Neuromuscular Diseases

The meninges of the brain and spinal cord become inflamed due to an infectious organism

Can be bacterial or viral in origin

Vaccines are now available to help protect against __________

The inflammatory response can result in hydrocephalus, edema, and increased intracranial pressure Increased risk of infarction (obstruction of local circulation resulting in necrosis of tissue)

Stretch or pressure to the meninges will cause the cardinal sign of meningitis

Meningitis typically caused by __________ found in the upper respiratory tract

Neurological deficits remain in approximately ____%of people with bacterial meningiti

As inflammation progresses in bacterial meningitis, flexion of the neck will produce pain in the hips and knees (_____________ sign), due to tension on the meninges and neural structures

Bacterial meningitis may have pain with combined hip flexion and knee extension (Kernig’s sign), due to tension on the meninges and neural structures________

 Bacterial meningitis is treated with

 Antibiotics must be able to cross the ______ _____________ and achieve sufficient concentrations within the cerebrospinal fluid (CSF)

Treatment for viral meningitis is typically

An acute inflammatory disease of the tissue of the brain caused by direct viral invasion or hypersensitivity initiated by the virus.  Inflammation primarily of the gray matter

Encephalitis, an acute inflammatory disease of the tissue of the brain caused by direct viral invasion or hypersensitivity initiated by the virus.  Inflammation primarily of the _____ matter

Encephalomyelitis can result from other viral infections:

Clinical manifestations of encephalitis depend on the causal agent but can include

Rare forms of spongiform  encephalopathies  transmitted by prions  (infectious proteins that are abnormal forms of normal cellular proteins) that include: ◦Creutzfeldt-Jakob Disease ◦Bovine spongiform encephalopathy

The _______contains no nucleic acid but can replicate within the nervous system

Accelerated death of _______cells (nerve cells in the middle layer of the cerebral cortex )resulting in ataxia (the inability to coordinate voluntary muscular movements)

purkinje’s cells

the inability to coordinate voluntary muscular movements

treatment for prion diseases;

Once diagnosed, rapid progression typically occurs and eventually fatal

• AMYOTROPHIC LATERAL SCLEROSIS (ALS) • MULTIPLE SCLEROSIS (MS) • PARKINSON’S DISEASE

Cause is unknown and characterized by degeneration and scarring of the motor neurons in the lateral aspect of the spinal cord, brainstem, and cerebral cortex

“Most physically devastating of the neurodegenerative diseases”

More commonly known as Lou Gehrig’s disease

Peripheral nerve changes result in muscle fiber atrophy

Familial ALS (FALS) occurs in ____% of all ALS cases (inherited ________ trait)

ALS effects the pyramidal cells (pyramid shaped cells in the cerebral cortex) of the _____________and the surrounding premotor areas, sensory cortex and temporal cortex. Extensor muscles become weaker than flexor muscles

May see ________signs (neurons originating in the medulla) ◦ Difficulty with articulation ◦ Difficulty chewing ◦ Difficulty swallowing

Inability to hold the eye closed against pressure is a standard clinical

Fasciculations (_____________) of the tongue are present with lower motor neuron dysfunction

Dysarthria associated with ____ dysfunction ◦ Inability to shout or sing ◦ Hoarse voice quality

In ALS death usually occurs in ___ years after diagnosis, primarily from pneumonia related to respiratory compromise

Progressive dementia characterized by a slow decline in memory, language, visual spatial skills, personality and cognition

Incidence increases with age  Growing evidence of relationship between AD and vascular disease  May have a genetic component

No single cause  Loss of neurons  Changes in neurotransmitter

region of the brain involved in forming, storing and processing memory

Amygdala

First brain structure to show pathologic findings is the cerebral cortex ◦Select neurons: pyramidal cells with __________ __ _________ projections

Two characteristic brain findings of AD

Prognosis: fatal in 7-11 years

No cure, only symptomatic and supportive care  Often first functional sign is the inability to learn new information

Progressive hereditary disease characterized by abnormalities of movement, personality disturbances, dementia

Choreic movement:

Autosomal dominant disease; all that inherit the gene will develop symptoms of the disease

Risk of Huntington's disease in each child of an affected adult

Ventricles enlarge as a result of the atrophy of the _______ ______ in Huntington's disease.

Extensive loss of small and medium sized neurons  Atrophy of the putamen correlates with neurological symptoms  Early on, involuntary movements may be thought of as “restlessness” (face and UEs)

In Huntington' disease, atrophy seen in the __________ and putamen

Chorea is increased by activities that require concentration or by increased emotions or task complexity. How do you test?

Neuropsychologic and psychiatric disorders are a part of Huntington’s disease:

____% of persons affected with Huntington’s disease will attempt suicide

Patient education regarding safety issues  If bradykinesia present, may have tendency to “freeze”   Motor learning may be limited due to the disease processes  Positioning important

Hallmark: Sclerotic plaques distributed throughout CNS (Central Nervous System)

In MS demyelization is ?

In MS there is primarily _______ matter damage or lesions

lesions slow or block neuronal transmission resulting in

MS occurrence in females:males is

In US approximately __________ people are affected by MS

Uncommon before adolescence. Incidence rises from teen years through age 35 and declines gradually

MS a disease of ___________climates. In both hemispheres, prevalence ___________ with decreasing latitude