8/2/2019 Neurological Disorders Janelle M James
1/12
Neurological Diseases andDisorders
Janelle M. James
January 23, 2012
8/2/2019 Neurological Disorders Janelle M James
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
8/2/2019 Neurological Disorders Janelle M James
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
8/2/2019 Neurological Disorders Janelle M James
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
8/2/2019 Neurological Disorders Janelle M James
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
8/2/2019 Neurological Disorders Janelle M James
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)
8/2/2019 Neurological Disorders Janelle M James
7/12
Schilder's Disease
Signs
vomiting
dementia
aphasia
tremors
muscle weakness
balance instability
Symptoms
personality changes
incontinence
headaches
vision and speech
impairment
8/2/2019 Neurological Disorders Janelle M James
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
8/2/2019 Neurological Disorders Janelle M James
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
8/2/2019 Neurological Disorders Janelle M James
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
8/2/2019 Neurological Disorders Janelle M James
11/12
Dyssynergia Cerebellaris Myoclonica
Signs
myoclonus
seizures
epilepsy
ataxia
unsteady gait
adiadochokinesia
Symptoms
cognitive impairment
8/2/2019 Neurological Disorders Janelle M James
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?