Other Neuro Topics Flashcards
What age does Tourette’s Syndrome symptoms typically start?
Most commonly 6 years of age (5-10 years)
How long does it typically take (duration) for Tourette’s Syndrome to be diagnosed?
About 2 years from onset of symptoms
When do tics increase in severity or frequency?
Under stress (new school, move)
What are the three tic disorders?
Tourette’s
Persistent
Provisional
How is Tourette’s defined?
Both motor and vocal tics persistent for at least 1 year
How is Persistent tic disorder defined?
Motor or vocal tics persistent for at least 1 year
How is Provisional tic disorder defined?
Motor or vocal or both
Present for at least 1 year
How is Tourette’s diagnosed?
Clinical
What is the diagnostic requirements for Tourette’s?
-2+ motor ticks
-1+ vocal tics
-Tics present for at least 1 year
-Tics onset prior to age 18
-Symptoms are not related to medication or other medical condition
What are the other top two diagnoses that Tourette’s presents with?
ADHD and Anxiety
What is the goal of Tourette’s treatment?
Tic suppression
What drug class is the only approved for Tourette’s syndrome?
Neuroleptics
(haloperidol, pimozidole, aripiprazole)
What are two off label drug classes used to treat Tourette’s?
Alpha adrenergic’s and stimulants
Why would you not prescribe a stimulant to a child with Tourette’s?
Although shown helpful at reducing tic frequency they are contraindicated for Tourette’s due to MOA
Do you typically treat Tourette’s with pharmacologic therapy?
No, behavioral therapy is more appropriate as a first line. Only severe tics are treated with neuroleptics.
What is the disease trajectory of Tourette’s Syndrome?
Typically tics decrease during adolescence/early adulthood, and sometimes disappear completely. Rarely worsens with age (10-15% of cases).
What does ALS stand for?
Amyotrophic Lateral Sclerosis
What is the Epidemiology of ALS?
Rare progressive neurological disease
Most common in men than women, diagnosed most commonly between 55-75 years of age (can strike at any age)
More in caucasians and non-Hispanics
What occupation is highly associated with ALS?
Military service
What is the life expectancy after diagnosis with ALS?
Most die from respiratory failure usually 3-5 years from symptom onset
What is the pathology of ALS?
Not well understood, thought to have a genetic predisposition with environmental triggers.
Degeneration of the upper and lower motor neurons
What are the two types of ALS?
Sporadic and Familial
What is the sporadic type of ALS?
90% of cases, diagnoses at random with no clear associated risk factors or family history
What is the familial (genetic) type of ALS?
5-10% of cases, inherited from one autosomal dominant parent
What is the initial presentation of ALS?
Early muscle weakness and stiffness (generally painless), gradual in onset
Begins with neck/head muscles, limb, respiratory muscles
What are the later symptoms of ALS?
Fasciculations in arm, leg, shoulder or tongue
Muscle cramps and spacicity
Muscle weakness (eventually diaphragm)
Slurred and nasal speech, uncontrollable laughing/crying
Difficulty chewing or swallowing
Why is nutrition status critically important in ALS management?
Patients burn calories at a faster rate, concern for weight loss and malnourishment
Are sensory or motor neurons impaired in ALS?
Only motor neurons affected
What is the diagnosis and work-up for ALS?
Detailed Hx and PE (presence of upper and lower motor neuron involvement)
Other testing to rule out concerns (test for polio and HIV)
What is the treatment for ALS?
No treatment, treatment geared towards controlling symptoms (constipation, pain, sleep) or preventing complications
What are some non-pharmacological treatment for ALS?
PT, OT, Speech therapy and nutritional support
What is “breath stacking”
series of small breaths without exhaling, hold then cough
Used in ALS because exhalation doesn’t require a lot of work/energy
What is the Epidemiology of MS?
Most often appears in young adulthood, with incidence peaking at 30 years of age (more common in females, 3:1)
MS cases on the rise
What is the pathophysiology of MS?
T-cell mediated autoimmune disease - Cytotoxic T-cells target myelin and myelin producing cells that activate B-cells producing auto-antibodies against self (CNS damage)
Viral precursor theory?
What is a geographical (environmental) factor of MS?
Living further away from the equator
What are some risk factors of MS?
Age, gender, smoking, low vitamin D, and obesity, family history (first degree relative)
What are the most common symptoms of MS?
Fatigue, weakness, parasthesias, vision problems (retinal detachments, pain with occular movement), bladder problems, cognitive changes (brain fog, difficulty focusing, emotional changes)
What are the four types of MS
- Clinically Isolated Syndrome (CIS)
- Relapsing-remitting MS (RRMS)
- Secondary Progressive MS (SPMS)
- Primary Progressive MS (PPMS)
What is the most commonly diagnosed type of MS?
Relapsing-remitting MS (RRMS)
What is Clinically Isolated Syndrome (CIS)?
First episode of symptoms caused by inflammation, unsure diagnosis of MS. Seizure mimicking.
If brain lesions (white matter lesion) on MRI, 2nd episode highly likely to be diagnosed as RRMS - if no brain lesions then unlikely to develop
What is RRMS?
Relapses of new or increasing neuro sx then remission (where all symptoms are gone). No progression during remission.
What does RRMS progress to?
SPMS, progressive worsening of neuro function. Unique to every patient but progression is occuring even when in remission stages.
What is the worst stage of MS?
PPMS, worsening accumulation of disability from onset of symptoms early relapse or remission (persistently getting worse)
How is MS diagnosed?
Clinical diagnosis of history and PE
McDonald Criteria
- Damage in 2 areas of the CNS that date at different times
- CSF + oligoclonal bands (Spinal Tap)
What is an MRI finding of MS?
White matter lesions (dating can be helpful in diagnosis for different attacks)
What is the treatment for MS?
Immunotherapy agents (injectables, oral, and infused therapies)
Symptom management - fatigue is most common (stimulants)
What is the relapse management for MS?
Severe symptoms (vision loss, severe weakness) high dose steroids (prednisone or methylprednisolone) or ACTH (inflammatory process)
What is the most common cause of death in MS patients?
Secondary complications caused by immobility, chronic UTI, compromised swallowing and/or breathing
How is normal pressure hydrocephalus (NPH) simply defined?
Too much CSF in the ventricles
When does NPH usually occur?
Older adults (> 60 years of age), developing slowly over time
What is the presentation in NPH?
Dementia, difficulty walking, urinary incontinence, headache, nausea, difficulty with vision focus (subtle sx that gradually get worse)
Symptoms usually attributed to “getting older”
What is the diagnosis of NPH?
History and PE with mental status and gait evaluation
Labs, CT, MRI, or LP to rule out other cause
LP can demonstrate need for shunt placement
What is the treatment of NPH?
No cure or definitive management
Surgical shunt vs. non-surgical
What are the three W’s of NPH
Weird, wet, and wobbly (all are reversible)
What is the epidemiology of Myasthenia Gravis
Affects M:W equally (earlier in females and later in men)
What is neonatal myasthenia?
Mom with M. gravis pushes antibodies to baby in-utero and when born babies demonstrates symptoms (weakness). Typically resolves 4-6 weeks after birth.
What is the pathophysiology of M. gravis?
Interruption at the neuromuscular junction in that antibodies block acetylcholine receptors, impacts skeletal muscle fibers
What is the role of the thymus in M. gravis?
Thymus remains large (lymphoid hyperplasia), but the function is not fully understood (maybe incorrect instructions in developing T-cells)
What is the first noticeable symptom in M. gravis?
Weakness of eye muscles
What are the symptoms of M. gravis?
Ptosis (drooping of eyelids)
Diplopia
Facial expression changes
Dysphagia
Shortness of breath
Dysarthria (impaired speech)
Weakness in arms, hands, fingers, legs and neck
What is the most sensitive test for diagnosing MG?
Single fiber EMG - detects impaired nerve to muscle signal transmission
How is MG diagnosed?
Edrophonium test
History and PE
Blood work- Ach receptor antibodies and anti-MuSK antibodies
Single fiber EMG (not done by all neurologists)
CT/MRI of thymus (thymoma)
PFT’s
What is the treatment of MG?
Thymectomy (maybe a cure in up to half of patients)
Anti-cholinesterase medications (Pyridostigmine)
IVIG
What is a common complication of MG?
Weakness of respiratory muscles (require intubation, IV immunoglobulin, plasmapheresis)
What can trigger a MG crisis?
Infection
Stress
Surgery
Adverse reaction to medication
What is the prognosis of MG?
With treatment, most severe weakness will improve and patients can live full lives
