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General Neurology -MND, MS, Muscle Disease, Systemic Disease of NS
Define MND.
Progressive condition characterized by degeneration
of UMN and LMN. Unknown cause, possibly genetic,
viruses, toxins or minerals.
Which cells are affected by MND?
Ventral horn cells become thinned, especially in the
Csp and L/S region and the upper motor neuron.
Would an upper or a lower motor neuron
lesion picture be expected?
What part of the body is affected first and
how does it progress?
Some show strong evidence of LMNL, some purely
UMNL picture with spastic paraparesis mimicking
cord compression.
MND, synonymous with Lou Gehrig disease. Classic
variant, combination of UMNL and LMNLprogressive mms atrophy; damage ventral horn cells
and no evidence of damage to corticospinal or
corticobulbar disease. Reflexes aren’t enhanced.
Plantar reflex flexor and weakness is in a myotomal
pattern not a pyramidal distribution.
Hands and feet affected.-asymmetric wasting and
weakness, then mild spastic paraparesis in legs (75%).
Bulbar or pseudobulbar features (25%)-dysphagia
(difficulty in swallowing) or dysarthria (impaired
articulatory ability).Key feature is a mixture of
upper and lower motor neuron involvement with
normal sensation.
Limb onset ALS-results from involvement of the
corticospinal tracts and ant horn cells.
Bulbar onset=progressive bulbar palsey-presents
with a combination of corticobulbar degeneration and
lower cranial nerve motor nuclei involvement.
What other condition must it be differentiated
from?
Cervical spondylosis-this will present with loss of
reflexes- if wasting and weakness is due to ventral
What is amyotrophic lateral sclerosis (ALS)?
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What functions are never affected by MND?
What is the prognosis for MND?
What is Multiple Sclerosis?
What is neuropathic type of MS?
What must neuropathic type of MS be
differentiated from?
Describe subacute cord compression type of
MS
What is Muscle disease?
horn cell disease reflex not disrupted.
Sensation never affected.
Bladder never affected
Ocular mms never affected
ALS is typically relentless in progression, with 50%
of patients surviving for less than 3 years after
diagnosis, whereas approximately 20% of patients
survive for 5-10 years.
In contrast to ALS, clinically "pure" PLS, defined by
isolated upper motor neuron (UMN) signs 4 years
after symptom onset, is a syndrome of slow
progression with high levels of independence for
years or decades
Major cause of spinal cord disease. Immune-mediated
inflammatory disease that attacks myelinated axons in
CNS. Relapsing and remitting course-normally
characterized by focal disturbance of function.
Diagnosed after at least 1 episode. Classically made
worse by heat. Commonly affects optic nerve giving
shadow in the visual field.
Sensory symptoms predominate and consist of
tingling peripheral paraesthesia around the arms and
legs, reflexes will be brisk and plantar response may
be extensor (so this can be used to distinguish bt MS
and peripheral neuropathy as reflexes will be
decreased or absent in peripheral neuropathy).
Hyperventilation syndrome-these pt will notice
tingling also around the mouth and symptoms will
come and go. Reflexes may be brisk due to anxiety
but pathological reflexes will not be present.
Very similar to progressive cervical myelopathy
(myelopathy means pathology of the spinal cord)
Progressive disorders of muscle, characterised by
cycles of mms fibre necrosis, regeneration, eventual
fibrosis followed by replacement with fatty tissue.
Congenital myopathies are associated wit
morphological mms abnormalities without nerosis
and with a more benign prognosis. The metabolic
myopathies present withwith pain, weakness or
fatigue.
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What should be looked for in a child?
Child will ‘climb up himself’ when standing up.
What should be asked in case Hxx taking if
MD is suspected?
-Family Hxx
-Patten of weakness? Proximal weakness will cause
difficulty descending stairs. Distal weakness will
present as difficulty with latch keys, difficulty
ascending stairs.
-Pain and cramp and their relationship to Exx? In
disorders of glycolysis a cramp develops in exercising
mms after a min or so…whereas in Carnitine palmityl
transfer transferase deficiency cramp develops some
hrs later.
-Fatiguability
Assess
Walking-look for waddling or foot-drop.
Distribution of weakness and wasting-will
distinguish proximal, distal or generalised
myopathies.
What should the examination include if MD
is suspected?
Systemic disease and the nervous system
Local and distant effects of neoplasm,
connective tissue disorders, endocrine
disorders, organ and system failures –
mechanisms, symptoms and signs.
Deficiency states such as SCDC (subacute
combined degeneration of cord
Describe the clinical features and what would
be found on examination of a pt with of
SCDC.
Deficiency of vit B12 produces SCDC. Can occur due
to:
Inadequate diet-strict vegans
Increased need-pregnancy
Defective absorption
Pathology-SC demyelination and eventual with
eventual axon loss-affects posterior columns and lat
columns.
Clinical features-Paraesthesia of extremities,
followed by numbness and distal weakness. Walking
becomes unsteady and spasticity is evident in the
lower limbs.
Examination findings-gait is ataxic with positive
Rhomberg sign. Mms power diminished. Plantar
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response extensor. Loss of JPS and Vib in lower
limbs. Stocking and glove sensory loss when
peripheral nerves are involved.
Infections including tuberculosis, syphilis,
herpes, parasites
Define MSDescribe what MS does to a
person, reconciling structural changes with
pt experience
• Discuss possible causation, prognosis and
possible treatm
Describe the 1st nerve signs in MS-how
would they be tested?
Can myelin repair?
How and why can the gait change in MS?
What other tracts are commonly affected?
What UMN signs occur?
Is the urinary system affected?
What treatment options are there for MS?
What is primary progressive MS?
What is secondary progressive MS?
What are the 3 basic forms of motor neurone
disease?
How would you differentiate UMNL from
LMNL in CNXII
If pt reports visual changes; shadow in visual field…
Check blind spot as it may have increased in size.
Colour vision-may change (as fibres coming from
cones are damaged).
Look at optic disc-may look perfectly white as
myelin is yellow, one eye may look lighter.
Limited capacity to repair, may not heal perfectly,
leaving scar.
Damage to cerebellar peduncles. Pt will present
with difficulty walking; verging on ‘wide, drunken,
realing’.
Ask pt to demonstrate walking, ask ‘is it worse any
time of day?’ as this shouldn’t make a difference to
MS suffers and distinguishes it from other problems.
Dorsal columns/ Medial leminsus- this gait may be
superimposed on cerebellar gait (high stepping with
hard footfalls). Great reliance on light. To test dorsal
columns do either joint position test assessment or
Rhomberg Test.
Spinothalamic tract so temperature appreciation
diminished.
Legs feel stiff, tripping.
Spastic bladder; Christmas tree bladder.
Hyperbaric O2 chamber
Steroids, glucocorticoids. Immunosuppressants; but
these allow oportunustic infections. Perin, Raymondosteopathic techniques for people w MS.
LMNL
UMNL
Mix of the 2 above
LMN: fasciculations & wasting (can test tone of
mms)
UMN: hypertonic
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Explain when a Pt has mixed UMNL in the
foot & LMNL in the arm
Connective Tissue Disorders
Name the connective tissues in
the body?
What are connective tissue
disorders?
Aetiology
Name CT disorders that effect
the NS
Marfan’s Syndrome
Arm: cells are dying of in the ventral horn of the
cervical enlargement
Leg: S1 cells are dying of in the motor cortext
Check this???
I believe he’s saying in this eg there is damage
throughout the PNS
The cervical enlargement is prone to being damaged
due to having so many cell bodies
○Blood and vessels
○Bone
○Fascia
○Tendons
○Ligaments
○Endoneureum, Perineureum and Epineureum
○Glial cells in the CNS
○Swann cells in the PNS
○Endomysium, Perimysium and Epimysium
○Fat, lipid and Adipose sites
Disorders that are largely immunologically driven and auto
immune driven which lead the body to mount an attack on upon
those tissues
Partly genetic and in some cases probably be triggered off by
environmental toxins and that sort of thing or infections quite
possibly .
Reiter’s Syndrome
Marfan’s Syndrome
Ehlers Danlos
Ankylosing Spondylitis
Rheumatoid Arthritis RA
Osteoarthritis
Polymyalgia Rheumatica
Begnin Hyper Mobility Syndrome
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A person with Marfan’s tends to be long and thin in the body but
also their arm span to height ratio is larger than most people’s.
Part of the reason for that is they seem to have very long fingers it
is called arachnodactyly – like spiders legs. They tend to be prone
to heart valve disorders like lots of people with connective tissue
disorders.
The lens of the eye tends to dislocate as well - The lens of the eye
being connective tissue. Marfans is incredibly rare.
What is Ehlers Danlos?
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Another rare disorder of connective tissue
Hyper elastic connective tissue
If they were to pull their cheeks out they would look like
‘Mr Fantastic’
Or the skin on the back of the hand might lift up.
So they are very elastic and that might have reprocussions
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for other supportive connective tissue
They get dislocations of the lens and heart valves
And it could also effect other parts of the musculoskeletal
system
Not usually included in the connective tissue disorders but it is of
connective tissue.
If you get advanced O.A the connective tissues are not really
healthy as such, because they change in structure.
But it certainly is an inflammatory condition and an auto immune
condition. It doesn’t seem you can modify O.A by giving things
like immune suppressants - but you can modify R.A that way.
So this will be one of the things which will mark out what’s often
called a CT disorder.
You get pannus formation of the synovium. The synovium
becomes a creeping destroyer of cartilage, in effect.
So you get a massive synovial tissue which has been changed into a
cartilage eating monster and it starts destroying the tissue.
We will talk about differentiating between those a bit more next
time.
Nerves may become pinched
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Does O.A affect NS?
Does RA affect NS?
AS
MS
How about MS?
When does it tend to be
diagnosed?
What is MS?
What is Ataxia?
Typical Symptoms
Symptoms cntd…
What structures does it effect &
in which order?
It is normally more benign
20-30s
A condition which is spread throughout the CNS (it used to be
called dessiminate sclerosis). Replacement of myelin with fiborous
material. Thought to be auto-immune
GET A PROPER DEFINITION
Gross lack of coordination of mm movements
Early onset
Replapsing Remitting
1. Optic nerves (because they are an extension of the CNS, they
are bathed in cerebro-spinal fluid, they are heavily mylenated)
2. Cerebellum & its connections commonly become ataxic
3. Sensory Tracts note it tends to be a good prognosis if
symptoms start in the Sensory tracts. And poor if Pt is male
and it starts in the motor tracts
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Type
Amyotrophic
lateral sclerosis
(ALS)
Primary lateral
sclerosis (PLS)
Progressive
muscular atrophy
(PMA)
Progressive bulbar
palsy
Pseudobulbar
palsy
UMN
degeneration
LMN
degeneration
yes
yes
yes
no
no
yes
no
yes - bulbar region
yes - bulbar region
no