Neurological Disorders III

What is Multiple Sclerosis (MS)?

Progressive demyelinating disease of the CNS

What is MS Pathophysiology?

Nerve fibers of brain, spinal cord, and optic nerve are affected

What are Symptoms of MS?

Depending on the location and extent of the lesions: Optic neuritis: visual disturbances like diminished acuity, dimness, loss of color perception.

What does Unilateral mean?

Usually refers to symptoms or conditions affecting one side of the body.

What is Diploplia?

A condition characterized by double vision.

What Fatigue?

A state of extreme tiredness or exhaustion.

What is Muscle weakness?

Reduced strength in limbs and face.

What is Swallowing difficulty?

Challenges in the ability to swallow food or liquids.

What is Speech disturbance?

Problems with speaking clearly or coherently.

What is Spasticity?

Involuntary muscle spasms that can be spontaneous or movement-induced.

What is Cognitive dysfunction?

Impairment in cognitive functions such as memory and reasoning.

What are Bladder and bowel problems?

Issues related to the control and function of the bladder and intestines.

What are Sensory symptoms of MS?

Numbness, tingling, and/or pain experienced in various body parts.

What is a Tremor?

Involuntary, rhythmic muscle contractions leading to shaking movements.

What is Vertigo?

A sensation of spinning or dizziness.

Define Ataxia.

Lack of voluntary coordination of muscle movements.

Define Corticosteroid.

Drug used to treat acute symptoms, such as glucocorticoids like prednisone.

What is Interferon-beta?

A drug used to modify the course of multiple sclerosis and treat chronic symptoms.

Define Immunosuppressants.

Medications like methotrexate that help modify the immune response.

What is Baclofen?

A muscle relaxant used to treat spasticity.

Define Amyotrophic Lateral Sclerosis (ALS).

A neuromuscular disease also known as Lou Gehrig's disease that progressively affects motor function.

Define Onset of ALS.

Typically occurs between 40-60 years of age.

What is the Mean survival in ALS?

Average survival time is 2-5 years after diagnosis.

Define Mitochondrial dysfunction.

A condition leading to neuron apoptosis in ALS.

Define TAR DNA-binding protein of 43 Kda (TDP-43).

A protein associated with misfolded proteins in ALS pathology.

What is Riluzole?

An anti-glutamate drug used in the treatment of ALS.

Define Parkinson's Disease?

A progressive neurodegenerative disease affecting dopamine neurons in the midbrain.

What are Cardinal symptoms of Parkinson's Disease?

Include rigidity, bradykinesia, and resting tremor.

What are symptom of Parkinson's disease?

Loss of sense of smell, Insomnia, and Drooling difficulty swallowing, Difficulty initiating and stopping movements, Stooped posture and shuffling gait, Dementia, Lack of arm swing, Occasional 'freezing' episodes, Mask-like face and seborrhea, and Micrographia and microphasia

Define Pill rolling rhythmic tremor.

A symptom of Parkinson's disease characterized by a specific type of tremor.

What is the etiology of Parkinson's disease?

The cause is not fully understood.

Define Familial PD.

A small percentage of Parkinson's disease cases that are hereditary due to a genetic mutation in the α-synuclein gene.

Define Protein misfolding and aggregation.

A process implicated in the pathophysiology of Parkinson's disease.

Define Idiopathic PD.

Parkinson's disease with no known cause, potentially influenced by multiple factors.

Pathological features of Parkinson's disease

Alpha synuclein misfolding and aggregation, Protein degradation dysfunction, Microglial activation, Lewy body formation.

What is the Current treatment for Parkinson's disease?

no cure.

What are Treatment strategies for PD?

Includes increasing dopamine levels, stimulating dopamine receptors, decreasing dopamine breakdown, and inhibiting acetylcholine receptor activation.

Define Progressive neurodegenerative disease.

Alzheimer's disease, which leads to dementia.

What is the Diagnosis of alzheimer's for >60 yrs of age?

Typical age, although early onset is possible.

What is the Early onset genetic component for alzheimer's?

5%.

Define Cortical atrophy.

A characteristic of Alzheimer's disease involving the loss of neurons, particularly in the parietal and temporal lobes.

What is Enlargement of the ventricles?

A characteristic feature of Alzheimer's disease.

Define Choline acetyltransferase.

An enzyme whose deficit results from the loss of cortical neurons, leading to a deficit in ACh.

Define Neuritic (senile) plaques.

Hallmark feature of Alzheimer's Disease, consisting of patches of degenerating nerve terminals surrounding a core of amyloid β peptide (Aβ).

Define Aβ42.

A particularly problematic form of amyloid β peptide that is toxic and may induce inflammatory responses.

Define Neurofibrillary tangles.

Bundles of filaments made of hyperphosphorylated tau protein that encircle or displace the nucleus.

Define Tau hyperphosphorylation.

A process triggered by Aβ peptide that leads to the formation of neurofibrillary tangles.

What are Early symptoms of Alzheimer's Disease?

Short term memory loss and inability to acquire new information, difficulty handling money, and longer time to complete daily tasks.

What are Progressive symptoms of Alzheimer's Disease?

Compromised long term memory, changes in mood and personality, impaired cognition leading to dementia, and difficulty in performing learned motor tasks.

What is the Etiology of Alzheimer's Disease?

Unknown cause of the disease.

What is a Definitive diagnosis of Alzheimer's Disease?

Post-mortem histology of the brain.

What are Risk factors for Alzheimer's Disease?

diabetes, hypertension, hypercholesteronemia, and smoking; decreased risk includes exercise, intellectual activities, and a healthy diet.

What are Genetic risk factors for Alzheimer's Disease?

Mutations in the genes Presenilin 1 and 2, mutations in the gene for APP on chromosome 21, and the APO E4 allele.

What are Cholinesterase inhibitors?

Current treatment strategy for Alzheimer's Disease that alleviates symptoms, e.g., Aricept.

What is a NMDA receptor antagonist?

Current treatment strategy for Alzheimer's Disease that alleviates symptoms, e.g., Namenda.

Define Huntington's Disease.

Progressive neurodegenerative genetic disorder affecting 1/10,000 people due to a defect in the gene encoding for the huntingtin protein.

Define CAG repeats.

Polyglutamine (polyQ) expansion in the huntingtin protein that occurs when repeats exceed 40.

Define Inheritance of Huntington's Disease.

Offspring of an affected patient has a 50/50 chance of developing the disease.

What are Symptoms of Huntington's Disease?

Constant involuntary writhing or dance-like movements, gradual loss of cognitive function leading to dementia, with onset between 20-50 years old.

What is the Fatality of Huntington's Disease?

The disease is fatal in approximately 15 years.

What is the Pathophysiology of Huntington's Disease?

Considered a hyper dopaminergic condition due to less GABA release in the striatum, leading to increased dopamine release and massive neuronal loss in the caudate and putamen.

What is the Treatment of Huntington's Disease?

No cure exists, but treatments such as antipsychotics can help manage symptoms by blocking dopamine receptors.

What is Parkinson's Disease (PD)?

A neurodegenerative disorder characterized by the degeneration of dopamine-producing neurons in the brain.

What is Alzheimer's Disease?

A progressive neurodegenerative disease leading to memory loss and cognitive decline.

Define Huntington's Disease (HD).

A genetic neurodegenerative disorder characterized by the progressive breakdown of nerve cells in the brain.

Define Neuritic plaques.

Abnormal clusters of protein fragments that accumulate between nerve cells in Alzheimer's disease.

Define Senile plaques.

Another term for neuritic plaques, specifically associated with aging and Alzheimer's disease.

What is the Pathophysiology of MS?

Involves immune-mediated damage to myelin in the CNS, leading to neurological symptoms.

What is the Treatment of MS?

Includes disease-modifying therapies, corticosteroids, and symptomatic treatments.

What is the Pathophysiology of ALS?

Involves degeneration of motor neurons, leading to muscle weakness and atrophy.

What are Symptoms of ALS?

Common symptoms include muscle weakness, difficulty speaking, and respiratory issues.

What is the Treatment of ALS?

Currently focuses on symptom management and supportive care.

Define Neurochemical imbalance in PD.

Characterized by a deficiency of dopamine in the brain, leading to motor and non-motor symptoms.

What is the Etiology of PD?

The exact cause is unknown, but genetic and environmental factors are believed to contribute.

What are Symptoms of PD?

Common symptoms include tremors, rigidity, bradykinesia, and postural instability.

What is the Prognosis of PD?

Progressive disease with varying rates of progression; treatment can help manage symptoms.

What is the Pathophysiology of Alzheimer's disease?

Involves the accumulation of amyloid plaques and tau tangles, leading to neuronal death.

What are the Microscopic findings in Alzheimer's disease?

neuritic plaques and neurofibrillary tangles.

What are Symptoms of Alzheimer's disease?

memory loss, confusion, and changes in behavior.

What are Treatment strategies for Alzheimer's disease?

Focus on medications to manage symptoms and support for patients and caregivers.

What is the Pathophysiology of Huntington's disease?

Involves genetic mutation leading to neurodegeneration and movement disorders.

What is the Treatment of Huntington's disease

Includes medications to manage symptoms and supportive therapies.

What Area of the CNS is affected by MS?

The whole CNS

What type of neuron is affected by MS?

Nerve fibers of brain, spinal cord, and optic nerve are affected

What type of NT is affected by MS?

Acetylcholine (ACh), dopamine, glutamate and GABA

How are NT'S are affected by MS?

immune system triggers inflammation along the nerves and at the glial cells and Messages that pass along a demyelinated nerve become delayed or blocked.

What is the Theory or Etiology for MS?

unknown

What is the prognosis of MS?

can lead to disability and make it difficult to do routine things without assistance over time.

What are the hallmark features of MS?

fatigue, numbness or tingling, vision problems, balance and coordination difficulties, muscle weakness or spasms, and pain

What Area of the CNS is affected by ALS?

upper motor neurons in the cerebral cortex and the lower motor neurons in the brain stem and spinal cord

What type of NT is affected by ALS?

GABA, Glutamine, glutamate

How are NT'S are affected by ALS?

Loss of motor neurons

What are the hallmark features of ALS?

progressive muscle weakness, atrophy, and spasticity

What is the prognosis of ALS?

life expectancy is three to five years

What Area of the CNS is affected by PD?

nigrostriatal pathway in the midbrain

What type of NT is affected by PD?

dopamine and acetylcholine

What type of neuron is affected by PD?

dopaminergic neurons

What are the hallmark features of PD?

Bradykinesia, Tremor, Rigidity & aches, Hypomimia (masked face), Lack of global spontaneous body movements, Difficulties with fine motor skills, Difficulties turning in bed

What Area of the CNS is affected by AD?

parietal and temporal lobes

What type of neuron is affected by AD?

excitatory neurons