Neuro Final: Disorders

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Hypokinetic dyskinesia

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reduced voluntary movement
akinesia – no movement; bradykinesia – slowed movement
result of:
-loss of dopaminergic input from SNc (Parkinson’s disease)
loss of striatal inhibition of GPi & SNr in direct pathway
loss of GPe inhibition of subthalamic nucleus in indirect pathway

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Hyperkinetic dyskinesia

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increased involuntary movement
Results from decreased inhibition of the thalamus

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Rigidity

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increased resistance to passive limb movement due to Simultaneous contraction of flexors and extensors
- Clasped knife rigidity with UMN lesions
- Cogwheel rigidity associated with Parkinson’s disease: rigidity with superimposed tremor

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Dystonia

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sustained distorted body positions
-Torticollis: neck muscles
-Spasmodic dysphonia: laryngeal muscles
-Writer’s cramp: hand muscles
treat w/ botulinum toxin

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Resting (static) Tremor

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when limbs relaxed, decreases with volitional movement
-Basal Ganglia lesion (parkinsons)

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Postural Tremor

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seen when voluntarily maintaining a position against gravity, disappears at rest
-Cerebellar damage or Neuromuscular disease (MS)

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Kinetic (Action) Tremor

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occurs during active voluntary movement, absent at rest
-Intention (ataxic): tremor increases as goal-directed movement nears target; indicates cerebellar lesion, a feature of appendicular ataxia

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Idiopathic Parkinson’s disease

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A hypokinetic disorder, caused by degeneration of dopaminergic neurons in SN pars compacta
Clinical triad: bradykinesia, cogwheel rigidity, resting tremor
Develops asymmetrically, unilateral
Parkinsonian gate: slow, shuffling, narrow base

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Treatments for Idiopathic Parkinson's

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dopamine replacement by prescribing L-dopa
Pallidotomy: lesion globus pallidus to reduce inhibition of thalamus
Deep brain stimulation: Implant stimulators under patient control in STN or GPi

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(Atypical) Parkinsonism

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symmetrical, usually no resting tremor, non-responsive to L-dopa

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(Atypical) Parkinsonism:
Striatonigral degeneration

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parkinsonism with no response to L-dopa (input neurons from SNc and target neurons in stratum are gone; applies to all 3 syndromes here)

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(Atypical) Parkinsonism:
Shy-Drager syndrome

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parkinsonism + autonomic disturbances;
IMLCC also degenerates (orthostatic hypertension, incontinence, impotence)

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(Atypical) Parkinsonism:
Olivopontocerebellar atrophy

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parkinsonism + ataxia

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(Atypical) Parkinsonism:
Progressive supranuclear palsy

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widespread degeneration at midbrain-diencephalic junction
loss of vertical eye movements (neurodegeneration in the tectum); wide-eyed stare
rigidity initially more proximal (neck more than limbs), bradykinesia, frequent falls

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(Atypical) Parkinsonism:
Dementia with Lewy bodies

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Lewy bodies (sign of neurodegeneration) in SN responsible for parkinsonism; Lewy bodies throughout cerebral cortex responsible for dementia

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(Atypical) Parkinsonism:
Dementia pugilistica

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parkinsonism with cognitive decline, noted in boxers

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Huntington’s Disease

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initially presents as hyperkinetic disorder
Neurodegeneration of GABAergic striatal neurons (caudate & putamen)
-loss of "brake" on hypo
initial symptoms: clumsiness, mild chorea and behavioral/psychological changes
characteristic symptom: dyskinetic gait with choreic, ballistic or writhing movements while walking

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Hemiballismus

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•Hyperkinetic disorder due to lesion of contralateral subthalamic nucleus
•Loss of excitatory input to GPi/SNr in indirect pathway results in severe depression of inhibitory output to thalamus = disinhibition of thalamus and an inability to brake extraneous movements
•Patient presents with unilateral flinging movements, usually of upper extremity

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Wilson’s Disease

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disorder of copper excretion, causing progressive degeneration of liver and striatum
-Dysarthria & facial dystonia
-rigidity and “wing-beating” tremor

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Sydenham’s (rheumatic) chorea

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onset of acute rheumatic fever
Antistreptococcal antibodies cross-react with striatal neurons
Develops into chorea, obsessive-compulsive behaviors

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Tourette’s syndrome

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Persistent motor & vocal tics
•Abnormal signaling between basal ganglia and cortex results in an inability to suppress unwanted behaviors; involvement of motor, prefrontal & limbic channels

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bilateral temporalectomy
patient HM

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severe declarative memory

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dorsal medial thalamic nucleus damage
Patient NA

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severe anterograde amnesia and retrograde amnesia of 2 years

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Korsakoff’s/Wernicke-Korsakoff syndrome

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thiamine deficiency (alcoholism)
bilateral necrosis of mammillary bodies and medial diencephalic structures
anterograde and retrograde amnesia
-Confabulate: spurious answers to questions rather than saying they do not remember – frontal lobe dysfunction

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Hypoxia of Hypocampal pyramidal neurons

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short term deprivation - transient global amnesia - unaware of events preceding accident and temporarily unable to form new memories
longer term deprivation, eg. cardiac arrest - death of pyramidal neurons and defective memory formation

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Seizures

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episode of abnormally synchronized and high-frequency firing of neurons resulting in abnormal behavior or experience

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epilepsy

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disorder with a tendency for recurrent unprovoked seizures: ictal – during, postictal – immediately after, interictal – between

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Amygdalectomies (surgically or trauma)

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will eliminate/decrease aggressive behavior, but also produce an emotionally "flat" individual

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bilateral temporalectomy
patient HM

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severe declarative memory

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dorsal medial thalamic nucleus damage
Patient NA

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severe anterograde amnesia and retrograde amnesia of 2 years

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Korsakoff’s/Wernicke-Korsakoff syndrome

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thiamine deficiency (alcoholism)
bilateral necrosis of mammillary bodies and medial diencephalic structures
anterograde and retrograde amnesia
-Confabulate: spurious answers to questions rather than saying they do not remember – frontal lobe dysfunction

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Hypoxia of Hypocampal pyramidal neurons

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short term deprivation - transient global amnesia - unaware of events preceding accident and temporarily unable to form new memories
longer term deprivation, eg. cardiac arrest - death of pyramidal neurons and defective memory formation

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Seizures

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episode of abnormally synchronized and high-frequency firing of neurons resulting in abnormal behavior or experience

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epilepsy

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disorder with a tendency for recurrent unprovoked seizures: ictal – during, postictal – immediately after, interictal – between

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Amygdalectomies (surgically or trauma)

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will eliminate/decrease aggressive behavior, but also produce an emotionally "flat" individual

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Klüver-Bucy syndrome - removal of both temporal lobes in monkeys

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psychic blindness: see without understanding; indiscriminately approach and examine even though harmful
over attentiveness: orally examine everything
sexual hyperactivity: indiscriminate sexual activity
-(removal of occipital-temporal ‘what’ visual pathway → compensation from occipital-parietal ‘where’ stream)
emotional changes - tame and placid (removal of amygdala)

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Post-traumatic stress disorder (PTSD)

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emotional illness that was first formally diagnosed in soldiers and war veterans caused by terribly frightening, life-threatening experiences
symptoms include re-experiencing the trauma, avoidance, hyperarousal

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Aging affects on brain

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Reduced volume of cortex: reduction in size of gyri (increased sulci), enlarged ventricles
-not loss of neurons, loss of white matter connection
Impaired memory functioning: impaired declarative, functioning
--non-declarative intact
Impaired attentional mechanisms: reduced suppression of irrelevant information, reduced filter

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Agnosia

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(lack of knowledge) loss of ability to recognize familiar objects, can be very specific (e.g., faces: prosopagnosia) and affect a single sense (e.g., auditory, visual, tactile)

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Anosognosia

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lack of awareness of functional deficits

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Apraxia

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loss of ability to correctly perform a motor response in the absence of damage to language, primary motor or cerebellar pathways

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Wada’s Procedure

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preparation for neurosurgery
complete loss of sensation and paralysis on opposite side of body
no speech if anesthetized hemisphere dominant
if bilateral, some deficits in speech production (but not total loss) with injection on either side

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Dominant (left) hemisphere damage

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Aphasia
Right hemiparesis
Right-sided sensory loss
Right visual field defect
Poor right conjugate gaze
Dysarthria
Difficulty reading, writing, or calculating

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Non-dominant (right) hemisphere damage

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Hemineglect, e.g., neglecting of left visual field or left body, and spatial disorientation
Left hemiparesis
Left-sided sensory loss
Left visual field defect
Poor left conjugate gaze

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Aphasia

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Partial or complete loss of language abilities following damage (usually to the dominant hemisphere), often without the loss of cognitive faculties, sensory loss, or the ability to move the muscles used in speech
infarct of left MCA
Affects both written (reading and writing) and spoken language

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Agraphia

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impaired writing ability
most aphasics also exhibit agraphia
can occur in isolation with lesion in inferior parietal lobe of dominant hemisphere

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Alexia w/o Agraphia

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PCA lesion in dominant occipital cortex into corpus callosum
contralateral visual field deficit
disconnection syndrome: lesion of the connection between right visual areas and left hemisphere language centers
cannot decode language-related visual information
comprehends auditory speech and can write and speak normally

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Alexia with agraphia

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dominant parietal lobe (angular gyrus); aphasia may be mild, limited to dysnomia and paraphasias (unintentional substitution of syllables, words or phrases

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Gerstmann's syndrome

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Larger lesion in area of angular gyrus of parietal lobe
Severe disorder of both reading and writing but auditory comprehension and speech are intact.
Agraphia
Acalculia
Right-left disorientation
Finger agnosia

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Apraxia

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inability to correctly perform motor action in response to command
Approximately 1/3 of aphasics also exhibit apraxia

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aphemia-foreign accent syndrome

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apraxia of speech articulation, sparing written language resulting from small lesion in operculum of dominant hemisphere (foreign accent syndrome)

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Brocas's Aphasia

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Telegraphic style of speech, nouns and verbs only, no function words (ifs, ands,buts)
Difficulty in speech production, but makes sense
Anomia - "no name "- cannot find right word
Comprehension generally good, except subject/object discriminations
Impaired repetition
Writing and reading similarly impaired
Can result from infarct in left MCA, superior division: additional symptoms would include dysarthria, right hemiparesis of arm and face, frustration (aware of deficit)

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Wernicke's Aphasia

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Word Salad: sounds fluent, effortless, but nonsense
Lack of Comprehension
Reading and writing similarly affected
Naming impaired
Makes many paraphasic errors (substituting words or parts of words)
Impaired repetition
Can result from infarct in left MCA, inferior division, often accompanied by contralateral superior quandrantanopia
Limited awareness of deficiency, unconcerned

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Global Aphasia

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Wernicke's + Broca's, large MCA lesion or subcortical damage

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Conduction Aphasia

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damage to arcuate fasciculus, sparing Broca's and Wernicke's areas
impaired repetition
normal fluency and comprehension
impaired naming, paraphasic errors

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Sensory Transcortical aphasias

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Wernicke’s with repetition intact
MCA-PCA watershed
Damages connections from Wernicke's to parietal and temporal lobes

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Motor Transcortical aphasias

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Broca’s but with repetition intact
ACA-MCA watershed
Damages connections from Broca's area to prefrontal cortex

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Anomic Aphasia

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Difficulty with naming, may include paraphasias
May be category specific, associated with limited damage to areas of temporal lobe in “what stream” of visual processing

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Dyslexia

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Developmental deficit primarily affecting reading
Normal intelligence and spoken language
difficulty learning relationships between written alphabetic language and sounds
early intervention helps compensate (rewire)

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Stuttering

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developmental disorder
abnormal cortical activation patterns during spoken speech, appear to initiate motor program before preparation of articulatory code