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Cerebellar-Cerebral
MT II-lectures 13-15
108
Medical
Graduate
07/01/2013

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Term
Skeffington's 4 Circles
Definition
Anti-gravity (VOR)
Centering (saccades/vergence/magno)
Identification (fixations/pursuits/acc./parvo)
Speech Language (left hemisphere)
Term
5 parts of the cerebellum discussed in class
Definition
Dorsal Vermis
Fastigial Nucleus
Flocculus
Paraflocculus
Peduncles
Term
Which part of the cerebellum is responsible for opsoclonus
Definition
Fastigial Nucleus
Term
The inferior peduncle talks to the ________-
Definition
Medulla
Term
Expected presentation of Floccululs & Paraflocculus lesions
Definition
Impairment of:
fixation (nystagmus)
Smooth pursuit
VOR
Term
Which types of nystagmus may manifest in Flocculus & Paraflocculus lesions?
Definition
Gaze-evoked
Downbeat
Rebound
Term
What type of nystagmus stems for Nodulus/Uvula Lesions (in the dark)
Definition
PAN
Term
A Broken Nodulus means a broken ___________
Definition
Broken VOR
Term
What type of nystagmus in primary position due to lesion of nodulus/uvula
Definition
Downbeat in primary position (PAN in dark)
Term
How is VOR affected by lesion of the nodulus/uvula
Definition
Increased duration of vestibular response
Term
Ocular manifestations of dorsal vermis lesion
Definition
Ipsilesional hypometric saccades
Contralesional hypermetric saccades
Eso deviation
longer pursuit latency
Term
Ocular manifestations of fastigial nucleus lesion
Definition
Saccadic hypermetria
Term
2 places in the brain that can 'cause' lateropulsion
Definition
Dorsal Medullary
Cerebellum
Term
Vascular Cerebellar Infarctions occur in which 3 arteries
Definition
Superior Cerebellar
Posterior Inferior Cerebellar
Anterior Inferior Cerebellar
Term
Which is the most common type of cerebellar stroke
Definition
Superior Cerebellar Artery Infarction
Term
Which vessel supplies the lateral medula, inferior cerebellar peduncles, nodulus & uvula
Definition
Posterior Inferior Cerebellar Artery
Term
Which vascular cerebellar infarct is most commonly misdiagnosed as lateral medullary syndrome
Definition
Anterior Inferior Cerebellar Artery (most uncommon)
Term
Superior Cerebellar ArterySyndrome cause saccadic ipsipulsion or contrapulsion
Definition
Saccadic Contrapulsion
Term
Term for hypermetric contralesional saccades and hypometric ipsilesional saccades
Definition
Contrapulsion
Term
Superior Cerebellar Artery Syndrome causes ipsilateral or contralateral ataxia of gait and limbs
Definition
Ipsilateral
Term
Which cerebellar artery syndrome causes isolated vertigo and nystagmus and thus is often misdiagnosed as inner ear disease
Definition
Posterior Inferior Cerebellar Artery Syndrome
Term
Which cerebellar artery syndrome is associated with Bell's palsy and Hearing loss
Definition
Anterior Inferior Cerebellar Artery syndrome
Term
4 Unique Characteristics of Anterior Inferior CerebellarArtery Syndrome
Definition
Gaze-evoked Nystagmus
Horizontal Gaze Palsy (6th nerve)
Ipsilateral facial motor palsy (7th nerve)
Deafness (8th nerve)
Term
3 Congenital Abnormalities of the Cerebellum discussed in class
Definition
Arnold-Chiari Malformation
Dandy-Walker Malformation
Walker-Warburg Syndrome
Term
Which type of Arnold Chiair has adult onset and symptoms are positional
Definition
Type I
Term
Symptoms of Arnold Chiari Type I
Definition
Regular headaches
Dizziness & Vertigo
Term
Which type of Arnold Chiari has childhood onset
Definition
Type II
Term
Term for cerebellar herniation outside the skull cavity
Definition
Encephalocele
Term
Which congenital cerebellar syndrome is due to an enlarged 4th ventricle
Definition
Dandy-Walker
Term
Dandy-Walker Malformation results in an absence of which neural structures
Definition
Corpus collosum
Cerebellar vermis
Term
Eye movement abnormalities in Dandy-Walker
Definition
Mild saccadic dysmetria
Nystagmus
Strabismus
Term
Walker-Warburg Syndrome is accompanied by which systemic disease
Definition
Muscular Dystrophy
Term
Life expectancy of Walker-Warburg
Definition
<3 years
Term
Which ocular sign (posterior pole) occurs in 100% of patients with Walker-Warburg?
Definition
Persistent Hyperplastic Primary Vitreous
Term
Posterior PHPV may resemble what form of retinopathy
Definition
ROP
Term
3 ocular manifestations of Walker-Warburg
Definition
Retinal Dysplasia
Microphthalmia
Coloboma
Term
Which are the types of oculomotor ataxia that are triggered by neurological stress?
Definition
Episodic Ataxias
Benign Recurrent Vertigo
Term
50% of patients with Episodic Ataxia Type I respond to this drug
Definition
Acetazolamide
Term
Episodic Ataxia Type I vs Type II
Which has an earlier age of onset
Definition
Type I
Term
Episodic Ataxia Type I vs Episodic Ataxia Type II vs. Benign Recurrent Vertigo
Which has the latest age of onset
Definition
Benign Recurrent Vertigo
Term
Episodic Ataxia Type I vs Episodic Ataxia Type II vs. Benign Recurrent Vertigo
Which has a female predominance
Definition
Benign Recurrent Vertigo
Term
Episodic Ataxia Type I vs Episodic Ataxia Type II vs. Benign Recurrent Vertigo
Which has the shortest duration of attacks
Definition
Type I
Term
Episodic Ataxia Type I vs Episodic Ataxia Type II vs. Benign Recurrent Vertigo
Which does not have stress as a trigger
Definition
None, all are triggered by stress
Term
Episodic Ataxia Type I vs Episodic Ataxia Type II vs. Benign Recurrent Vertigo
Which can be triggered by sleep deprivation and hormones
Definition
Benign Recurrent Vertigo
Term
Episodic Ataxia Type I vs Episodic Ataxia Type II vs. Benign Recurrent Vertigo
Which more commonly occurs with migraines
Definition
Benign Recurrent Vertigo
Term
Episodic Ataxia Type I vs Episodic Ataxia Type II vs. Benign Recurrent Vertigo
Which has the most oculomotor manifestations
Definition
Type II
Term
Episodic Ataxia Type I vs Episodic Ataxia Type II vs. Benign Recurrent Vertigo
Which is least responsive to Acetazolamide
Definition
Type I
Term
Episodic Ataxia Type I vs Episodic Ataxia Type II vs. Benign Recurrent Vertigo
Which is most responsive to Acetazolamide
Definition
Type II
Term
Which of the types of SCA are autosomal dominant
Definition
1, 2, 3, 6, 7, 8
Term
Describe Saccades seen in SCA1
Definition
hypermetric, slow saccades
Term
Which form of SCA has the longest lifespan
Definition
SCA7 -45 years
Term
Which 2 forms of SCA are associated with Pigment Maculopathy
Definition
SCA1 and SCA7
Term
Which 3 forms of SCA are associated with a geographical location
Definition
SCA2-Cuba
SCA3 Portugal
SCA6 Germany
Term
Which form of SCA is associated with downbeat nystagmus
Definition
SCA 6
Term
3 forms of recessive SCA
Definition
Friedrichs
Vitamin E deficiency
SCA with Saccadic Intrusions
Term
2 systemic conditions that may accompany Friedrich's Ataxia
Definition
Diabetes
Scoliosis
Term
Most common recessive ataxia
Definition
Freidreichs
Term
Eye movement abnormalities in Friedreichs Ataxia
Definition
Square wave jerks, decreased VOR gain
Term
Ocular abnormalities in Vitamin E Deficiency Ataxia
Definition
INO and slow saccades lead to ophthalmoplegia
Term
Low cholesterol can cause which of the ataxias
Definition
Vitamin E Deficiency Ataxia
Term
Do ocular motor apraxias affect voluntary or reflexive eye movements
Definition
Generally the voluntary are affected
Term
4 forms of Congenital Ocular Motor Apraxia
Definition
Cogan OM Apraxia
Ataxia Telangiectasia
Ataxia Oculomotor Apraxia
Joubert
Term
How do patients with Cogans OM Apraxia initiate their eye movements
Definition
They must use the reflexive eye movements-so they use head thrusts
Term
Which congenital OM Apraxia is accompanied by difficulty speaking
Definition
Ataxia Telangiectasia
Term
Apraxia-Telangiectasia
Compare the response to rapid head impulses vs. head translation
Definition
Good response to impulses, poor response to translation
Term
Which congential OM Apraxia is characertized by high cholesterol, low serum proteins and mental impairment
Definition
Ataxia with Oculomotor Apraxia Type I
Term
What causes Ataxia with Oculomotor Apraxia type I
Definition
Cerebellar Atrophy
Term
How is Friedriech's Ataxia differentially diagnosed from Ataxia-Oculomotor Apraxia type I
Definition
Freidreich's does NOT have oculomotor apraxia
Term
How may bloodwork differ between the 2 types of Ataxia-Oculomotor Apraxia
Definition
Type 2 has elevated IgG
Term
Which specific part of the cerebellum atrophies in Ataxia-Oculomotor Apraxia 2
Definition
Vermis
Term
Which substance, when used during pregnancy, can cause Dandy-Walker
Definition
Warfarin
Term
Joubert syndrome is caused by the absence or underdevelopment of which neural structure
Definition
Cerebellar Vermis
Term
What is unique about Joubert syndrome among the other oculomotor apraxias
Definition
Retinal Dystrophy
Term
What sign on MRI is indicative of Joubert
Definition
Molar Tooth sign (elongated superior cerebellar peduncles)
Term
Eye Movement Abnormalities in Joubert's Syndrome
Definition
Strabismus
Pendular/Seesaw Nystagmus
Alternating Skew Deviation
Term
FASD Triad
Definition
Facial Dysmorphology
Growth Deficiency
CNS Dysfunction
Term
Characteristic features of the ear in FASD
Definition
Railroad Track Ear and Hockey Stick Crease
Term
FASD involves the hippocampus, how does this affect their behaviour
Definition
Attention and memory deficits
Term
FASD involves the corpus callosum, how does this affect their behaviour
Definition
Hyperactivity
Term
3 Facial signs of FASD
Definition
Epicanthanl Folds
Smooth Philtrum
Thin Upper Lid
Term
Ocular Characteristics of FASD
Definition
Microcornea
Optic nerve hypoplasia
Strabisumus/Amblyopia
Congenital Cataracts
Term
How does FASD affect saccades
Definition
Saccadic dysfunction by increased latency
Term
Name 3 areas in the frontal lobe that contribute to eye movements
Definition
Frontal Eye Fields
Supplementary Eye Fields
Dorsolateral Prefrontal Cortex
Term
How do frontal eye field lesions affect saccades
Definition
Increased latency
Hypometria of saccades to contralateral side
Impaired suppression of saccades
Term
How do frontal eye field lesions affect pursuits
Definition
Impaired for targets moving towards side of lesion
Term
Defects in which 2 parts of the frontal lobe affects Memory guided saccades
Definition
Supplementary Eye fields and Prefrontal Cortex
Term
The Triad
Oculomotor Ataxia
Oculomotor Apraxia
simultagnosia
describes which syndrome
Definition
Balint Syndrome
Term
Location of lesion that causes Balint Syndrome
Definition
Bilateral Parietal lobe lesion
Term
Visually guided saccades in unilateral parietal lobe lesion vs. bilateral
Definition
Unilateral-increased latency
Bilateral-Increased latency and decreased accuracy
Term
Visual inattention in unilateral parietal lobe lesion vs. bilateral
Definition
Unilateral= Contralesional inattention
Peripheral= Peripheral inattention, aka Simultagnosia
Term
Abnormal eye movements due to lesion in primary visual cortex
Definition
Unable to generate saccades or pursuits to stimuli in blind hemifield
Term
Saccade abnormalities in Huntington's
Definition
difficulty initiating (apraxia)
difficulty suppressing antisaccades
Slow saccades
Term
Lesion of dorsal vermis leads to _____deviation
Definition
Eso deviation
Term
Lesion of NRTP leads to impaired _______________
Definition
Convergence
Term
Lesion of fastigial oculomotor region leads to ______deviation
Definition
Exodeviation
Term
Eye Movement abnormalities in Substantia Nigra Pars Reticulata
Definition
saccadic instrusios directed to contralateral side of lesion
Term
Blepharospasm is known to accompany which congenital condition
Definition
Congenital Glaucoma
Term
Tourette Syndrome Treatment
Definition
Clonidine
Term
Saccades in Early Parkinsons
Definition
Hypometric (esp upward) but with normal velocity
Term
Saccades in Advanced Parkinsons
Definition
Slow saccades
Term
Contrast saccades in Early Parkinsons vs. PSP
Definition
Parkinsons-hypometric in upward saccades with normal velocity
PSP-slow downward saccades with full range of motion
Term
Which of the Duane White Diagnoses is common in Parkinson's Disease
Definition
Convergence Insufficiency
Term
3 pharmacological treatments for Parkinsons
Definition
Levodopa/Carbidopa
MAOI-type B (Selegiline)
Anticholinergics
Term
Surgery options for Parkinson's disease
Definition
Thalamotomy
Thalamic Deep brain stimulation
Pallidotomy/pallidal stimulation
Subthalamotomy
Transplant of dopamine-producing cells
Term
Parkinson vs. PSP
which has the clinical feature of resting tremor
Definition
Parkinsons
Term
Parkinson vs. PSP
Which has the clinical feature of subcortical dementia
Definition
PSP
Term
Parkinson vs. PSP
One especially affects upward saccades and the other downward saccades, which is which
Definition
PD-hypometric saccades, esp upward
PSP-slow vertical saccades, esp. downward
Term
Distinctive eye movement abnormalities in Lewy Body Dementia
Definition
Vertical gaze palsy
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