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NEUROMUSCULAR > lecture 4 GD: demyelinating diseasees and ataxic disorders > Flashcards

lecture 4 GD: demyelinating diseasees and ataxic disorders Flashcards

1
Q

what is Acute Disseminated Encephalomyelitis (ADEM)

A

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

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2
Q

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

A

children

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3
Q

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

A

bilateral symmetric inflammatory changes

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4
Q

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

A

monophasic

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5
Q

Acute Transverse Myelitis is a___ ___ disorder

A

spinal cord

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6
Q

Acute Transverse Myelitis presents with

A

back pain , sensory level , sphincter disturbance and paraparesis

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7
Q

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

A

Acute Transverse Myelitis

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8
Q

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

A

optic neuritis

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9
Q

when will u have pain with optic neuritis

A

eye movements

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10
Q

what will u loss with optic neuritis

A

color vision

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11
Q

optic neuritis is a very common initial symptoms in what

A

MS

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12
Q

what are factors increasing risk for MS

A

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

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13
Q

what is the treatment for optic neuritis

A

IV steroids

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14
Q

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

A

multiple sclerosis

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15
Q

what is an immune mediated disease of CNS

A

MS

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16
Q

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

A

MS

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17
Q

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

A

MS

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18
Q

MD reduces life expectancy by how many years

A

7-14 years

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19
Q

MS can lead to what 3 things

A

physcial disability , cognitive impairment , and decreased quality of life

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20
Q

what are the multiple environmental risk factors for MS

A

– EBV exposure
– Low sun exposure
– Obesity
– Smoking

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21
Q

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

A

MS

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22
Q

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

A

relapsing remitting MS

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23
Q

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

A

primary progressive MS

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24
Q

what is initial relapsing remittign ms that suddenly being to have decline without periods of remission and follows on from relapsing/remitting

A

secondary progressive MS

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25
Q

what is progressive relapsing MS

A

steady decline since onset with super imposed attacked

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26
Q

what is better and worse prognosis for MS .. a low lesion load on initial MRI or high lesion load

A

high is worse

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27
Q

what are the main symptoms of MS for the central systems

A

-faitgue
-cognitive impairment
-depression
-unstable mood

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28
Q

what are symptoms int eh visual system for MS

A

-nystagmus
-optic neuritis
-diplopia

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29
Q

____ and ___ tract lesions are motor symptoms in MS

A

corticospinal and ccorticobulbar

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30
Q

what are the symptoms for corticopsinal and corticobulbar tract lesions for MS

A

UMN spastic weakness and spastic parapresis most common

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31
Q

what are the cerebellar pathway motor symptoms in MS

A

Ataxia, tremor, incoordination, scanning speech.

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32
Q

for the senosory symptoms for MS ____ tract lesion is come resulting in dysesthesias and pain

A

spiniothalamic

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33
Q

what phenomenon is this Electric sensation passing down the back and limbs upon flexion of the neck.

A

lhermitte’s

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34
Q

what are brainstem symptoms

A

Nystagmus,
diplopia,
dysphagia
facial weakness ,
vertigo,
trigeminal neuralgia.

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35
Q

internuclear ophthalmoplegia is due to lesion where and there is inability to ____ ____ eye with nystagmus on abduction of contralateral eye

A

medial longitudinal fasciculus
adduct ipsilateral

36
Q

what is “subcortical” dementia in patients with MS

A

information processing , visuospatial , memory , and executive dysfunction

37
Q

what diagnostic testing do u use to diagnosis MS

A

MRI (brain and SC) most important
evoked potentials
CSF

38
Q

if the MRI is not abnormal can it be MS

A

no

39
Q

what may be elevated in CSF for MS

A

white blood cells (<50) and protein

40
Q

Oligoclonal bands: Presence of 2 or more antibody clone what does it indicate

A

damaged blood brain barrier or immunoglobulin production in the brain

41
Q

according to the 2017 mcdonald criteria for MS what does the diagnosis require

A

evidence of at least 2 areas of damage at different times

42
Q

what are the 4 things the 2017 mcdonald criteria for MS include

A

-prior clinical attacks
- new lesions on subsequent MRI
- MRI with active or enhancing lesions with coexisting inactive lesion
-CSF oligoclonal banding

43
Q

what are the 3 separation in time and space

A

– 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

44
Q

what is Severe demyelinating disease of CNS due to reactivation of **JC virus **infecting oligodendrocytes

A

Progressive Multifocal Leukoencephalopathy (PML)

45
Q

Progressive Multifocal Leukoencephalopathy (PML)
is an ____ infection in up to 86% of population

A

asymptomatic

46
Q

Progressive Multifocal Leukoencephalopathy (PML)
is reactivation due to _____ associated with disease

A

immunosuppressant

47
Q

Progressive Multifocal Leukoencephalopathy (PML)
is usually fatal within ___ year

A

1

48
Q

what is the goal from MS

A

to prevent long term disability

49
Q

a damaged brain fails to generate or sends inadequate inhibitory signals when then leads to what

A

spasticity

50
Q

what is a Spinal cord TM longitudinally extensive lesion (>3 vertebral levels)

A

Neuromyelitis Optica (Devic’s Disease)

51
Q

what is the prognosis of Neuromyelitis Optica (Devic’s Disease)

A

poor

52
Q

what is the diagnostic testing for Neuromyelitis Optica (Devic’s Disease)

A

NMO IgG antibody serum

53
Q

what is ataxia

A

disturbance in the smooth performance of voluntary motor acts

54
Q

someone with ataxia will have errors in what 4 things

A

rate , range, force, and duration

55
Q

which ataxia will they be unable to stand with feet together with eyes open or closed

A

cerebellar

56
Q

what ataxia will they often to able to stand with feet together and eyes open but not with eyes closed

A

sensory

57
Q

which ataxia is vertigo present , nystagmus present , may be able to stand with feet together but typically worse with eyes closed

A

vestibular

58
Q

what ataxia is limb ataxia present , impaired vibration and position sense and depressed ankle reflexes

A

sensory

59
Q

what is the lateral hemisphere of the cerebellum responsible for

A

motor planning for extermies

60
Q

what is the flocculonodular lobe of the cerebellum responsible for

A

balance and vestibular reflexes

61
Q

what is the vermis of the cerebellum responsible for

A

proximal limb and truncal coordination

62
Q

what is the intermediate zone of the cerebellum responsible for

A

distal limb coordination

63
Q

what are the clinical manifestations of cerebellar dysfunction

A

-limb ataxia /trunk ataxia , gait ataxia
- imparied stance
-tremor (intention)

64
Q

when do u have truncal ataxia

A

vernal lesions

65
Q

what side do u have ataxia for cerebellar dysfucntion

A

ipsilateral to lesion of cerebellar hemispheres

66
Q

what is dysmetria

A

pateint misses targeted object

67
Q

what is dysdiadochokinesia

A

imparied performance of rapidly alternating movements

68
Q

what are 3 other clinical manifestation of cerebellar dysfucntion

A

• Hypotonia
• Speech deficits: mutism , ‘scanning’, dysarthria
• Oculomotor deficit-nystagmus or abnormal saccades

69
Q

what is casues of an acute onset of ataxia

A

-infections
-trauma
-vascular lesion: strokes
- intoxication with alcohol

70
Q

• 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

A

subacute

71
Q

• 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

A

chronic

72
Q

what are the 4 cerebella syndromes

A

• Rostral vermis syndrome (anterior lobe)
• Caudal vermis syndrome (flocculonodular and
posterior lobe)
• Hemispheric syndrome • Pancerebellar syndrome

73
Q

rostral vermis syndrome is usually chronic ___

A

alcoholics

74
Q

what does rostral vermis syndrome feature with

A

-wide based stance and gait
-ataxia of gait
- arm coordination usually spared
- infrequent nystagmus , hypotonia and dysarthria

75
Q

what is typically in children with meduloblastoma

A

caudal vermis syndrome

76
Q

what are features of caudal vermis syndrome

A

– Axial dysequilibrium and staggering gait
– Little or no limb ataxia
– Sometimes spontaneous nystagmus and rotated postures of the head

77
Q

what are features of cerebellar hemispheric syndrome

A

– Incoordination/ataxia of ipsilateral movements
– Particularly those that require fine motor control

78
Q

what casues panacerebellar syndrome

A

– Infections, hypoglycemia, hyperthermia,
paraneoplastic, toxic-metabolic, hereditary

79
Q

what are features of pancerebellar syndrome

A

– Bilateral cerebellar signs affecting limbs, trunk, and cranial musculature

80
Q

spinocerebellar ataxia is ___ dominant

A

autosomal

81
Q

what does ur cerebellum look like with spinocerebellar ataxia

A

small

82
Q

friedreich ataxia is autosomal ___

A

recessive

83
Q

which ataxia is gene located on chromosome 9

A

friedreich

84
Q

friedreich ataxia is a multi organ disease process that affects ___, ___ and ____

A

heart , brain and nerves

85
Q

• 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

A

friedreich ataxia

NEUROMUSCULAR (15 decks)

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