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No one think about me....:(

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Access Google Drive with a Google account (for personal use) or Google Workspace account (for business use).

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Access Google Drive with a Google account (for personal use) or Google Workspace account (for business use).

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Cause:
Heredity!!!!!
Gene explanation: patients have more than 44 repeats of CAG --> abnormal!!!!
Can be autosomal dominant, autosomal recessive, or X-linked --> all possibilities =.=...
There are more than 100 types of SCA.....

S & S
SCA-I patients usually develop dysphagia, oculomotor disturbance, pyramidal and extrapyramidal signs, sensory deficits & mild cognitive decline.

Sign and symptom (Subramony & Ashizawa, 2014)

-progressive cerebellar ataxia

-dysarthria

-deterioration of bulbar function

[Early phase]

gait disturbance

slurred speech

difficulty with balance

slurred speech

balance problem

brisk deep tendon reflex

hypermetric saccades

nystagmus

mild dysphagia

[Later]

lower saccadic velocity

upgaze palsy

dysmetria

dysdiadochokinesia

hypotonia

[Advanced]

muscle atrophy

decreased deep tendon reflex

loss of proprioception

cognitive impairment

chorea

dystonia

bulbar dysfunction

Diagnosis:
MRI severe atrophy over brainstem & cerebellum.
EEG: show salient feature of epilepsy
EMG: reveal continuous motor unit activity
DNA test can be done on children.

Treatment:
No curable treatment.
Symptomatic relieve:
-Mm relaxant
-PT
-ST
-stem cell therapy (under investigation XD)

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Above is Fei Jai

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Gender - Male
Ethic - white people
Genetic syndrome - Li-Fraumeni syndrome, neurofibromatosis, nevoid basal cell carcinoma syndrome, tuberous sclerosis, Turcot syndrome, and von Hippel-Lindau disease
Ionizing radiation exposure
Work exposure to carbon tetrachloride
Increase of sugar intake
EM field exposure

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Improve strength, balance, endurance, decrease pain.

functional and aerobic exercise: low intensity
Also improve pulomnary function and prevent DVT

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-Grade IV
-Anaplastic, highly cellular tumor
-Aggressive proliferation to other brain tissue
a) cerebrum: through the temporal (31%), parietal (24%), frontal (23%), and occipital (16%) lobes
b)hypothalamus-thalamus area (20%)
c)cerebellum and posterior fossa (15%)
d)rare to brain stem and spinal cord
-Microvasculaization
-Presence of necrosis, hemorrhagic necrotic areas

Two classification:
Primary: Sudden onset without preexisting lesion
Secondary: Pathological evolution from low-grade astrocytic tumors and anaplastic astrocytomas (AA)

AA: Grade III
May not proliferate to other brain tissue
Lack of necrotic area
Moderate rate of mutation of gene

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patho.
-Malignant primary brain tumor with predominant astrocytic differentiation

-Grade IV-Glioblastoma :Cellularity, anaplasia, mitoses, microvascular proliferation and necrosis
-Usually supratentoria
-Glioblastomas are usually highly malignant—a large number of tumor cells are reproducing at any given time, and they are nourished by an ample blood supply. Dead cells may also be seen, especially toward the center of the tumor. Because these tumors come from normal brain cells, it is easy for them to invade and live within normal brain tissue. However, glioblastoma rarely spreads elsewhere in the body
Two type:
primary: de novo
secondary: low-grade astrocytomas

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Prolong bed rest—>DVT—PT mobilization

Brain motor cortex affected—>ataxia-->gait training, PNF to improve coordination and proximal stability, interactive games (Wii, Seeme)

Weakness—strengthening ex.

Fall prevention—balance training

Fatigue—Regular light intensity exercise (Yoga, Taichi) + educate energy conservation technique

Pain—TENS

Cognition—suggest using memory tools e.g. schedule book

Alternating electric field therapy???

-a type of electromagnetic field therapy using low-intensity electrical fields to treat cancer.
- A  device manufactured by the Israeli company Novocure is approved in the United States and Europe for the treatment of newly diagnosed and recurrent glioblastoma multiforme (GBM)
-  despite earning regulatory approval, the efficacy of this technology remains controversial among medical experts

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No known cure for PSP.
Aims of treatments: supporting people living with PSP to manage symptoms.

Pharmacological treatment:
No medication is effective in halting the progression of the disease

Antiparkinsonian agents e.g. levodopa: slowness, stiffness, and balance problems, minimal and short-lasting improvement

Antidepressant drugs: pain relief and decreasing drooling

Onabotulinumtoxin A: rigidity, dystonia (e.g. blepharospasm, bruxism, and focal limb dystonia), sialorrhea

Botulinum: Excessive eye closing

Methylcellulose or methyl alcohol drops in the eyes: chronic conjunctivitis (reduced blink rate)

Ø  Recent approach - clearance of abnormally accumulated tau in the brain (related to pathophysiology of PSP)

Non-pharmacological intervention:

Gastrostomy: when there are swallowing disturbances or the definite risk of severe choking.

Electroconvulsive therapy (ECT): brain stimulation, treat major depression

Bifocals or special glasses called prisms: remedy the difficulty of looking down

Conventional treatment:
PT: Focus on fall prevention and exercises to improve balance.  Use weighted walking aids to prevent them from falling backwards, such as a walker.
Coordination exercises and reciprocal rhythmic movements to reduce rigidity, transfer training exercises for balance, gait training using weights strapped to ankles in parallel bar and visual tracking exercises.
Found to be useful in improving gait and balance and decrease in fall incidence
ST: Address eating and swallowing problem, associated with the progression of PSP.

HOWEVER
All individuals will lose their ability to walk eventually and need to progress to using a wheelchair. Severe dysphagia often follows, and patients will die within months.

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What are the differences between progressive supra-nuclear palsy and Parkinson's Disease?

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What are the differences between progressive supra-nuclear palsy and Parkinson's Disease?

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The key to diagnosing PSP is identifying early gait instability and difficulty moving the eyes, speech and swallow abnormalities, as well as ruling out other similar disorders, some of which are treatable.

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poor eyelid function, face muscles contracture, stiff neck

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gum arm ge! ngor hai fei por<3

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How is the prognosis of Progressive Supra-nuclear Palsy

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How is the prognosis of Progressive Supra-nuclear Palsy

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Progression since the onset of disease
Survivor function estimates by year since symptom onset in patients with PSP.

Median Time from Disease onset to First key motor impairment = 4 Years
Survival rate = x̄ = 6-7 Years
Major cause of death not due to the disease itself, but the potential COMPLICATIONS.

Common Complications:
- Aspiration pneumonia
- Pulmonary Embolism
- Falling

Timeline for symptoms development
S/Ss more common in the early stages: (~onset to 3 years after onset)
- Motor impairment
- Reading difficulty
- Postural instability
S/Ss more common in the Later stages:
- Constipation and urinary incontinence
- Supra-nuclear gaze palsy

Negative Prognostic Factors:
- Male gender
- Old age at onset
- Fall in first year after onset
- Early dysphagia
- Incontinence
- Onset of falls, speech problems, or diplopia within 1 yr
- Swallowing problems within 2 yr
- Presence of visual hallucination
- Under anti-psychotics medications
- Voice disturbance

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ngor hai fei jai

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:D

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Hihi

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Nice background pic!

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I am sleepy

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Hello! Look forward to class on Friday :)

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Hello! Look forward to class on Friday :)