Multiple Sclerosis
Diagnosis, Treatment, Disability
J. Scott Pritchard, DO
NADE National Training
Conference - 2011
History
• Other clinical names-encephalomyelitis disseminata
-disseminated sclerosis
• First described by Jean-Martin Charcot in
1868 – Charcot’s triad
• Anecdotal histories suggest the disease
has been recognized since 1200 - Halldora
• First well documented case – Sir Frederick
D' Este’s personal diary 1822 - 1846
• Inflammatory auto-immune disease
affecting the fatty myelin sheaths of the
brain/spinal cord axons
• Demyelination and remyelination
• Scarring – Sclerosis (plaques/lesions)
• Loss of conduction – electrical signal
• Genetic, environmental and infectious
etiologies have been suggested
• Progressive and incurable
Great Mimic
• The initial symptoms are varied, vague and
extremely difficult to link to a specific cause
• Numbness, tingling, muscle weakness,
changes in cognition, nystagmus, optic
neuritis, diplopia, difficulty swallowing or
speaking, unexplained fatigue or depression
• Symptoms often increased with stress or
increased heat (Uhthoff’s phenomenon)
exercise or environmental
Physical examination
• Often very non-focal. Mild motor weakness. Nondermatomal sensory loss
• Lhermitte’s phenomenon
• Optic Neuritis
• INO-intranuclear ophthalmoplegia
• Myelopathy-seen with transverse myelitis
• Motor weakness. Fatigability of muscles with
activity. Generalized fatigue
• Spasticity – Ashworth scale – zero to four (severe)
• Ataxia
• MMSE
Clinical testing
• SSEP/VEP - evoked potentials
• +CSF for increased IgG synthesis and
oligoclonal bands
• MRI-brain/spinal cord w/gadolinium
• McDonald ‘s criteria-clinical, laboratory
and radiographic evidence
• NPS
• Myelin Basic Protein- CSF
• NMO antibodies
Classification of MS
• Relapsing-remitting-periods of clinical
worsening that resolves with time.
However, baseline function is not restored
• Secondary progressive (galloping MS)
• Primary progressive-later onset
w/minimal recovery
• Progressive relapsing-progressive decline
with superimposed attacks (Devic’s dz)
Treatment
• The goal of all therapies is slow disease
progression.
• Inflammation – Solu-Medrol
• Immunomodulation – Avonex/Rebif,
Betaseron, Copaxone (ABC therapies)
• Antineoplastics – Mitoxantrone
• Monoclonal AB - Natalizumab (Tysabri)
New Treatments
•
•
•
•
•
Extavia- Interferon -generic
Cladribine- antineoplastic
Rituximab- biologic agent-antineoplastic
Myelin Basic Protein Supplement
Oral therapies recently released
1. Ampyra – Improves gait in RR MS
2. BG – 12 - RRMS
3. Gilenya – decreases freq/severity
Disability
• 25 foot walk testnormal- 5 seconds- men
- 6 seconds- women
• EDSS-Expanded Disability Status Scale
Steps from 0-10-Inviduals 1.0-4.5 are
fully ambulatory. 5.0-9.5-increasing
ambulatory impairment. 10-Death
A claimant with an EDSS of 5.0 or >
would typically be disabled.
Listings
• 11.09 A - Reference listing to 11.04 B
• 11.09 B - Reference to listings 2.02,2.03,2.04
and 12.02
• 11.09 C –Reproducible, substantial
motor weakness w/repetitive
use or movement
SSR 96 – 8p
• DI 24510.057
• Sustainability of an RFC – consider the
functional impact of fatigue and pain
• Is the claimant able to sustain a 40 HR
work week?
Case Studies
• 57 y/o clt. 1970- numbness of the face and
double vision. 1982-RLE numbness that
spread to the LLE not relieved by
surgery.1983 OS visual loss w/Lhermitte’s
phenomenon. Subsequent years
intermittent numbness, diplopia. 1993
major exacerbation with difficulty
walking. These episodes progressed to
present. She reports mild impairment to
mobility and ADL’s. Profound fatigue.
#2
• 62 y/o clt. Onset of symptoms 1997. On
Copaxone from 1998-2002. Four courses of
Mitoxantrone. 2002-2003. Progressive
weakness and fatigue. Last major relapse
2005. MRI’s document increasing plaque
burden in the thoracic cord.
• 25 foot walk-11 seconds with a walker
• Progressive weakness in the afternoon
#3
• 54 y/o clt – onset of R optic neuritis
followed by L hemiparesis
• Deep demyelinating lesion R hemisphere
and cervical spine at C3
• Weakness occurs after walking 10-15
minutes
• Spasticity of the LUE/LLE and 3/5 motor
• Wide based gait and decreased RAM LUE
#4
• 38 y/o clt – initial bout of optic neuritis at
age 26. No other symptoms/clinical
findings until the acute onset of midthoracic and abdominal pain. MRI
documents a T12 lesion in the spinal cord
A complete work-up rules out all other
etiologies for her pain. Dx neuropathic
pain secondary transverse myelitis at T12
All modalities to relieve pain were
ineffective.
Thank YOU !!!!
Acknowledgements
• Movies from the Neurologic Exam and
PediNeurologic Exam were used with the
permission of Paul d. Larsen, MD.,
University of Nebraska Medical Center
and Suzanne S. Stensass, PhD. University
of Utah School of Medicine. Additional
materials were drawn from resources
provided by Alejandro Stern, Stern
Foundation, Buenos Aires, Argentina,
Kathleen Digre, MD., University of Utah
and Daniel Jacobson, MD., Marshfield
Clinic, Wisconsin. The movies are licensed
under a Creative Commons AttributionNon-commercial ShareAlike 2.5 license