neurological disorders janelle m james

8/2/2019 Neurological Disorders Janelle M James

1/12

Neurological Diseases andDisorders

Janelle M. James

January 23, 2012


2/12

Klver-Bucy Syndrome

Rare behavioral impairment

associated with damage to BOTH of the anterior temporal lobes of

the brain

May be caused/associated with herpes encephalitis or trauma, both

of which can result in damage to the brain

It is not life-threatening, but with treatment, symptoms may

decline, although there is currently no cure


3/12

Klver-Bucy Syndrome

Signs

hyperorality

hypersexuality

seizures

dementia

Symptoms

visual agnosia (inability to

visually recognize objects)

distractability

memory loss

docility

dietary changes and/or

hyperphagia


4/12

Sturge-Weber Syndrome

neurological disorder

indicated at birth

port-wine stain birthmark on the forehead and upper eyelid of one

side of the face

birthmark can vary in color from light pink to deep purple and is

caused by an overabundance of capillaries around the trigeminal

nerve just beneath the surface of the face


5/12

Sturge-Weber Syndrome

Signs

port wine stain (birthmark)

focal deficits

hemiparesis

hemianopsia

glaucoma

Symptoms

seizures that begin in infancy

and may worsen with age

learning disabilities

blindness

headaches


6/12

Schilder's Disease

a rare progressive demyelinating disorder which usually begins in

childhood

variant of multiple sclerosis

course and prognosis of Schilder's disease are unpredictable

not the same as Addison-Schilder disease

(adrenoleukodystrophy)


7/12

Schilder's Disease

Signs

vomiting

dementia

aphasia

tremors

muscle weakness

balance instability

Symptoms

personality changes

incontinence

headaches

vision and speech

impairment


8/12

Alternating Hemiplegia

rare neurological disorder

develops during childhood

some children with a family history of the disorder have mutations

in the genes CACNA1A, SCN1A, and ATP1A2


9/12

Alternating Hemiplegia

Signs

recurrent episodes of

paralysis that involve one or

both sides of the body,

multiple limbs, or a single

limb

seizures

walking and balance

problems

Symptoms

stiffening or "dance-like"

movements of a limb

can have normal or delayed

development


10/12

Dyssynergia Cerebellaris Myoclonica

formerly called "Ramsay Hunt syndrome type I"

degenerative neurological disorder

may be inherited in an autosomal recessive pattern or may occur

in someone who has no history of the disorder in his or her family

symptoms worsen over time

onset usually occurs in adulthood

caused by the impairment of a regulatory mechanism between

cerebellar and brainstem nuclei


11/12

Dyssynergia Cerebellaris Myoclonica

Signs

myoclonus

seizures

epilepsy

ataxia

unsteady gait

adiadochokinesia

Symptoms

cognitive impairment


12/12

QUIZ

What is Klver-Bucy Syndrome caused by? (What is it associated

with?)

What was Dyssenergia Cerebellaris Myoclonica formerly known

as?

Which body parts do alternating hemiplegia affect?

Name the most prominent "feature" of Sturge-Weber syndrome.

What kind of disorder is Schilder's disease?

neurological disorders janelle m james

Documents