Demyelinating and Ataxic Disorders

Question 1

Acute Disseminated Encephalomyelitis

Answer 1

-ADEM
-acute demyelinating illness following infection of vaccination
-common in children
-MRI shows bilateral symmetric inflammatory changes

S/s:
-sickness with decreased mental status
-no brainstem involvement

Question 2

Acute Transverse Myelitis

Answer 2

-spinal cord dysfuntion
-may be first episode of MS i
-can be caused by infections or autoimmune causes
if recurrent
S/s:
-back pain, sensory lecel, sphincter disturbance, paraparesis

Exam:
-UMNL symptoms
-bilateral (SCI)

Question 3

Optic Neuritis

Answer 3

-acute or subacute onset of monocular visual loss of blurring
-common with initial MS if recurrent

S/s:
-central blind spot
-pain with mmt
-loss of color vision and acuity
-afferent pupillary defect (optic)

Question 4

Multiple Sclerosis Etiology/Basics

Answer 4

-immune mediated disease of CNS
-movement of autoreactive T cells and demyelinating antibodies from systemic circulation into CNS through disruption of BBB
-leading cuase of non traumatic disability in young adults
-far from equator= higher rates
-females >males
-onset 20-30y
-reduces life expectancy by 7-14 years

Question 5

Multiple Sclerosis Causes

Answer 5

-cause unknown
-~20% of heritability
-movement of autoreactive T cells and demyelinating antibodies from systemic circulation into CNS through disruption of BBB

Environmental RK:
-EBV virus exposure
-low sun exposure
-obesity
-smoking

Question 6

Multiple Sclerosis Types

Answer 6

Progressive -Relaspsing MS:
-steady decline since onset with super attacks
-<10%

Secondary Progressive MS:
-intial relapsing remitting MS that suddenly begins to have decline w/o remission

Primary Progressive MS:
-steady increase in disability w/o attacks
-10-20%

Relapsing-Remitting MS:
-unpredictable attacks
-leave permanent deficits followed by remission
-80-90%

Question 7

Multiple Sclerosis Progression

Answer 7

Onset:
-85%: RRMS
-15%: PPMS

After 10 years:
-40%: RRMS
-15%: PPMS
-45%: SPMS

-as time goes on=more relapses=less brain volume= more disability and cognitive dysfunction= less relapses

most relapses at relapsing remitting after initial event, clinical diagnosis

Question 8

Multiple Sclerosis Prognosis

Answer 8

Better:
-caucasian
-monofocal onset
-low relapse rate
-no or low disabiltiy @5 yrs
-low lesion load on initial MRI

Worse:
-non white/males
-smoking or obesity
-multifocal onset
-high relapse rate
-disability @5yr
-MRI with high lesion load

Question 9

Multiple Sclerosis Symptoms (system)

Answer 9

Cognitive:
-fatigue
-cognitive impairment
-depression
-subcortical dementia

Motor:
-corticospinal and corticobulbar tract lesion w/ UMN spatic weakness (80%)
-spatic paraparesis most common
-Cerebellar Pathways w/ ataxia, temor, scanning speech (usually later)

Sensory:
-spinothalamic lesion (dysesthesias and pain)
-DCML less common
-Lhermitte’s Phenomenon: electric sensation passing down back and limbs with neck flx

Brainstem:
-nystagmus, diplopia, facial weakness, vertigo, dysphagia, trigeminal neuralgia
-internuclear ophthalmolegia

Question 10

Intranuclear Ophthalmoplegia

Answer 10

-lesion in medial longitudinal fasciulus
-inabiltiy to adduct ipsi eye with nystagmus on abd of contra

Question 11

Diagnosis and Treatment of MS

Answer 11

Diagnosis:
-uhthoff’s phenomenon (bathtub test)
-MRI of brain and SC (plaques)
-evoked potentials
-CSF: oligoclonal bands, high protein, elevated WBC

Tx:
-steroids
-plasma exchange
-symptomatic treatment (spasticity, sexual dysfunction, B/B, cognitive, depression, fatigue, temmors, sensory

Question 12

Progressive Multifocal Leukoencephalopathy (PML)

Answer 12

-severe demyelinating disease of CNS due to reactivation of JC virus infecting oligodendrocytes
-asymptomatic infection for 86%
-fatal in 1 yr

Question 13

Neuromyelitis Optica

Answer 13

-Devic’s Disease
-transverse myelitis AND optic neuritis often Bilat
-SC TM longitudinally extensive lesion
-more severe than MS
-female>males

Associated with:
-Postrema syndrome: hiccups, nausea, vom
-acute brainstem synrome
-narcolepsy

Diagnostic:
-NMO IgG anticbody

Question 14

Myelin Oligodendrocyte Glycoprotein Antibody disease

Answer 14

-children
-monophasic or relapsing
-TM, ON, and ADEM
-positive for MOG, neg for NMO

Question 15

Cerebellar Dysfunction

Answer 15

Ataxia:
-ipsi lesions: hemiphere
-trunk ataxia: vermis

Impared Stance
Tremor (intention)
Dysmetria
Dysdiadochokinesia
Hypotonia
Speech Deficits
Oculomotor Deficits

Question 16

Ataxia Causes

Answer 16

Acute Causes:
-intoxication
-strokes
-trauma
-infections

Subacute Causes:
-brain tumors
-alcoholic nutritional deficiency
-Demyelinating

Chronic Causes:
-friedreich ataxia
-spinocerebellar ataxia
-multi symptoms atrophy
-hereditary metabolic diseasea

Question 17

Rostral Vermis Syndrome

Answer 17

-usually chronic alcholics
-anterior lobe

S/s:
-wide based stance and gait
-ataxic gait
-arm coordination impared
-infrequent nystagus, hypotonia, dysarthia

Question 18

Caudal Vermis Syndrome

Answer 18

-flocculonodular lobe and posterior lobe
-typically in children with medulloblastoma

S/s:
-axial dysequilibium and staggering gait
-little to no ataxia
-sometimes spontaneous nystagmus

Question 19

Cerebellar Hemispheric Syndrome

Answer 19

-infarts, tumor, abscess

S/s:
-incoordination/ataxia of ipsi
for fine motor control

Question 20

Pancerebellar Syndrome

Answer 20

Causes:
-infections, hyoglycemia, hyperthermia, hereditary

S/s:
-bilat cerebellar signs of limbs, trunk, and head muscles

Question 21

Spinocerebellar Ataxias

Answer 21

-autosomal dominant inherited
-large groups of disorders
-onset at 3rd decade

S/s:
-ataxia, dysarthria, saccadic pursiut
-UMN, LM, seizures, tremors

Question 22

Friedreich Ataxia

Answer 22

-autosomal recessive inherited
-multi organ diseases affecting heart, brain, and nerves

S/s:
-ataxia in childhood
-loss of ambulation 10-15yrs
-sensory loss DCML
-areflexia
-foot deformities
-scoliosis
-cardiomyopathy
-death by age 40