Transcript abnormalities - Blogs @ Butler

A Variety of Neurological and
Muscular Disorders
2009
Objectives



Be able to identify disease processes given
clinical (H&P, lab, radiographic, etc) findings.
Given a differential diagnosis be able to
choose a clinical finding that helps identify the
actual diagnosis.
Be able to discuss the pathophysiology,
treatment and prognosis of the diseases
presented to the extent these characteristics
are presented.
Diagnostic Catechism
Is there something wrong with the
patient?
 If so, where is the abnormality?
 If so, what is the abnormality?

Is there something wrong with
the patient?

Remember, some abnormalities are
considered normal variants
 Reflexes
(without clonus, asymmetry or
other problems) are non-pathological even
if abnormal.
 Aging Changes
 Decreased
Reflexes
 Decreased Muscle Bulk
 Tremor
 Decreased Proprioception and Vibratory
Sensation in LE
Where is the abnormality?
Cortex
 Basal Ganglia
 Brainstem
 Spinal Cord
 Root
 Plexi
 Peripheral Nerve

The “complexity” of
problems decreases
the more inferior and
distal ones gets in the
nervous system.
What is the abnormality?
Allergic
 CTD
 Congenital Defect
 Genetic
 Metabolic-Degenerative
 Inflammatory

Physical Injury
 Tumors
 Toxic
 Vascular
 Psychiatric
 Remember the best diagnosis is the
simplest diagnosis that explains all
of the clinical findings!

How to cover this material

Algorithms/Cases & Mini lectures
 Neuro
guide (demo)
Trauma?
No
Yes (1-13)
Higher functions abnormal?
No
Yes
Level of consciousness
abnormal?
Yes
No
14-20
21-29
Motor
Muscle
30-35
Mixed
Sensory
See Next
Slide
80-84
Something
in between
Nerve
36-40
41-48
Mixed
Single nerve or dermatome involved?
Yes
49-56
No
Significant non-neuro symptoms?
Yes
No
57-71
73-79.5
Trauma?
No
Yes (1-13)
Higher functions abnormal?
No
Yes
Level of consciousness
abnormal?
Yes
No
14-20
21-29
Motor
Muscle
30-35
Mixed
Sensory
See Next
Slide
80-84
Something
in between
Nerve
36-40
41-48
Section 1-13
Case #1
 Batter
gets “beaned” without helmet on
side of head
 LOC followed by at least a partial return of
function
 Second depression of consciousness
What is differential diagnosis,
i.e. what head injury types are
associated with a depressed
level of consciousness?
Ones
 See
that involve brain injury!
Trauma (#1-13)

Brain injuries as seen via computed tomography. Clockwise from upper left, these CT scans show a left
frontal epidural hematoma; a right frontal subdural hematoma with midline shift and ventricular compression;
a subarachnoid hemorrhage in the left sylvian fissure; diffuse axonal injury with diffuse edema, obliteration
of the ventricular system, and scattered hyperdense foci throughout the parenchyma (consistent with shear
injury); and a second example of subdural hematoma.

mdchoice.com/emed/images/cover/12-01Fig.jpg
General Points about Head
Injuries


Patients who have lost consciousness for
2 minutes or more following head injury
should be admitted to the hospital for
observation, as should patients with focal
neurologic deficits, lethargy, or skull
fractures.
Sometimes surgical evacuation will be
necessary. Why and in whom?

Because injury to the spine may have
accompanied head trauma, cervical
spine radiographs (especially in the
lateral projection) should always be
obtained in comatose patients and in
patients with severe neck pain or a
deficit possibly related to cord
compression.
Where else shall I look for
additional injury?
www.braininjury.com/ injured.html
 Increased
intracranial pressure may
result from ventilatory obstruction,
abnormal neck position, seizures,
dilutional hyponatremia, or cerebral
edema; an intracranial hematoma
requiring surgical evacuation may also
be responsible.
 Why care?
 Contralateral
hemiparesis with ipsilateral
fixed and dilated pupil (or vice versa)
(herniation)
 Blood flow to brain
Herniation

missinglink.ucsf.edu/.../HerniationBlum.jpg

Other measures that may be necessary to
reduce intracranial pressure include
induced hyperventilation, intravenous
mannitol infusion, and intravenous
furosemide.
 How

do you suppose these treatments work?
Corticosteroids provide no benefit in this
context.
Case #2
 Batter
gets “beaned” without helmet on
side of head.
 Grabs head screaming in pain and falls to
ground but does not lose consciousness
What is differential diagnosis,
i.e. what head injury types are
associated without a
depressed level of
consciousness?
Depressed Skull Fracture
2001/toc0106.htm
www.surgicalpa.com/

Remember there is a difference
between skull injuries and brain
injuries, though they can certainly
occur together.

Scalp lacerations and depressed
skull fractures require specific Rx.


Basilar skull fractures can be difficult to see
radiologically
Clinically suspect if:
 CSF
otorrhea or rhinorrhea via “ring sign” - CSF
separates from blood when placed on filter paper
and produces a clinically detectable sign—ring
sign, double ring sign, or halo sign.

Measure the level of beta-2-transferrin in the fluid?
 Ecchymosis
in mastoid area (Battle’s sign)
 Hemotympanum
 Raccoon eyes
Raccoon Eyes

www.surgical-tutor.org.uk/core/ trauma/head_trauma.htm
Raccoon Face
www.acm.vt.edu/~clint/download/imagedump/racc...
Case #3
A patient suffers a penetrating injury to
the right thoracic spine.
 The patient has weakness and
decreased proprioception in the right leg
and decreased pain and temperature
sensation in the left leg.


What spinal injuries exist (at least of the
ones listed on our diagnostic
spreadsheet) that can account for the
clinical findings?
http://medinfo.ufl.edu/year2/neuro/review/images/fig02.jpg
Transection of cord
Reflexes lost acutely, but “recover” after
days to weeks to hyperreflexia and
extensor plantar responses. Plus return
of bowel and bladder emptying.
 Spasticity occurs days to weeks after
injury.
 Can result in spasms especially if
patient develops bed sores or a urinary
tract infection





Treatment of the injury consists of immobilization and
- if there is cord compression - decompressive
laminectomy and fusion.
Early treatment with high doses of corticosteroids has
been shown to improve neurologic recovery if
commenced within 8 hours after injury.
Anatomic realignment of the spinal cord by traction
and other orthopedic procedures is important.
Subsequent care of the residual neurologic deficit paraplegia or quadriplegia - requires treatment of
spasticity and care of the skin, bladder, and bowels.
Trauma?
No
Yes (1-13)
Higher functions abnormal?
No
Yes
Level of consciousness
abnormal?
Yes
No
14-20
21-29
Motor
Muscle
30-35
Mixed
Sensory
See Next
Slide
80-84
Something
in between
Nerve
36-40
41-48
Section 14-20
Case #1

A 50 yo w male suffers a heart attack
and cardiac arrest. A pulse is regained
after 15 minutes of ACLS, but the
patient has not awakened. Indeed, he is
completely unresponsive (except for
brainstem reflexes) and has remained
so for several days. What do you think
is going on?
Coma
Structural lesions of bilateral
cerebrum or brainstem
 What can be learned from pupils,
breathing, posturing?
 Only perform LP if CT negative

Breathing patterns

Cheyne-Stokes ("Torsades like") - May occur with
bihemispheric or diencephalic disease or in
metabolic disorders.

Central neurogenic hyperventilation - Occurs with lesions of the
brainstem tegmentum.

Apneustic breathing (in which there are prominent endinspiratory pauses) - Suggests damage at the pontine level (eg,
due to basilar artery occlusion).

Atactic breathing (a completely irregular pattern of breathing with
deep and shallow breaths occurring randomly) - Associated with
lesions of the lower pontine tegmentum and medulla.
Pupils

Small





Pupils slightly smaller than normal but responsive to light - Present
in many metabolic encephalopathies.
Unilateral Horner's syndrome (Ptosis, miosis {still reactive},
anhydrosis)- Implies hypothalamic disease
Small but reactive pupils - Implies bilateral diencephalic (thalamus
and hypothalamus) involvement or destructive pontine
lesions.
Pinpoint (but responsive) - Implies opiates.
Large


Ipsilateral pupillary dilation with no direct or consensual response to
light - Occurs with compression of the third cranial nerve, eg, with
uncal (medial portion of temporal lobe) transtentorial
herniation.
Fixed and dilated - Implies overdosage with atropine,
scopolamine, or glutethimide or death.
Posturing

Decorticate (elbows flexed) –
 Implies

insult in internal capsule
Decerebrate (elbows extended) –
 Implies
insult in midbrain pons
Rx of Coma





Supportive therapy for respiration or blood
pressure is initiated; in hypothermia, all vital signs
may be absent and all such patients should be
rewarmed before the prognosis is assessed.
The patient can be positioned on one side with the
neck partly extended, dentures removed, and
secretions cleared by suction; if necessary, the
patency of the airways is maintained with an
oropharyngeal airway.
Dextrose 50% (25 g), naloxone (0.4-1.2 mg), and
thiamine (50 mg) are given intravenously.
Treat underlying metabolic problems if present.
Hold non-essential drugs.
Comments about some of the
other disorders in the differential.
Stupor?
No, because while level of
consciousness is depressed in stupor,
the patient will respond to repeated
vigorous stimulation.
 Bascially stupor is like a coma but less
severe; it is a “coma-lite”, if you will.

Brain Death
All brainstem reflexes (pupillary,
corneal, oculovestibular, oculocephalic,
oropharyngeal) absent for minimum of 6
hours.
 No posturing
 Spinal reflexes may be present

Locked-in State




Patient IS conscious and alert but doesn’t
look like it.
Ophthalmoplegia present except for vertical
plane
Caused by acute destructive lesion of the
pons
Can also be associated with diseases of the
peripheral motor nerves or paralysis
produced by the administration of
neuromuscular blocking agents.
Trauma?
No
Yes (1-13)
Higher functions abnormal?
No
Yes
Level of consciousness
abnormal?
Yes
No
14-20
21-29
Motor
Muscle
30-35
Mixed
Sensory
See Next
Slide
80-84
Something
in between
Nerve
36-40
41-48
Section 21-29
Case #1

Patient has cognitive impairment,
hallucinations, bradykinesia and
muscular rigidity.
Lewy Body Dementia

Diagnosis is considered probable if 2 of 3 features are
present:




Supportive evidence consists of:




Fluctuations in cognition,
Visual hallucinations, and
Parkinsonism
Repeated falls,
Syncope, and
Sensitivity to antipsychotics
Overlap of symptoms in Lewy body dementia and
Parkinson's disease may complicate diagnosis.

When early cognitive impairment and behavioral disturbances
predominate, Lewy body dementia is usually diagnosed.
CT and MRI show no characteristic
changes but are helpful initially in
ruling out other causes of dementia.
 Definitive diagnosis requires autopsy
samples of brain tissue.

Differences Between Alzheimer's Disease and Lewy Body Dementia
Feature
Alzheimer's Disease
Lewy Body Dementia
Epidemiology
Affects twice as many women
Affects twice as many men
Day-to-day fluctuation
Some
Prominent
Short-term memory
Lost early in the disease
Less affected
Deficits in alertness and attention
more than in memory acquisition
Parkinsonian symptoms
Very rare, occurring late in the
disease
Prominent, obvious early in the
disease
Autonomic dysfunction
Rare
Common
Hallucinations
Occur in about 20% of
patients, usually when
disease is moderately
advanced
Occur in about 80%, usually when
disease is early.
Most commonly, visual
Lewy Body Dementia Rx
Trial of acetylcholinestrase inhibitors
 Behavioral problems should result in
searching for precipitants such as
fecal impaction, not rewarding bad
behavior with increased attention,
reducing unnecessary meds.
 Use simple language and “distract
rather than confront”.

What are the other diseases in
this grouping that are associated
with deficits in higher functions
but little muscular weakness?
Differences Between Delirium and Dementia*
Feature
Delirium
Dementia
Onset
Sudden, with a definite beginning
point
Slow and gradal, with an uncertain
beginning point
Duration
Days to weeks, although it may be
longer
Usually permanent
Cause
Almost always another condition
(eg, infection, dehydration, use or
withdrawal of certain drugs)
Usually a chronic brain disorder (eg,
Alzheimer's disease, Lewy body
dementia, vascular dementia)
Course
Usually reversible
Slowly progressive
Attention
Greatly impaired
Unimpaired until dementia has
become severe
Level of consciousness
Variably impaired
Unimpaired until dementia has
become severe
Use of language
Slow, often incoherent, and
inappropriate
Sometimes difficulty finding the right
word
Memory
Varies
Lost, especially for recent events
Need for medical attention
Immediate
Required but less urgently
*Differences are generally true and helpful diagnostically, but exceptions are not rare. For example,
traumatic brain injury occurs suddenly but may result in severe, permanent dementia; hypothyroidism may
produce the slowly progressive picture of dementia but be completely reversible with treatment.
Wernicke’s encephalopathy
Thiamine deficiency
 Clinical findings

 Incoordination
 Nystagmus
 Lateral

rectus weakness
Replace thiamine before giving glucose
Pick’s Disease


Associated with shrinking of the frontal and
temporal anterior lobes of the brain on a
genetic(?) basis.
Depletion of seratonergic and glutamatergic
neurons is present in many patients.
 In
contrast to AD, the cholinergic system is
relatively spared in Pick’s.


Symptoms of language disturbance, including
difficulty making or understanding speech.
Spatial skills and memory remain intact.
Persistent vegetative state


Considered a partial recovery phase of coma.
The vegetative state can be diagnosed according to the following
criteria:








(1) no evidence of awareness of self or environment and an inability
to interact with others;
(2) no evidence of sustained, reproducible, purposeful, or voluntary
behavioral responses to visual, auditory, tactile, or noxious stimuli;
(3) no evidence of language comprehension or expression;
(4) intermittent wakefulness manifested by the presence of sleepwake cycles;
(5) sufficiently preserved hypothalamic and brain-stem autonomic
functions to permit survival with medical and nursing care;
(6) bowel and bladder incontinence; and
(7) variably preserved cranial-nerve reflexes (pupillary,
oculocephalic, corneal, vestibulo-ocular, and gag) and spinal
reflexes.
Patients in a vegetative state are usually not immobile. They may move
the trunk or limbs in meaningless ways.
Binswanger’s dementia




One of top three causes of dementia.
Caused by widespread, microscopic areas of damage
to the deep layers of white matter in the brain
secondary to atherosclerosis of arteries that feed
the subcortical areas of the brain.
The most characteristic feature of BD is
psychomotor slowness - an increase in the length
of time it takes, for example, for the fingers to turn the
thought of a letter into the shape of a letter on a piece
of paper.
A characteristic pattern of BD-damaged brain
tissue can be seen with modern brain imaging
techniques such as CT scans or magnetic resonance
imaging (MRI).
Alzheimer's disease



Most common cause of dementia.
Alzheimer's disease (AD) is a progressive,
neurodegenerative disease characterized in the brain
by abnormal clumps (amyloid plaques) and tangled
bundles of fibers (neurofibrillary tangles) composed of
misplaced proteins.
Three genes have been discovered that cause early
onset (familial) AD. Other genetic mutations that
cause excessive accumulation of amyloid protein are
associated with age-related (sporadic) AD.


Clinical findings:
 Early loss of memory
 Visuospatial abilities (ability to copy
geometric designs on paper) also lost early
 Mid stage
 Inappropriate sexual behavior
 Agitation
Rx like Lewy Dementia
Case #2
A 40 yo patient presents with UMN and
LMN symptoms in the extremities
 Alterations in cognitive functions such
as depression and problems with
decision-making and memory.
 EMG is abnormal and suggests nerve
loss, but NCV is OK.

ALS (Amyotrophic Lateral
Sclerosis)


Damage secondary to glutamate?
The earliest symptoms are variable and may
include:
 Twitching,
 Cramping,
or
 Stiffness of muscles;
 Muscle weakness affecting an arm or a leg;
 Slurred and nasal speech; or
 Difficulty chewing or swallowing.
ALS



Regardless of the part of the body first affected by the
disease, muscle weakness and atrophy spread to
other parts of the body as the disease progresses.
Symptoms of upper motor neuron involvement
include tight and stiff muscles (spasticity) and
exaggerated reflexes (hyperreflexia) including an
overactive gag reflex.
Symptoms of lower motor neuron degeneration
include muscle weakness and atrophy, muscle
cramps, and fleeting twitches of muscles that can be
seen under the skin (fasciculations).
ALS

To be diagnosed with ALS, patients
must have signs and symptoms of both
upper and lower motor neuron damage
that cannot be attributed to other
causes.
ALS Rx

Riluzole, 50 mg orally twice daily, which reduces the presynaptic
release of glutamate, may slow progression of amyotrophic lateral
sclerosis. There is otherwise no specific treatment.

Symptomatic and supportive measures:






Anticholinergic drugs or use of a portable suction machine if
drooling is troublesome. Behavioral modification (eg, exercising
facial muscles and encouraging frequent swallowing) or over-thecounter decongestants may also help mild drooling.
Braces or a walker to improve mobility, and physical therapy to
prevent contractures.
Spasticity may be helped by baclofen or diazepam.
A semiliquid diet or nasogastric tube feeding may be needed if
dysphagia is severe. Gastrostomy or cricopharyngomyotomy is
sometimes resorted to in extreme cases of predominant bulbar
involvement.
Tracheostomy may be necessary if respiratory muscles are
severely affected.
However, in the terminal stages of these disorders, the aim of
treatment should be to keep patients as comfortable as possible.
Differential? i.e. What are the other
diseases in this grouping that are
associated with deficits in higher
functions AND muscular weakness?

Myopathies
 Muscular
dystrophy
 Mitochondrial myopathy
Muscular dystrophy


Inherited
A genetic defect on the short arm of the X
chromosome has been identified in Duchenne
dystrophy.




The affected gene codes for the protein dystrophin, which is
markedly reduced or absent from the muscle of patients with
the disease.
Dystrophin levels are generally normal in the Becker variety,
but the protein is qualitatively altered.
One might expect that muscles would atrophy, but it looks
like muscles are larger than normal (pseudohypertrophy)
when fat replaces muscle
Associated with problems with skeleton and heart


a. Hematoxylin and eosin staining of control tissue
b. Hematoxylin and eosin staining of DMD patient, which shows abnormal variation in fiber size,
degenerating and regenerating fibers, immune cell infiltration, and increased fibrosis
c. Immunofluorescence analysis of dystrophin in control tissue biopsy
d. Immunofluorescence analysis of dystrophin in a young DMD patient biopsy, illustrating the loss of
sarcolemmal staining in DMD
www.chb-genomics.org/.../dmd_path_photos.jpg
Mitochondrial myopathy

Pathologic examination of skeletal
muscle with the modified Gomori stain
show characteristic "ragged red fibers"
containing accumulations of abnormal
mitochondria.
Trauma?
No
Yes (1-13)
Higher functions abnormal?
No
Yes
Level of consciousness
abnormal?
Yes
No
14-20
21-29
Motor
Muscle
30-35
Mixed
Sensory
See Next
Slide
80-84
Something
in between
Nerve
36-40
41-48
Section #30-35
Case #1
40 yo develops gradual onset of
proximal muscle weakness and
dysphagia. CK is elevated.
 Fever, AV block present

Polymyositis (PM)




An autoimmune etiology of the inflammatory
myopathies is indirectly supported.
PM occurs in association with a systemic
autoimmune or connective tissue disease, with a
known viral or bacterial infection or with exposure to
certain drugs, especially D-penicillamine or
zidovudine (AZT).
Biopsy provides definitive diagnosis
Rx



Glucocorticoids
Immunosuppressives
Immunomodulators
PM continued





The 5-year survival rate for treated patients with PM
and DM is ~95% and the 10-year survival 84%.
Death is usually due to pulmonary, cardiac, or other
systemic complications.
Most patients improve with therapy, and many make
a full functional recovery, which is often sustained
with maintenance therapy.
Up to 30% may be left with some residual muscle
weakness.
Relapses may occur at any time.
Fast facts about other diseases
in differential



Myotonic dystrophy and myotonia congenita
both cause stiffness, but the congenita type
is present at birth
In “myopathy associated with other disorders”
CK is usually normal, except in cases with
hypothyroidism!
Inclusion body myositis shows early
involvement of distal muscles unlike PM or
DM
Fast facts (continued)

Dermatomyositis is associated with malignancy and a
characteristic rash accompanying, or more often preceding,
muscle weakness. The rash may consist of a blue-purple
discoloration on the upper eyelids with edema (heliotrope rash),
a flat red rash on the face and upper trunk, and erythema of the
knuckles with a raised violaceous scaly eruption (Gottron rash).
The erythematous rash can also occur on other body surfaces,
including the knees, elbows, malleoli, neck and anterior chest
(often in a V sign), or back and shoulders (shawl sign), and may
worsen after sun exposure. Dilated capillary loops at the base of
the fingernails are also characteristic. The cuticles may be
irregular, thickened, and distorted, and the lateral and palmar
areas of the fingers may become rough and cracked, with
irregular, "dirty" horizontal lines, resembling mechanic's hands.
Dermatomyositis
Mechanics’ Hands
Giant mechanics’ hands

http://www.cinemastrikesback.com/news/films/scienceofsleep/1280-1.jpg
Trauma?
No
Yes (1-13)
Higher functions abnormal?
No
Yes
Level of consciousness
abnormal?
Yes
No
14-20
21-29
Motor
Muscle
30-35
Mixed
Sensory
See Next
Slide
80-84
Something
in between
Nerve
36-40
41-48
Section #36-40
Case #1


A 45 yo female presents with diplopia, dilated
and non-reactive pupils, ptosis, facial
weakness, dysphagia, and nasal speech,
followed by respiratory difficulty and finally by
weakness that appears last in the limbs. She
also has dry mouth, constipation, ileus,
postural hypotension.
She is an avid gardener and cans her own
vegetables.
Botulism
Blood work identified the botulinum toxin
 Patients should be hospitalized in case
respiratory assistance becomes
necessary.
 Treatment is with trivalent antitoxin,
once it is established that the patient is
not allergic to horse serum.

Fast facts about other disorders
in the differential
Myasthenia Gravis





Due to a variable degree of block of neuromuscular
transmission caused by autoantibodies binding to
acetylcholine receptors.
Usually occurs in younger females
Initially powerful movements fatigue readily.
Dx - Antibody assay, edrophonium
RX



Anticholinesterase drugs
Thymectomy under age 60
Corticosteroids
Lambert Eaton Syndrome
(Myasthenic Syndrome)



There is defective release of acetylcholine in
response to a nerve impulse, caused by
calcium-channel antibody
Associated with the presence of small cell
carcinoma
Unlike myasthenia gravis, however, power
steadily increases with sustained
contraction.
Periodic paralysis





Periodic paralysis may have a familial (dominant inheritance)
basis.
The syndromes to be described are channelopathies that
manifest as abnormal, often potassium-sensitive, musclemembrane excitability.
Attacks precipitated by awakening, exercise, heavy meals
Normal strength between short lived attacks
Rx



Avoid excessive exertion
Low-carbohydrate low salt diets may help prevent attacks
In the hypokalemic periodic paralysis disease give potassium
chloride to abort acute attack, BUT NOT IN HYPERKALEMIC
VARIETY!
Trauma?
No
Yes (1-13)
Higher functions abnormal?
No
Yes
Level of consciousness
abnormal?
Yes
No
14-20
21-29
Motor
Muscle
30-35
Mixed
Sensory
See Next
Slide
80-84
Something
in between
Nerve
36-40
41-48
Section #41-48

A type 1 diabetic presents with postural
hypotension, decreased cardiovascular
response to Valsalva's maneuver,
gastroparesis, alternating bouts of
diarrhea (particularly nocturnal) and
constipation, inability to empty the
bladder, and impotence.
Diabetic neuropathy pathology

Metabolic theory
 Hyperglycemia
> Increased levels of
intracellular glucose in nerves > Saturation
of the normal glycolytic pathway and
shunting of extra glucose into the polyol
pathway > Accumulation of sorbitol and
fructose lead to a whole host of neuro toxic
reactions.

Vascular (ischemic-hypoxic) theory
 Endoneurial
ischemia develops because of
increased endoneurial vascular resistance
to hyperglycemic blood.

Altered neurotrophic support theory
 Nerve
growth factor (NGF) promotes
survival of sympathetic and small-fiber
neural crest–derived elements in the
peripheral nervous system.
 In animals with diabetes, both production
and transport of NGF are impaired.

Autoimmune theory
 Autoimmune
diabetic neuropathy is
postulated to result from immunogenic
alteration of endothelial capillary cells.
Rx for DM autonomic
neuropathy




There is no consistently effective treatment for
diabetic autonomic neuropathy.
Metoclopramide has been of some help in treating
diabetic gastroparesis over the short term.
Erythromycin appears to bind to motilin receptors in
the stomach and has been found to improve gastric
emptying.
Diarrhea associated with autonomic neuropathy has
occasionally responded to broad-spectrum antibiotic
therapy, although it often undergoes spontaneous
remission. Therapy with loperamide.
Constipation usually responds to stimulant laxatives
such as senna.



Bethanechol in doses of 10-50 mg three times a day
has occasionally improved emptying of the atonic
urinary bladder.
Mineralocorticoid therapy with fludrocortisone, 0.2-0.3
mg/d, and elastic stockings or pressure suits have
reportedly been of some help in patients with
orthostatic hypotension occurring as a result of loss
of postural reflexes.
There are medical, mechanical, and surgical
treatments available for treatment of erectile
dysfunction.
Motor neuropathy can be aided
with appropriate splinting.
Let’s try a demo!
Fast facts



AIDS
Diphtheria toxin
 Accommodation and palate problems with throat infection
and skeletal muscles affected when neighboring skin is
infected
Primary lateral sclerosis
 Primary lateral sclerosis (PLS) is a rare neuromuscular
disease characterized by progressive muscle weakness in
the voluntary muscles.
 In PLS, there is no evidence of the degeneration of spinal
motor neurons or muscle wasting (amyotrophy) that occurs
in amyotrophic lateral sclerosis or ALS (Lou Gehrig's
disease), which it resembles. Only UMN deficits

Progressive Bulbar Palsy (PBP)– LMN of cranial nerves
 Pseudobulbar palsy


Progressive Spinal Muscular Atrophy


UMN version of above
Degeneration of anterior horn cells in spinal cord, so LMN.
Essentially the PBP spinal equivalent.
Pronator teres
 The median nerve gives off its motor branch, the anterior
interosseous nerve, below the elbow as it descends between
the two heads of the pronator teres muscle. Damage to this
nerve causes a motor only version of carpal tunnel
syndrome.
Trauma?
No
Yes (1-13)
Higher functions abnormal?
No
Yes
Level of consciousness
abnormal?
Yes
No
14-20
21-29
Motor
Muscle
30-35
Mixed
Sensory
See Next
Slide
80-84
Something
in between
Nerve
36-40
41-48
Section #79.5-84
Case #1


The distribution of symptoms is stronger
distally and less proximally and is termed a
glove-stocking distribution.
The symptoms include the following:
Burning sensations
 Skin tingling
 Allodynia - Painful sensation on contact with something
that typically would not hurt (eg, bed sheets)
 Hyperalgesia - Abnormally exaggerated response to
painful stimuli
 Sensation of pins and needles


This suggests painful diabetic
neuropathy!
Fast facts

Atypical Facial Pain and Trigeminal
Neuralgia (TN) both cause pain in head.
 TN
more fleeting and in distribution of TGN
unilaterally.
 ?Compression of nerve by blood vessel

Meralgia paresthetica



Pain and numbness outer aspect of thigh
The lateral femoral cutaneous nerve, a sensory nerve arising
from the L2 and L3 roots, may be compressed or stretched in
obese or diabetic patients and during pregnancy.
The nerve usually runs under the outer portion of the inguinal
ligament to reach the thigh, but the ligament sometimes
splits to enclose it. Hyperextension of the hip or increased
lumbar lordosis, such as occurs during pregnancy, leads to
nerve compression by the posterior fascicle of the ligament.

Postherpetic neuralgia
 May
persist for months or even years
 Rx
 1)
The incidence of postherpetic neuralgia may
be reduced by the treatment of shingles with
oral acyclovir or famciclovir
 2) Zoster vaccine markedly reduces morbidity
 3) If simple analgesics fail to help, a trial of a
tricyclic antidepressant
Trauma?
No
Yes (1-13)
Higher functions abnormal?
No
Yes
Level of consciousness
abnormal?
Yes
No
14-20
21-29
Motor
Muscle
30-35
Mixed
Sensory
See Next
Slide
80-84
Something
in between
Nerve
36-40
41-48
Mixed
Single nerve or dermatome involved?
Yes
49-56
No
Significant non-neuro symptoms?
Yes
No
57-71
73-79.5
Section #49-56
Case #1

A 23 yow male presents with:
 Inability
to extend the wrist or fingers of the
right hand.
 Awoke with this problem.
 Unremarkable history except for having
attended a party the evening before and
“passing out” in a chair with his right arm
draped over the chair back.

PE reveals:
 Prominent
flexion.
wrist drop and fingers held in
Radial Nerve Palsy
The history strongly suggests a
Saturday Night Palsy (Radial Nerve
Compression).
 Prognosis is good and recovery can be
expected within 6-8 weeks.

Fast facts

Bell’s palsy





Idiopathic facial paresis of lower motor neuron type that has been
attributed to an inflammatory reaction involving the facial nerve.
No treatment may be necessary, but the only medical treatment
that may influence the outcome is administration of corticosteroids,
but this is unclear.
It is helpful to protect the eye with lubricating drops (or lubricating
ointment at night) and a patch if eye closure is not possible.
Only about 10% of all patients have permanent disfigurement or
other long-term sequelae.
Patients with clinically complete palsy when first seen are less likely
to make a full recovery than those with an incomplete one. A poor
prognosis for recovery is also associated with advanced age,
hyperacusis, and severe initial pain.

Carpal Tunnel
 Modify
patient's hand activities and have the
affected wrist splinted for 2-6 weeks.
 NSAIDs can also be added.
 Patients should be referred to a specialist when
they do not improve or when thenar muscle
atrophy or weakness develops.
 Muscle strength returns gradually, but complete
recovery cannot be expected when atrophy is
pronounced.

Femoral
 May
occur in diabetics or from
compression by retroperitoneal neoplasms
or hematomas (e.g., expanding aortic
aneurysm).
 Symptoms occur in quads and
anteromedian thigh

Sciatic
 Misplaced
deep intramuscular injections are
probably still the most common cause of sciatic
nerve palsy.
 Trauma to the buttock, hip, or thigh may also be
responsible.
 Clinical findings:
Problems with dorsiflexion and eversion of foot
 Decreased sensation anterolateral aspect of calf and
dorsum of foot


Tarsal Tunnel



The tibial nerve, a branch of the sciatic, passes through the
tarsal tunnel behind and below the medial malleolus, giving
off calcaneal branches and the medial and lateral plantar
nerves that supply small muscles of the foot and the skin on
the plantar aspect of the foot and toes.
Compression of the posterior tibial nerve or its branches can
occur between the bony floor and ligamentous roof of the
tarsal tunnel.
Clinical findings:

Pain or numbness over the bottom of the foot, especially at
night, with sparing of the heel.

Ulnar
 In
the condylar groove, the ulnar nerve is exposed
to pressure or trauma.
 Clinical findings:
Problems with adduction and abduction of the fingers
(interossei)
 Problems with adduction of the thumb (adductor pollicis)
 Problems with flexion and adduction at the wrist
 Sensory changes in the medial 1 1/2 digits and along the
medial border of the hand.


Root
 Single
dermatomal level involved
General neuropathic comments


Physical therapy helps prevent contractures,
and splints can maintain a weak extremity in a
position of useful function.
Anesthetic extremities must be protected from
injury.
 To
guard against burns, patients should check the
temperature of water and hot surfaces with a
portion of skin having normal sensation.
 Shoes should be examined frequently during the
day for grit or foreign objects in order to prevent
pressure lesions.
Mixed
Single nerve or dermatome involved?
Yes
49-56
No
Significant non-neuro symptoms?
Yes
No
57-71
73-79.5
Section #73-79.5
Case #1

A 17 yow male presents with:
 Progressive
weakness of the legs and arflexia.
 Spreading to the arms and face.
 Distal paresthesias.
 Abnormal sweating.

PE reveals:
 Tachycardia
and hypertension.
 Weakness as described.
This patient has Guillain-Barre
Syndrome.
 Path

 Probably
immunologic
 Primarily demyelination
 Campylobacter jejuni enteritis

Can be confused with CIDP
 CIDP
should be suspected when a patient
presumed to have GBS deteriorates after 9
weeks
Most patients make a recovery over
months, though 10-20% are left with a
persisting disability.
 Prednisone is ineffective and
contraindicated.

Plasmaphoresis and immunoglobulin
can be helpful.
 Intubate and admit to ICU if FVC
reaches 15ml/kg, dyspnea becomes
evident or O2 saturation declines.

Case #2
Weakness, numbness, tingling, in a
limb.
 A few month ago patient was diagnosed
with retrobulbar neuritis.
 A few weeks before had urinary urgency
with a negative UA.

Multiple Sclerosis

Path





Auto-immune
Genetic susceptibility
Focal - often perivenular - areas of demyelination with reactive
gliosis found scattered in CNS and optic nerves
Infection, trauma, pregnancy may precipitate exacerbations.
Clinical findings:

MRI



Multiplicity of lesions.
Two or more different areas of white matter must be affected.
Rx



Corticosteroids hastens recovery from acute attack
Beta-interferon reduces frequency of exacerbations
Progression cannot be stopped, but recovery from acute relapses
can be hastened.
Case #3
Low back pain
 Bilateral sciatica
 Saddle sensory disturbances
 Bladder and bowel dysfunction
 Diffuse lower extremity motor and
sensory loss.

Cauda Equina Syndrome

No proven medical treatment exists, and therapy
generally is directed at the underlying cause of CES.

Some may suggest methylprednisolone in a regimen similar
to that for traumatic spinal cord injury or another regimen of
steroid for the acute syndrome.



For penetrating trauma, steroids have not shown significant
benefit.
Surgery is controversial. The timing of decompression is
controversial, with immediate, early, and late surgical
decompression showing varying results.
Consultations

Early neurosurgical, neurologic, or orthopedic consultations are
recommended, depending on the suspected etiology of CES.
Fast Facts

Cervical rib


Brachial and LS plexopathies




Involves intrinsic muscles of hand on a compressive basis
and can also affect pulse on the same side
Can occur in association with multiple problems
Brachial in the upper extremity and LS in the lower extremity
Brachial can also be associated with Horner's syndrome
HMSN II (Charcot-Marie-Tooth) and III (DejerineSottas)


II – Older pts, axons, lower extremities mostly
III – Younger pts, myelin, palpably enlarged nerves
Mixed
Single nerve or dermatome involved?
Yes
49-56
No
Significant non-neuro symptoms?
Yes
No
57-71
73-79.5
Section #57-71
Case #1
Recurrent abdominal pain.
 A rapid heart rate, high blood pressure,
sweating, and restlessness are also
common during attacks.
 Weakness, usually beginning in the
shoulders and arms.
 Recovery from symptoms occurs within
a few days.

Porphyria

Dx
 Presents
in adulthood is technically called
“acute intermittent porphyria”
 Caused by deficiency of porphobilinogen
deaminase with subsequent increased
excretion of aminolevulinic acid and
porphobilinogen in the urine.
http://www.scielo.br/img/fbpe/bjmbr/v32n3/3174i01.gif
 Pain
from abnormalities in autonomic
innervation and causes no fever or
leukocytosis
 Etiology of innervation pathology unclear
and may result from decreased heme
production or from over-stimulation of
GABA receptors as ALA is structurally
analogous to GABA
 Confirmation by increased porphobilinogen
in urine during attack

Rx
 Avoid
precipitating factors like
sulfonamides, barbiturates, starvation diets
 Suppress ALA synthetase
 High
carbohydrate diet
 IV glucose
 Hematin administration
 Analgesics
Fast facts

A whole host of mixed neuropathies can be associated with
“non-neuro” symptoms and/or telltale exposures:











Leprosy
AIDS/HIV
Lyme disease
Sarcoid
RA
DM
Uremia
Alcohol
Paraproteinemias
Malignancies
Industrial agents

There are also genetically based neuro disease in this
category

HMSN I (Also known as Charcot-Marie-Tooth)
• Foot deformities (Hammertoes, high arches, tight achilles tendon)
• Cold feet with hair loss and edema
• Palpable nerves in 25%

Friedrich’s ataxia
• Bilateral pes cavus

HMSN IV (Refsum’s disease)
•
•
•
•
Abnormal phytanic acid metabolism
Retinal degeneration
Cardiomyopathy
Cutaneous findings
Pes cavus
www.edu.rcsed.ac.uk/images/167.jpg
Pez universalis
cache.halloweenmart.com/images/ip015173.jpg