In The Name of God CASE PRESNTATION OPTIC NEUROPATHY

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Transcript In The Name of God CASE PRESNTATION OPTIC NEUROPATHY

In The Name
of
God
CASE PRESNTATION
OPTIC NEUROPATHY
WITH
PROGRESSIVE MYELOPATHY
Loghman Hospital
Y. KHOLGHI MD
HISTORY
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A 51 years old married right handed female that was in
good health until 11 years PTA.
 She has history of lower limbs heaviness in
combination with feeling of coldness in 11 years PTA,
that persist 1-2 month and then subside without any
treatment.
 After 1 year she had complain of visual loss in right
eye and then 3-4 month later in left eye with visual
acuity of complete visual loss in right and 6/10 in left
eye.
 Each of visual attacks treated with Methylprednisolone
pulse and oral Prednisolone after it, that lead to
complete recovery.
 Concurrent with visual attacks she had sensory
complain as coldness and tingling with feeling of
heaviness in lower limbs that persists in subsequent 4
years in the spite of normal living activities.
 She had not any urinary complains in 5 years duration.
HISTORY
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Since 6 years PTA, she has history of progressive
stiffness and weakness in lower limbs with involvement
of hands as tingling and heaviness after 2-3 years, that
lead to inability to walking without assistance and loss
of dexterity in hands in recent 1-2 months respectively.
 She has history of fatigue, frequency, urge
incontinence and prolonged constipations since 6 years
PTA.
 Review of Lhermitte’s sign, diplopia, vertigo, unsteady
gait, abnormal speech, difficulty swallowing, and other
systems was unremarkable (no joints pain and skin
lesions).
 She was under treatment of interferon in 2 subsequent
years that discontinue it since 2 years PTA.
 She is under treatment of Amantadine, Baclofen,
Asentra, Alprazolam for reliving stiffness, fatigue and
anxiety. She previously treated with MTX.
Positive finding in N/E
 Norma
reaction of both pupils to light.
 Normal visual acuity.
 Mild pallor of optic disk in both eyes.
 Spastic quadriparesia preferentially (with
all signs), loss of abdominal reflex, and
muscle force of 4/5+ in lower limbs with
flexion deformity of ankles in rest position.
 A sensory level at T7-T8 with abnormal
deep sensation in lower limbs.
 Ability to walking without assistance (up
to10 m).
Abstract of History and N/E
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A 51 years old married right handed female with
history of visual blurring, progressive weakness
and stiffness of lower limbs, fatigue, frequency,
incontinence and constipation since 11 years
PTA.
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She has bilateral optic disk changes, spastic
quadriparesia, a sensory level at T8-T9 and
abnormal deep sensation of lower limbs in N/E.
Localization
 With
a normal cranial exam, causing
lesion of spastic quadriparesia is in
cervical region.
 But with visual loss in different time, she
has a additional localization sign for
correct diagnosis as multiple lesions in
multiple sites with dissemination in
times.
Differential Diagnosis
 Toxic
and metabolic diseases.
 Degenerative diseases.
 Tumor and mass lesions.
 Infectious diseases.
 Vascular diseases.
 Demyelinating diseases.
Toxic and metabolic diseases
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Drugs (Cisplatin, Heroin abuse and rarely
Cocaine use).
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Toxin (Conzo and lathyrism).
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Metabolic ( Vitamin B12 deficiency, Mercury
intoxication, Alcohol/tobacco amblyopia
Central pontine myelinolysis, MarchiafavaBignami syndrome).
Degenerative Diseases
 PLS.
 HSP.
 SCD.
 Hereditary
disorders of myelin
metabolism (ALD, MLD, Krabbe's).
 Arnold- Chiari malformation.
Tumor and Mass lesions
 Spinal cord mass
 Intramedullary
 Extramedullary
 Craniocervical
lesion:
junction tumor.
 Paraneoplastic
encephalomyelopathies.
Infectious Diseases
 HTLV
I & II
 LYME
 SYPHILIS
 HIV
 TB
Vascular Diseases
 Arteriovenous
Malformations.
 Cavernous Angiomas.
 Isolated CNS Vasculitis.
 Collagen Vascular Diseases.
 Stroke.
Inflammatory Demyelinating Diseases
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Acute Disseminated Encephalomyelitis.
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VARIANTS OF MULTIPLE SCLEROSIS.
1.
3.
Recurrent Optic Neuropathy
Devic's Disease (Neuromyelitis Optica)
Slowly Progressive Myelopathy
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Multiple Sclerosis (PPMS OR SPMS).
2.
Recurrent Optic Neuropathy
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There are patients whose entire clinical illness is
confined to the optic nerves.
 They may have sequential affection of one nerve,
then the other, or they may have simultaneous
bilateral vision loss, a state that is quite uncommon
in classic MS.
 Children and preadolescent patients are more likely
than adults to have recurrent or simultaneous optic
neuropathy.
 Rarely there is slowly progressive optic neuropathy,
similar to that seen with optic nerve sheath tumors,
such as meningioma.
 In bilateral ON, sarcoidosis is commonly a diagnostic
consideration.
Devic's Disease (Neuromyelitis Optica)
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A combination of bilateral optic neuropathy and
cervical myelopathy make up this condition.
 Reported cases indicate that the myelopathy
tends to be more severe, with less likelihood of
recovery.
 In some patients the optic neuropathy and the
myelopathy occur at the same time, in others
one or the other component is delayed (days to
weeks).
 The longer the interval, the more like typical MS
is the pathology and better prognosis.
Devic's Disease (Neuromyelitis Optica)
 Because
the optic nerve and the
cervical spinal cord are two of the
locations in the nervous system in
which the lesions of MS are typically
found, many patients could be
classified as having Devic's disease, or
syndrome.
Devic's Disease (Neuromyelitis Optica)
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Devic's like syndrome can be a manifestation
of ADEM or rarely of other autoimmune
disease, such as SLE or APAS.
 This seems to be especially true of patients
with relapsing Devic's syndrome, making up
approximately one half the patients.
 In a few patients the distinction between an
MS variant and SLE (so-called lupoid
sclerosis) is essentially impossible to make,
and some of these are patients with
neuromyelitis optica.
How differentiate Devic’s from
MS
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Failure to develop Brainstem, Cerebellar or
Cerebral demyelinative manifestations.
Normality of the cerebral white matter on MRI.
Almost uniform absence of OCB and
abnormality of IgG in CSF.
Necrotizing and cavitary nature of spinal cord
lesions, affecting gray and white matter.
Humoral nature of Devic’s with high titer of
antibody in blood in new studies.
Slowly Progressive Myelopathy
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If there are no sensory signs or symptoms, the
entity known as primary lateral sclerosis, one of
the group of motor neuron diseases, may be the
cause.
 HTLV-l infection, vitamin B12 deficiency, and
human immunodeficiency virus infection all can
be excluded by appropriate testing.
 Spinal dural arteriovenous fistula can cause a
steadily or stepwise progressive myelopathy,
usually in the lower spinal segments.
 Adrenomyeloneuropathy should be considered.
Slowly Progressive Myelopathy
A
number of patients their spinal MRI
results are repeatedly negative.
 VERs,
CSF OCBs, and MRI of the head
show no sign of demyelination elsewhere.
Slowly Progressive Myelopathy

Some clues that MS is
present are Lhermitte's
sign, sensitivity to
elevated temperature and
optic neuritis.
Primary progressive MS
 Progressive
myelopathy caused by MS
is part of the primary progressive MS
group and carries the poor prognosis
typical of that group.
Final Diagnosis
Progressive Myelopathy
caused by MS
(secondary progressive MS)
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The degree of compression
of the cervical cord by intervertebral disc disease is
often an issue in the middle-aged patient, because a
majority of persons have some degree of disc disease, and
following trauma there may be a T2 bright signal within the
cord due to contusion. There is little doubt that some
laminectomies have been carried out for cervical spondylosis
where MS was the final correct diagnosis.
. The choice of therapy is
difficult. Some patients do better for a time with monthly
intravenous corticosteroid therapy.
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Inflammatory diseases
Granulomatous angiitis
Systemic lupus erythematosus
Sjogren's disease
Beh.;:et's disease
Polyarteritis nodosa
Paraneoplastic encephalomyelopathies
Acute disseminated encephalomyelitis, postinfectious
encephalomyelitis
Infectious diseases
Lyme neuroborreliosis
Human T-celllymphotropic virus type I infection':'
Human immunodeficiency virus infection
Progressive multifocalleukoencephalopathy':'
Neurosyphilis"
Granulomatous diseases
Sarcoidosis
Wegener's granulomatosis
Lymphomatoid granulomatosis
Diseases of myelin
Metachromatic leukodystrophy (juvenile and adult)':'
Adrenomye Ioleukodystroph y.:.
Miscellaneous
Spinocerebellar disorders':'
Arnold-Chiari malformation
Vitamin Bl2 deficiency"
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Autoimmune
Acute disseminated encephalomyelitis
Acute hemorrhagic leukoencephalopathy
Multiple sclerosis
Infectious
Progressive multifocalleukoencephalopathy
Toxic/metabolic
Carbon monoxide
Vitamin B12 deficiency
Mercury intoxication (Minamata disease)
Alcohol/tobacco amblyopia
Central pontine myelinolysis
Marchiafava-Bignami syndrome
Hypoxia
Radiation
Vascular
Binswanger's disease
Hereditary disorders of myelin metabolism
Adrenoleukodystrophy
Metachromatic leukodystrophy
Krabbe's disease
Alexander's disease
Canavan-van Bogaert-Bertrand disease
Pelizaeus-Merzbacher disease
Phenylketonuria
Multiple sclerosis
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(e.g., vitamin B12 deficiency, compressive
spinal cord lesions, arteriovenous
malformations,
cavernous angiomas, Arnold-Chiari
malformation), infectious
causes (syphilis, HTL V-I, human
immunodeficiency
virus), or hereditary disorders (adult
metachromatic leukodystrophy,
adrenomyeloleukodystrophy, spinocerebellar
disorders) .