
– Heart failure
– Aspiration pneumonia
Nutrition
– Able to have grade 2 thickened fluids.
– With the major fluctuations in her other comorbidities,
she was not receiving adequate nutrition so additional
nasogastric tube feedings were considered.
Mrs SC: management issues
Preventing further strokes (secondary prevention)
– Likely embolic: Anticoagulation (presumed clot from the
fibrillating heart)
– Exclude other causes (carotid artery disease)
– Manage stroke risk factors
Functional status after this stroke
– Unable to “learn” due to dementia and aphasia.
Rehabilitation is not possible. Due to ongoing severe
weakness and dependence, she requires nursing home care.
– Friend applied for guardianship to organise her affairs
Stroke
Two types:
1. Infarction (interrupted blood supply) from
emboli, unstable atherosclerotic plaques or
small vessel disease
2. Haemorrhage secondary to artery
weakness (amyloid in elderly, aneurysms
etc) and hypertension
Stroke

Presents with sudden neurological deficit. The
distribution of weakness, sensory loss, visual or
speech impairment, or neglect depend on the
vascular territory involved. Unilateral weakness is
characteristic of many syndromes.
– eg, Middle cerebral artery territory which affects
face>arm>leg weakness to varying degrees and speech
if the dominant (usually left) hemisphere is involved
Stroke prognosis

Death ranges from 8-20% in first 30 days
(higher for haemorrhages)
 Death is mainly from cardiac (AMI,
arrhythmias) or respiratory (aspiration
pneumonia, pulmonary embolism) sources.
Less deaths are due to brain swelling with
herniation.
Dysphagia in stroke

27-50% of stroke patients have dysphagia
 50% of these die or recover spontaneously
 Dysphagia causes increase risk of chest
infections, malnutrition, increased length of
hospital stay and readmission to hospital.
Medical screening for dysphagia: sip test

If constantly alert, position patient in
upright position
 Give 50 ML water in 5 ml steps
 Listen to patient’s voice and watch for
cough between sips
– If coping with above, may have thin fluids and
appropriate diet
– If drowsy or if signs of aspiration, patient
“fails” and has formal speech path review of
swallowing
Dysphagia in stroke

Nasogastric tube feeding - easy, quick, relatively
noninvasive and safe.
 Patients can pull them out; nasal ulceration limits
duration.
 PEG - invasive procedure (complications
peritonitis, bleeding, perforation of abdominal
organs). In the long term, they are more
comfortable.
FOOD trials

Underweight patients had increased mortality (OR
for undernourished compared with normal, 1.82;
95% CI, 1.34 to 2.47)
 Adding oral protein-energy supplements to
standard hospital diet was not powered to show a
significant benefit
 Early (<1 week) versus late (>1 week) enteral
feeding resulted in an absolute reduction in
mortality of 5.8% (95% CI 0.8-12.5, p=0.09)
 PEG feeding was associated with increased
mortality and poor outcome (p=0.05)
Nutritionally relevant stroke risk factors

Hypertension
– Low salt
– Usually requires antihypertensive medication

Cholesterol
– Measure total chol, HDL (good), LDL (bad),
triglycerides
– Dietitian consult
– In vascular patients, unless contraindicated, we start a
statin to lower cholesterol
Nutritionally relevant stroke risk factors

Diabetes:
– Diagnosis
 Fasting BSL <5.5 mmol/L- no further action required
 Fasting BSL 5.5-6.9- OGTT required
 Fasting BSL >7 mmol/L- repeat fasting BSL to confirm
– Management
 Refer to dietitian for diabetic diet, diabetic education unit
and endocrine team (may need oral hypoglycemic)
Case 3: Ms LP

38 yo lady
 Presented 9 years ago with R visual disturbance
– Delayed R visual evoked response
– Dx: optic neuritis

9 months later, had clumsiness of her left limbs
– Left cerebellar incoordination noted
– Given pulse methylprednisolone (1g 3 days)

Diagnosed with multiple sclerosis after MRI,
lumbar puncture and visual evoked responses.
Ms LP: current issues

Subjective breathlessness and neuropathic
pain limiting oral intake- presented
dehydrated with marked weight loss
 No objective change in weakness,
clumsiness or sensory impairment of her
cranial nerves or limbs.
Ms LP:

Strained voice (combined pseudobulbar and
cerebellar dysarthria)
 Marcus Gunn phenomenon
 moderate asymmetric R>L limb clumsiness
with minimal pyramidal weakness,
generalised brisk reflexes, independently
mobile.
Ms LP: progress

Given 3 days IV methylprednisolone




Commenced on beta interferon


some improvement in the pain, but persistent subjective
SOB.
Already on gabapentin
Respiratory physicians investigating further
Aim to decrease frequency of relapses
Speech pathology, dietitian, physiotherapy, OT
and social worker reviews.
 Referred to rehabilitation
Ms LP: progress

Infrequent relapses requiring infrequent
steroids, no preventive treatment
– Relapses too few to qualify for beta interferon
initially

Slow functional decline after each relapse
however
 Still living independently but too disabled
to work. (previous nanny)
Sagittal MRI of spine showing increased signal
Axial MRI of spine showing increased signal
Axial MRI brain
showing acute
demyelination
The same areas of
demyelination
months later
Multiple sclerosis (MS)

A chronic disease most commonly in young
adults (peak incidence between 20-30 years
old, females) with areas of inflammation
and demyelination in the central nervous
system.
Multiple sclerosis subtypes
Two clinical pictures:
1. Relapsing remitting with secondary progressive MSUsually starts as a relapsing remitting course of isolated
exacerbations then turns into a chronic progressive,
unremitting course. (80% of cases over 10-15 years)
2. Primary progressive MSSymptoms
include weakness, sensory disturbance, blurred vision due
to optic neuritis, incoordination due to cerebellar
involvement, bladder instability from spinal cord disease,
and cognitive or personality change.
MS treatment

Acute relapses are shortened by intravenous
methylprednisolone
 Recurrent relapses may qualify the patient for betaferon
therapy which has been shown to decrease relapses
 Side effects of medications commonly used include:
– Baclofen for spasticity- nausea, sedative
– Oxybutinin for neurogenic bladder instability- dry mouth,
nausea, constipation, abdominal discomfort
– Recurrent antibiotics for infections- nausea and diarrhea
– Corticosteroids for relapses- osteoporosis
– Interferons for prevention of relapses- “flu like symptoms”
including nausea, fatigue and depression
Dysphagia and nutrition in MS





Incidence of dysphagia reported from 3-43% in trials.
Diet often limited by fatigue, reduced mobility, and other
disabilities affecting meal time efficiency (incoordination
or visual impairment)
No relationship found between dysphagia and nutritional
status in a group of 79 patients with a high incidence of
dysphagia (43%).
No direct evidence but it is suggested to use dietary
supplements (eg sustagen) as an effective means of
improving the nutrient intake without impairing appetite
for normal foods.
PEG feeding is an option also
Huntington disease: an example of a
neurodegenerative condition



Progressive hereditary disorder usually presenting in middle
age.
Course of disease usually runs over a period of 15 years. More
rapid in patients with earlier onset of age.
Characterised by:
•
•
•



Movement disorder (usually chorea)
Dementia
Personality disorder
Diagnosed by genetic testing
No known treatment to alter the course of the disease.
Treatment of depression and psychosis important
Parkinson disease (PD)another neurodegenerative condition
One of many causes of “parkinsonism”
(including drugs, other neurodegenerative
processes, trauma, tumour, postencephalitic)
 Degeneration of dopaminergic neurones in the
basal ganglia
 Mean age of onset 55 years
 Insidious onset, slow progression

PD: clinical features







Tremor at rest- “pill rolling”
Rigidity
Bradykinesia- slowed movements, small
handwriting, soft speech (hypophonia), loss of
facial expressions (hypomimia) and dysarthria
Flexed posture and shuffling gait
Poor balance and falls- loss of postural reflexes
“Freezing” phenomenon
Fluctuations increase with disease progression and
complexity of medical management- “on” vs
“off”, dyskinesias usually peak dose
PD: management

No treatment has been proven to alter the underlying
neurodegenerative process but the mortality rate has
dropped 50% since treatment with levodopa
Medications:





Dopamine precursor (levodopa)- the most commonly used
Dopamine agonists (bromocriptine)
Dopamine releaser/glutamate antagonist (amantadine)
Monoamine oxidase type B inhibitor (selegeline)
Anticholinergics (benztropine)
Surgery:


Ablation
Deep brain stimulation
Neurology patients can be
challenging…

Level of consciousness- variability
 Personality or cognitive impairmentscompliance, information processing
 Treatments may interfere with nutritiondrug side effects, ventilation
 Disability limiting access to best options
 Depression and fatigue are commonundermine motivation
Important references

Bath PMW et al. Interventions for dysphagia in acute
stroke (Cochrane review). The Cochrane library, Issue
1, 2002
 FOOD Trial Collaboration. Poor nutritional status on
admission predicts poor outcomes after stroke:
observational data from the FOOD trial. Stroke.
34(6):1450-6, 2003 Jun
 Donnan GA, Dewey HM. Stroke and nutrition: FOOD
for thought. Lancet. Feb 2005; 365: 729-730.
 Payne A. Nutrition and diet in the clinical management
of multiple sclerosis. The British Dietetic Association
2001. J Hum Nutr Dietet. 14; 349-357.