lecture 4 GD: demyelinating diseasees and ataxic disorders Flashcards
what is Acute Disseminated Encephalomyelitis (ADEM)
Acute demyelinating illness which commonly follows an infection or vaccination (75%)
Acute Disseminated Encephalomyelitis (ADEM)
is more common in children or adults
children
what does the MRI show for Acute Disseminated Encephalomyelitis (ADEM)
bilateral symmetric inflammatory changes
Acute Disseminated Encephalomyelitis (ADEM)
is usually ____ but may be recurrent
monophasic
Acute Transverse Myelitis is a___ ___ disorder
spinal cord
Acute Transverse Myelitis presents with
back pain , sensory level , sphincter disturbance and paraparesis
what may be the first espoisode of MS especially if brain MRI is abnormal
Acute Transverse Myelitis
what is acute or subacute onset of monocular visual blurring or loss of, central blind spot
optic neuritis
when will u have pain with optic neuritis
eye movements
what will u loss with optic neuritis
color vision
optic neuritis is a very common initial symptoms in what
MS
what are factors increasing risk for MS
younger age, female sex, previous sensory symptoms, multiple MRI lesions.
what is the treatment for optic neuritis
IV steroids
Left frontal lesion on FLAIR, posterior frontal-parietal WM lesion, and right frontal periventricular lesion and Dawson’s Fingers are common finding in what disease
multiple sclerosis
what is an immune mediated disease of CNS
MS
what is the leading cause of non traumatic disability in young adults
MS
Higher rates with increasing distance from the equator, especially north is common in what
MS
MD reduces life expectancy by how many years
7-14 years
MS can lead to what 3 things
physcial disability , cognitive impairment , and decreased quality of life
what are the multiple environmental risk factors for MS
– EBV exposure
– Low sun exposure
– Obesity
– Smoking
Movement of autoreactive Tcells and demyelinating antibodies from the systemic circulation into the CNS through disruption of the blood-brain barrier is a pathogenesis of what
MS
what is unpredictable attacks which may or may not leve permanent deficits followed by periods of remission and is 80-90% of cases of MS
relapsing remitting MS
what is steady increase in disability without attack and is 10-20% of MS cases
primary progressive MS
what is initial relapsing remittign ms that suddenly being to have decline without periods of remission and follows on from relapsing/remitting
secondary progressive MS
what is progressive relapsing MS
steady decline since onset with super imposed attacked
what is better and worse prognosis for MS .. a low lesion load on initial MRI or high lesion load
high is worse
what are the main symptoms of MS for the central systems
-faitgue
-cognitive impairment
-depression
-unstable mood
what are symptoms int eh visual system for MS
-nystagmus
-optic neuritis
-diplopia
____ and ___ tract lesions are motor symptoms in MS
corticospinal and ccorticobulbar
what are the symptoms for corticopsinal and corticobulbar tract lesions for MS
UMN spastic weakness and spastic parapresis most common
what are the cerebellar pathway motor symptoms in MS
Ataxia, tremor, incoordination, scanning speech.
for the senosory symptoms for MS ____ tract lesion is come resulting in dysesthesias and pain
spiniothalamic
what phenomenon is this Electric sensation passing down the back and limbs upon flexion of the neck.
lhermitte’s
what are brainstem symptoms
Nystagmus,
diplopia,
dysphagia
facial weakness ,
vertigo,
trigeminal neuralgia.
internuclear ophthalmoplegia is due to lesion where and there is inability to ____ ____ eye with nystagmus on abduction of contralateral eye
medial longitudinal fasciculus
adduct ipsilateral
what is “subcortical” dementia in patients with MS
information processing , visuospatial , memory , and executive dysfunction
what diagnostic testing do u use to diagnosis MS
MRI (brain and SC) most important
evoked potentials
CSF
if the MRI is not abnormal can it be MS
no
what may be elevated in CSF for MS
white blood cells (<50) and protein
Oligoclonal bands: Presence of 2 or more antibody clone what does it indicate
damaged blood brain barrier or immunoglobulin production in the brain
according to the 2017 mcdonald criteria for MS what does the diagnosis require
evidence of at least 2 areas of damage at different times
what are the 4 things the 2017 mcdonald criteria for MS include
-prior clinical attacks
- new lesions on subsequent MRI
- MRI with active or enhancing lesions with coexisting inactive lesion
-CSF oligoclonal banding
what are the 3 separation in time and space
– 2 different clinical attacks with different localizations
separated in time.
– 1 attack with MRI showing lesions – enhancing and non- enhancing
– 1 attack with MRI – and repeat MRI with new lesions 1 month later
what is Severe demyelinating disease of CNS due to reactivation of **JC virus **infecting oligodendrocytes
Progressive Multifocal Leukoencephalopathy (PML)
Progressive Multifocal Leukoencephalopathy (PML)
is an ____ infection in up to 86% of population
asymptomatic
Progressive Multifocal Leukoencephalopathy (PML)
is reactivation due to _____ associated with disease
immunosuppressant
Progressive Multifocal Leukoencephalopathy (PML)
is usually fatal within ___ year
1
what is the goal from MS
to prevent long term disability
a damaged brain fails to generate or sends inadequate inhibitory signals when then leads to what
spasticity
what is a Spinal cord TM longitudinally extensive lesion (>3 vertebral levels)
Neuromyelitis Optica (Devic’s Disease)
what is the prognosis of Neuromyelitis Optica (Devic’s Disease)
poor
what is the diagnostic testing for Neuromyelitis Optica (Devic’s Disease)
NMO IgG antibody serum
what is ataxia
disturbance in the smooth performance of voluntary motor acts
someone with ataxia will have errors in what 4 things
rate , range, force, and duration
which ataxia will they be unable to stand with feet together with eyes open or closed
cerebellar
what ataxia will they often to able to stand with feet together and eyes open but not with eyes closed
sensory
which ataxia is vertigo present , nystagmus present , may be able to stand with feet together but typically worse with eyes closed
vestibular
what ataxia is limb ataxia present , impaired vibration and position sense and depressed ankle reflexes
sensory
what is the lateral hemisphere of the cerebellum responsible for
motor planning for extermies
what is the flocculonodular lobe of the cerebellum responsible for
balance and vestibular reflexes
what is the vermis of the cerebellum responsible for
proximal limb and truncal coordination
what is the intermediate zone of the cerebellum responsible for
distal limb coordination
what are the clinical manifestations of cerebellar dysfunction
-limb ataxia /trunk ataxia , gait ataxia
- imparied stance
-tremor (intention)
when do u have truncal ataxia
vernal lesions
what side do u have ataxia for cerebellar dysfucntion
ipsilateral to lesion of cerebellar hemispheres
what is dysmetria
pateint misses targeted object
what is dysdiadochokinesia
imparied performance of rapidly alternating movements
what are 3 other clinical manifestation of cerebellar dysfucntion
• Hypotonia
• Speech deficits: mutism , ‘scanning’, dysarthria
• Oculomotor deficit-nystagmus or abnormal saccades
what is casues of an acute onset of ataxia
-infections
-trauma
-vascular lesion: strokes
- intoxication with alcohol
• Brain tumors such as medulloblastoma, astrocytoma, hemangioblastoma (usually with headache and papilledema)
• Alcoholic-nutritional (thiamine), vitamin E deficiency
• Paraneoplastic (related to cancer)
• Demyelinating cause (multiple sclerosis)
these are all casues of what kind fo ataxia
subacute
• Friedreich ataxia
• Spinocerebellar ataxia (SCA)
• Other cerebellar degenerations: Multi- Systems atrophy (parkinson’s like syndrome)
• Hereditary metabolic diseases: lipid storage diseases, mitochondrial
these are all causes of what kind of ataxia
chronic
what are the 4 cerebella syndromes
• Rostral vermis syndrome (anterior lobe)
• Caudal vermis syndrome (flocculonodular and
posterior lobe)
• Hemispheric syndrome • Pancerebellar syndrome
rostral vermis syndrome is usually chronic ___
alcoholics
what does rostral vermis syndrome feature with
-wide based stance and gait
-ataxia of gait
- arm coordination usually spared
- infrequent nystagmus , hypotonia and dysarthria
what is typically in children with meduloblastoma
caudal vermis syndrome
what are features of caudal vermis syndrome
– Axial dysequilibrium and staggering gait
– Little or no limb ataxia
– Sometimes spontaneous nystagmus and rotated postures of the head
what are features of cerebellar hemispheric syndrome
– Incoordination/ataxia of ipsilateral movements
– Particularly those that require fine motor control
what casues panacerebellar syndrome
– Infections, hypoglycemia, hyperthermia,
paraneoplastic, toxic-metabolic, hereditary
what are features of pancerebellar syndrome
– Bilateral cerebellar signs affecting limbs, trunk, and cranial musculature
spinocerebellar ataxia is ___ dominant
autosomal
what does ur cerebellum look like with spinocerebellar ataxia
small
friedreich ataxia is autosomal ___
recessive
which ataxia is gene located on chromosome 9
friedreich
friedreich ataxia is a multi organ disease process that affects ___, ___ and ____
heart , brain and nerves
• Ataxia begins in childhood or young adult hood’
• Loss of ambulation after10-15years
• Sensory loss (DRG/dorsalcolumndegeneration)
• Areflexia
• Foot deformities
• Scoliosis
• Cardiomyopathy
• Glucose Intolerance
• Death by age40
these are all clinical presentsation foe what
friedreich ataxia
