lecture 4 GD: demyelinating diseasees and ataxic disorders Flashcards by sophia carrasco

what is Acute Disseminated Encephalomyelitis (ADEM)

Acute demyelinating illness which commonly follows an infection or vaccination (75%)

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Acute Disseminated Encephalomyelitis (ADEM)
is more common in children or adults

children

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what does the MRI show for Acute Disseminated Encephalomyelitis (ADEM)

bilateral symmetric inflammatory changes

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Acute Disseminated Encephalomyelitis (ADEM)
is usually ____ but may be recurrent

monophasic

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Acute Transverse Myelitis is a___ ___ disorder

spinal cord

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Acute Transverse Myelitis presents with

back pain , sensory level , sphincter disturbance and paraparesis

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what may be the first espoisode of MS especially if brain MRI is abnormal

Acute Transverse Myelitis

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what is acute or subacute onset of monocular visual blurring or loss of, central blind spot

optic neuritis

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when will u have pain with optic neuritis

eye movements

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what will u loss with optic neuritis

color vision

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optic neuritis is a very common initial symptoms in what

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what are factors increasing risk for MS

younger age, female sex, previous sensory symptoms, multiple MRI lesions.

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what is the treatment for optic neuritis

IV steroids

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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

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what is an immune mediated disease of CNS

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what is the leading cause of non traumatic disability in young adults

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Higher rates with increasing distance from the equator, especially north is common in what

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MD reduces life expectancy by how many years

7-14 years

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MS can lead to what 3 things

physcial disability , cognitive impairment , and decreased quality of life

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what are the multiple environmental risk factors for MS

– EBV exposure
– Low sun exposure
– Obesity
– Smoking

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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

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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

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what is steady increase in disability without attack and is 10-20% of MS cases

primary progressive MS

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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

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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

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

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