Brain and Behavior: Demyelinating Diseases

Women are affected by MS … as often as men

twice

Those who move from a low-risk to a high-risk region … adopt the risk of MS associated with their new locale, perhaps a latent viral infection in childhood plays a role

younger than 15

There is increased incidence of MS in association with particular …

human leukocyte antigen alleles

…. separated in space and time are characteristic of MS

multiple white matter lesions

… can be produced by an MS lesion

any neurologic symptoms

In later stages of MS there maybe … (2) abnormalities

cognitive, behavioral

…: painful unilateral loss of visual acuity esp. with movement

optic neuritis

Visual affects of optic neuritis: … (2)

blurred vision, decreased color discrimination

In optic neuritis the optic disc may be … with …

swollen, papilledema

Previous optic neuritis is suggested by … (2)

optic disc pallor, atrophy

…: inflammatory demyelination in the spinal cord

transverse myelitis

Transverse myelitis is usually a partial lesion and there may be … below the lesion

unilateral or bilateral weakness or sensory loss

Transverse myelitis may lead to loss of … (2) function

bladder, bowel

Transverse myelitis may lead to .reflex exaggeration …, Babinski signs may be present

below the lesion

In transverse myelitis patient may report a band of … around the torso at the …

pain/tingling, level of lesion

Transverse myelitis causes a loss of … reflexes

abdominal

…: a tingling, electric sensation down the spine when the patient flexes the neck

lhermittes sign

…: worsening of symptoms and signs in the heat

Uhthoff’s phenomenon

To diagnose MS first you need to show dissemination in space by … in at least 2 of the 4 CNS regions (periventricular, juxtacortical/cortical, infratentorial, spinal cord)

at least 1 T2 lesion

To diagnose MS you then need to prove dissemination is time by … (3)

two clinical attacks at different times (in at least 1 year), MRI showing old and new lesions, CSF oligoclonal bands

Macroscopic findings of MS: … (2)

multifocal demyelinating plaques, spinal cord atrophy

Microscopic findings of MS: … (2)

acquired loss of myelin, preservation of axons

MS lesions typically appear in: … (3)

periventricular white matter, corpus callosum, cerebellar peduncles

MS CSF findings: … (3)

igG antibodies (oligoclonal), pleocytosis, elevated proteins

… document evidence of old optic neuritis with increased latency of the P100 wave on the affected side

visual evoked potentials

…: first clinical episode with features suggestive of MS, usually in young adults, usually recover

Clinically isolated syndrome

Clinically isolated syndrome affects: … (3)

optic nerves, brainstem/cerebellum, spinal cord

Risk factors doe MS: … (2)

clinically silent MRI lesions, CSF oligoclonal bands

Uncertain risk factors for clinically isolated syndrome: … (5)

vitamin D deficiency, Epstein-Barr virus infection, smoking, HLA gene, immunological abnormalities

Potential courses of MS post CIS: … (2)

1/3 benign course, ½ secondary progressive MS

Disease-modifying treatments delay the development from … → …

CIS, MS

…: minimal to no accumulated disability, few attacks, usually return to normal between attacks

Benign multiple sclerosis

…: no new disability between attacks, most patients begin with this course

relapsing-remitting multiple sclerosis

…: evolving from relapsing-remitting disease, these is a progressive disability with or without attacks

secondary progressive multiple sclerosis

…: steady increase in disability without attacks

primary progressive multiple sclerosis

Better prognosis if: … (5)

young age onset, female, rapid remission of initial symptom, mild relapse, sensory symptoms

three treatment modalities of MS: … (3)

acute relapse, chromic for disease process, symptomatic

Acute therapies for relapse: … (2)

corticosteroids, IV methylprednisolone

Chronic therapies to treat the underlying disease process: … to decrease the rate of relapse, the burden of lesions , and the rate of accumulated disability

immune-modulating agents

Chronic therapies are used in: … (2)

relapsing-remitting MS, secondary progressive disease

Spasticity can be managed with: … (4)

baclofen, diazepam, tizanidine, dalfampridine

Bladder dysfunction can be managed with: … (2)

anti-cholinergic agents, intermittent self-catheterization

…: monophasic illness leading to areas of demyelination within the CNS, commonly follow a viral infection or vaccination, multiple patchy lesions, present bilaterally all acute, may present with behavioral and cognitive abnormalities

Acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis CSF: … (2)

lymphocytic pleocytosis, elevated proteins

Acute disseminated encephalomyelitis is treated with …

IV corticosteroids

…: the development of transverse myelitis and optic neuritis, no demyelination of the brain, lesions which extend over multiple segments, severe pain

Neuromyelitis Optica

Neuromyelitis CSF: …

pleocytosis

Neuromyelitis optica treatment: … (3)

steroids, chemotherapeutic agents, plasmapheresis

Neuromyelitis optic has a poor prognosis sue to development of: … (2)

paralysis, blindness

…: cognitive decline, focal cortical dysfunction, and cerebellar abnormalities, seen in patients with AIDS, leukemia, lymphoma, immunosuppressant medications

progressive multifocal leukoencephalopathy

Progressive multifocal leukoencephalopathy is caused by … _. demyelination by infection of …

JC virus, oligodendrocytes

MRI of progressive multifocal leukoencephalopathy shows multiple focai of white matter abnormalities especially …

posterior regions

Progressive multifocal leukoencephalopathy CSF: …

normal

Progressive multifocal leukoencephalopathy treatment: …

ineffective

…: develops in patients with rapidly developing hypertension and eclampsia, immunosuppressants used in organ transplants

posterior reversible encephalopathy syndrome

Posterior reversible encephalopathy syndrome is characterized by: … (2)

acute confusion, cortical blindness with preserved pupillary reactivity

Posterior reversible encephalopathy treatment: …

addressing underlying cause

…: X-linked disorder, present in males, results in the accumulation of very long-chain fatty acids in body tissues and the posterior brain causing myelin damage

adrenoleukodystrophy

Adrenoleukodystrophy presents as: …(4)

seizures, hyperactivity, language problems, cognitive dysfunction

Adrenoleukodystrophy treatment: … (3)

lorenzos oil, stem cell transplant, adrenal insufficiency

…: rare demyelinating disorder, occurs often in alcoholic patients, too-radip correction of hyponatremia , affects pons > basal ganglia > thalamus > subcortical white matter

central pontine myelinolysis

Clinical presentation of central pontine myelinolysis: … (5)

acute confusional state, spastic quads, locked-in syndrome, dysarthria, dysphagia