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Differential
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abdominal distention
abdominal reflex, absent
abducens nerve paralysis
acanthocytosis
acetazolamide
acetylcholine
achilles tendon, enlarged
acoustic nerve
acoustic neurinoma, bilateral
acquired immunodeficiency syndrome
acquired immunodeficiency syndrome myelopathy
acromicria
Addison's disease
adrenoleukodystrophy
adrenoleukodystrophy, carrier
adrenomyeloneuropathy
adult-onset leukodystrophy, with neuroaxonal spheroids
advances in neurology
adverse drug reaction
aggression
agitation
akathisia
akinetic mute
alcohol
alcohol intolerance
alcohol, neurologic complications with
alcoholism
Alexanders disease
Alexanders disease, adult onset
algorithm
alkylating agents
alopecia
alpha-fetoprotein
altered states of consciousness
alternating hemiplegia
alternating hemiplegia of childhood
alternating rapid movement
alternating rapid movement, impaired
Alzheimer's disease
aminoacidopathies
aminoacidurias
amniocentesis
amyloid plaques
amyotrophic lateral sclerosis
anemia
Angelman syndrome
aniridia
ankle reflex, absent
anosmia
antibodies to voltage-gated calcium channels
anticholinesterase
anticonvulsants
anticonvulsants, effectiveness
antimetabolite
antioxidant
anxiety
aphasia
apnea
apnea, primary central
apraxia
apraxia of eye movements
areflexia
Arnold Chiari malformation
arrhythmia, cardiac
arylsulfatase A
asparginase
aspiration
astrogliopathy
asymptomatic
ataxia
ataxia telangiectasia
ataxia, cerebellar
ataxia, congenital
ataxia, hereditary
ataxia, paroxysmal
ataxia, progressive
ataxia, sensory
ataxia, truncal
ataxic gait
ataxic-dystonia syndromes
ataxin
ataxin-2
atherosclerosis, premature
athetosis
atonic bladder
ATP1A3 gene
attention
attention deficit disorder with hyperactivity
attention span
atypical
auditory evoked brainstem potentials
autism
autonomic dysfunction
autosomal dominant leukodystrophy
autosomal rcessive spastic ataxia of Charlevoix-Saguenay
axonal spheroid
B 12 deficiency
Babinski sign
baclofen
basal ganglia, calcification of
basal ganglia, degeneration
basal ganglia, lesion of
basal ganglia, lesion, bilateral
Bassen-Kornzweig syndrome
behavior, combative
behavioral disorder
biologic markers
biotin deficiency
biotinidase deficiency
bladder dysfunction
blindness
blood dyscrasias, neurologic findings with
bone marrow biopsy
brachycephaly
bradykinesia
brain atrophy
brain biopsy
brainstem, atrophy
brainstem, lesion of
brainstem, neoplasms of
bulbar palsy
cachexia
cafe au lait spots
CAG repeats
calcification, intracranial
calcium channel dysfunction
cane
carcinoembryonic antigen
carcinoma
cardiomyopathy
CAT scan
CAT scan, abnormal
CAT scan, emission
CAT scan, emission, abnormal
catalepsy
cataplexy
cataracts
cataracts, congenital
caudate nucleus, lesion of, bilateral
celiac disease, adult
central core disease
cerebellar ataxia, autosomal recessive
cerebellar ataxia, children
cerebellar ataxia, children, differential diagnosis of
cerebellar ataxia, hereditary
cerebellar ataxia, neuropathy and vestibular areflexia syndrome
cerebellar ataxia, primary
cerebellar atrophy, primary
cerebellar atrophy, secondary
cerebellar degeneration
cerebellar hemangioma
cerebellar hypoplasia
cerebellar lesion
cerebellar plaques, amyloid
cerebellar vermis
cerebellum
cerebellum, disease of
cerebellum, neoplasms of
cerebral cortex
cerebral cortical atrophy
cerebral edema
cerebral palsy
cerebrospinal fluid, elevated protein of
cerebrospinal fluid, lactic acid concentration
cerebrospinal fluid, pressure low
cerebrospinal fluid, proteincytologic dissociation
cerebrospinal fluid, xanthochromia of
cerebrotendinous xanthomatosis
cerebrovascular accident
cerebrovascular accident, mimics
cerebrovascular accident, young adult
channelopathy
Charcot-Marie-Tooth
chemotherapy, CNS treatment and complications with
chenodeoxycholic acid
chewing movements
children
chloride channel dysfunction
choking
cholestanol
chorea
chorea, familial
choreoathetosis
chromosomal abnormality
chromosome 11
chromosome 12
chromosome 14
chromosome 15
chromosome 18
chromosome 19
chromosome 2
chromosome 21
chromosome 6
chromosome 9
chronic progressive external ophthalmoplegia
ciguatera poisoning
cirrhosis
cisterna magna, enlarged
Clinical Pathologic Conference(C.P.C.)
clonus
clubfoot as related to neurologic disease
Cockayne's syndrome
cognition
cognition, slowed
cogwheel rigidty
Collier's sign
color vision, impaired
coma
coma, episodic
comorbidities
cone-rod dystrophy
confabulation
congestive heart failure
conjunctival biopsy
conjunctivitis
consanguinity
contractures, joint
controversies in neurology
conus medullaris, lesion of
copper metabolism, abnormal
cornea, abnormal
corpus callosum
corpus callosum, atrophy of
corpus callosum, lesion of
corpus callosum, thinning
cortical blindness
cough
cranial nerve palsies
cranial neuropathy, multiple
cranio-cervical junction
creatine phosphokinase(CPK)elevated
Creutzfeldt-Jakob disease, genetic
cry, abnormal
cry, weak
Cuba
cultured skin fibroblasts
cyst
cyst, peritumoral
cyst, neoplastic cerebellum
deafmute
deafness
deep gray nuclei
deep tendon reflexes
degenerative diseases of CNS
delay in diagnosis
delayed dentition
dementia
dementia, childhood
dementia, familial
dementia, frontotemporal
dementia, presenile
dementia, rapidly progressive
dementia, subcortical
dementia, transmissible
demyelinating disease
dentate nuclei, lesion of
dentatorubral-pallidoluysian atrophy
depression
dermatoglyphics
developmental disability
developmental milestones
developmental milestones, loss of
developmental retardation
dexterity, impaired
diabetes insipidus
diabetes mellitus
diabetes mellitus, chemical
diabetes mellitus, neurologic manifestations of
diagnostic criteria
diarrhea
differential diagnosis
difficulty climbing stairs
dilantin
diplopia
disability, neurological
disease modifying agents
distal muscle atrophy
dizziness
down-beat nystagmus
down-beat nystagmus, primary position of gaze
Dravet syndrome
drooling
drug abuse, inhalation
dwarfism
dysarthria
dysdiadochokinesia
dysmetria
dysmorphic
dysphagia
dyspraxia
dyssynergia cerebellaris myoclonica
dystonia
EAST syndrome
eating disorder
electrical sensation
electrocardiogram, abnormal
electroencephalogram
electroencephalogram, abnormalities of
electromyogram
electron microscopy
electroretinograph
encephalopathy
encephalopathy, progressive
enzyme, defect
epicanthal folds
epidemiology of neurology
episodic disorders
episodic neurologic deficits
erectile dysfunction
ethics in neurology
evoked potentials
excitotoxin
executive dysfunction
exercise
exercise intolerance
exome sequencing
extraocular muscle lesion
eye movement, disorders of
facial appearance, abnormal
facial expression abnormality
facial nerve palsy, bilateral
facial pain
failure to thrive
falling
false negative
familial
familial hemiplegic migraine
familial periodic ataxia
family planning
fasciculation
fatal familial insomnia
fatigue
feeding disorder
fever
finger nose finger test
fingerprint bodies
fish
fluorescene in situ hybridization
fluorouracil
flush syndrome
foam cells
foot deformity
foot drop
foot numbness
fornix, lesion of
Fragile-X associated tremor/ataxia-syndrome
fragile-X syndrome
fragile-X syndrome, carrier
frataxin
Friedreich's ataxia
Friedreich's ataxia, late onset
frontotemporal dementia, behavioral variant
fundus, abnormality of
gadolinium
gait disorder
gait, apraxic
gait, spastic
galactosemia
gargoylism
gastrointestinal disease, neurologic complications
gastrointestinal motility
gastroparesis
gaze palsy
gaze palsy, supranuclear
gaze palsy, vertical
gender
gene
gene mutation
genetic counselling
genetic diagnosis, prenatal
genetic linkage
genetic neurologic disorders
genetic screening
genetic testing
genu of corpus callosum
Gerstmann-Straussler-Scheinker disease
GFAP gene
Gillespie syndrome
glaucoma
gliosis
globus pallidus, lesion of
globus pallidus, lesion of, bilateral
glucose tolerance test, abnormal
GLUT1 deficiency syndrome
glutamate dehydrogenase deficiency
glutamic acid
granular osmiphilic material
gray hair
growth hormone deficiency
growth retardation
Hallervorden Spatz disease
Hallgren's syndrome
hallucination
hallucination, visual
hammertoes
hand clapping
hand flapping
hand wringing
handwriting
head injury
head nodding
headache
hearing loss
hearing problems in children
heart block
heel-knee-shin test
hemangioblastoma
hematuria, gross
hemiplegia
hemochromatosis
hemochromatosis, primary
hemophagocytic lymphohistiocytosis, cerebromeningeal
hemophagocytosis
hepatic encephalopathy
hepatic failure
hepatolenticular degeneration(Wilson's disease)
hepatolenticular degeneration(Wilson's disease), screening for
hepatolenticular degeneration, non-Wilsonian
hepatomegaly
hepatosplenomegaly
heralding manifestation
herniated disc, thoracic
HLA
hot cross bun sign
human T-lymphotropic virus type II(HTLV-II)
huntingtin
Huntington's chorea
Hurler's syndrome
hydrocephalus
hydroxytryptophan L-5(L-5 HTP)
hyperactivity
hyperammonemic encephalopathy
hyperhidrosis
hyperphagia
hyperreflexia
hypertension
hyperthermia
hypertonia
hypertriglyceridemia
hypocholesterolemia
hypodontia
hypofibrinogenemia
hypogonadism
hypogonadism, hypogonadotropic
hypokalemia
hypokalemic periodic paralysis
hypomyelination
hyponatremia
hypopigmentation of skin
hyporeflexia
hyposmia
hypotonia
hypotonia, infants
hypoxia
imbalance
imbalance, postural
immunodeficiency
immunologic disease
immunosuppression
impotence
impulsivity
inappropriate antidiuretic(A.D.H.)hormone, CNS involvement with
inattention
inborn errors of metabolism
inclusion bodies
inclusion bodies, eosinophilic cytoplasmic
inclusion bodies, eosinophilic intranuclear
inclusion bodies, intranuclear
inclusion bodies, ubiquitin
incontinence, fecal
incoordination
infection
infertility
insight, loss
insomnia
intellectual deficit
intellectual deterioration
intelligence quotient
internal capsule
internuclear ophthalmoplegia
internuclear ophthalmoplegia, bilateral
intestinal pseudoobstruction
intracranial pressure, increased
iris, abnormal
iron, brain
irritability
isoniazid
Jakob-Creutzfeldt disease
Jakob-Creutzfeldt disease, cerebellar variant
jaundice
jaw pain
joint hypermobility
karyotyping
Kayser-Fleischer ring
Kearns-Sayre syndrome
keratoconus
Korsakoff's psychosis
kyphoscoliosis, neurologic causes of
kyphosis
lactic acidemia
lathyrism
laughing, pathologic
Laurence-Moon-Bardet-Biedl syndrome
L-dopa
Leber's hereditary optic neuropathy
leg weakness, bilateral
Leigh's disease
Leigh's disease, adult variety
lenticular nucleus, lesion of, bilateral
leukemia
leukemia, neurologic findings assoc.with
leukodystrophy
leukoencephalopathy
leukoencephalopathy, adult onset, sporadic
leukoencephalopathy, differential diagnosis
leukoencephalopathy, hereditary diffuse
leukopenia
Lhermitte's sign
libido, decreased
life expectancy
lipid storage disorder of CNS
liver disease
liver function enzymes
liver transplantation
lymphadenopathy
lymphoma
lysosomal storage disease
macrocephaly
macrognathia
macular degeneration
malabsorption
malformation, CNS, congenital
malignant hyperpyrexia
maple syrup urine disease
marche a petits pas
Marinesco-Sjogren syndrome
masked facies
MELAS syndrome
memory, defect of recent
memory, impairment of
meningismus
meningitis, carcinomatous
meningoencephalopathy
mental retardation
MERRF syndrome
mesial temporal lobe
metabolic acidosis
metabolic disorder, primary
metachromatic leukodystrophy
metachromatic leukodystrophy, adult onset
metachromatic leukodystrophy, juvenile
methotrexate
methylhydrazine derivatives
Mexican
microcephaly
microdontia
microhemorrhage, intracerebral
midbrain, atrophy
middle cerebellar peduncle
middle cerebellar peduncle, lesion
middle cerebellar peduncle, lesion, bilateral
miglustat
migraine
migraine, hemiplegic
mimics
Minamata disease
Mini Mental Status Examination
misdiagnosis
mitochondrial disease
mitochondrial disease, pathogenesis
mitochondrial encephalomyopathy
MNGIE syndrome
molecular genetics
monoamine oxidase inhibitors
mood change
mortality
motor neuron disease
movement disorder
movement disorder, extrapyramidal
movement disorder, hyperkinetic
MRI
MRI, abnormal
MRI, CAT scan compared to
MRI, contrast enhanced
MRI, diffusion weighted
MRI, disappearing lesion on
MRI, FLAIR
MRI, high signal intensity of basal ganglia
MRI, negative
MRI, paramagnetic effect
MRI, punctate pattern
MRI, serial
MRI, spinal cord
MRI, T1 weighted high signal foci
MRS
mucopolysaccharidoses
multiple sclerosis
multiple sclerosis, misdiagnosis
multiple system atrophy
muscle atrophy, progressive
muscle biopsy
muscle cramp
muscle pain
muscle weakness
muscle weakness, proximal
muscular dystrophy
myasthenic syndrome
myelomalacia
myeloneuropathy
myelopathy
myelopathy, hepatic
myelopathy, vacuolar
myoclonic jerks
myoclonus
myoclonus, epilepsy
myokymia
myopathy
myopathy, mitochondrial
myopia
myotonia
myotonia congenita
myotonia dystrophica
N-acetylcysteine
Native Americans
nausea and vomiting
negative
neonatal screening, genetic neurologic disorders
neoplasm, metastatic to CNS
neoplasm, primary of CNS
neoplasm, primary of CNS-surgical treatment of
nerve biopsy
nerve conduction studies
nerve growth factor
neuritis
neuroaxonal dystrophy
neuroaxonal dystrophy, infantile
neuroaxonal dystrophy, juvenile
neuroaxonal leukodystrophy
neurocutaneous disease
neuroendocrinology
neurofibrillary degeneration
neurofibromatosis 1
neurogenic bladder
neurologic complications of, systemic cancer
neurologic complications of, systemic disease
neurologic disease
neurologic disease, diagnoses of
neurologic examination, focal
neurologic signs
neurologic testing
neuromyotonia
neuronal ceroid-lipofuscinosis
neuronal intranuclear inclusion disease
neuronopathy
neuronopathy, sensory
neuroophthalmology
neuropathology
neuropathology, brain
neuropathy
neuropathy, ataxic
neuropathy, demyelinating
neuropathy, hereditary peripheral
neuropathy, hypertrophic
neuropathy, onion bulb
neuropathy, peripheral
neuropathy, sensory
neurotoxin
neurotransmitter
neutropenia
next-generation sequencing
Niemann-Pick disease
night blindness
nitrogen mustard
nitrous oxide
NOTCH2NLC
nutritional deficiency
nystagmus
nystagmus, gaze-evoked
nystagmus, gaze-paretic
nystagmus, hereditary
nystagmus, monocular
nystagmus, periodic
nystagmus, primary position of gaze
nystagmus, rotary
nystagmus, upbeating-in primary position of gaze
nystagmus, vertical
obesity
ocular dysmetria
ocular motility, disorders of
ocular myopathy
oculodentodigital dysplasia
old age, neurology of
ophthalmoplegia
ophthalmoplegia, progressive external
opisthotonus
optic ataxia
optic atrophy
optic atrophy, hereditary
optic neuropathy
optical coherence tomography
optokinetic nystagmus, abnormal
orbit, tomograms of
orthostatic hypotension
oscillopsia
ovarian dysgenesis
ovarian insufficiency
pain
pain, abdominal
palatal myoclonus
pancytopenia
papilledema
paralysis
paramyotonia congenita
paranoia
paraparesis
paraparesis, familial spastic
paraparesis, familial spastic, classification
paraparesis, spastic
paraplegia
paresthesias
Parkinson disease
Parkinson disease, dystonia with
Parkinson disease, familial
Parkinsonism multiple-system atrophy
Parkinsonism syndrome
paroxysmal hemiplegia
paroxysmal neurologic deficits
PAS positive
PAS positive material in the brain
past pointing
pathology
patient information and support
Pelizaeus Merzbacher
penicillamine
periodic paralysis
peroxisomal disease
Perrault syndrome
personality change
pes cavus
pheochromocytoma
phlebotomy
photosensitivity, skin
physostigmine
phytanic acid
pigmentary retinopathy
pleocytosis of cerebrospinal fluid
pneumonia
poison, mercury
poison, neurologic problems with
POLG1 gene
POLR3B
polydactyly
polymerase chain reaction
polyneuropathy
pons, atrophy
pons, lesion of
pontocerebellar atrophy
portal caval shunt
positional head-hanging test
postural abnormality
potassium channel antibodies
potassium channel dysfunction
Prader-Labhart-Willi syndrome
precipitating factors
pregnancy, neurologic complications in
prenatal diagnosis by amniocentesis
pretectal syndrome
prevention of neurologic disorders
primary episodic ataxia
prion disease
prion protein gene
prisoners of war, neurologic complications in
procarbazine
progeria
prognathism
prognosis
progressive infantile poliodystrophy
progressive neurologic disorder
progressive pallidum atrophy
proprioception, abnormal
protein 14-3-3, cerebrospinal fluid
proteinuria
pseudobulbar palsy
pseudoretinitis pigmentosa
psychiatric disorder
psychiatric problems in neurologic disorders
psychological testing
psychological testing, neurologic problems
psychomotor retardation
psychosis
ptosis
ptosis, bilateral
pulmonary infection
Purkinje cell
pursuit eye movements, abnormal
putamen, lesion of
putamen, lesion of, bilateral
pyramidal
pyramidal tract
pyramidal tract dysfunction
pyruvate dehydrogenase deficiency
pyruvate metabolism, abnormality of
quadriparesis
quadriplegia
radiation hypersensitivity
radiation therapy, CNS treatment and complications with
radiation therapy, stereotactic
ragged-red fibers
rapid onset dystonia parkinsonism
rapidly progressing neurologic illness
rash
reading disorder, acquired
real-time quaking-induced conversion
recurrent
Refsum's disease
rehabilitation for neurologic disorders
release phenomena
remote effect of cancer on the nervous system
renal failure
respiratory failure
respiratory tract infection
retina, abnormal
retinal degeneration
retinal hemangioma
retinal lesion
retinitis pigmentosa
retinopathy
Rett's syndrome
reversible neurologic disorder
review article
RFC1 gene
rigidity
Riley-Day syndrome
Romberg's sign
Rosenthal fibers
saccadic eye movements, abnormal
salivation, excessive
schizophrenia
SCN1A gene
scoliosis
screaming
screening
seizure
seizure, children
seizure, familial
seizure, febrile
seizure, intractable
seizure, laughing as manifestation
seizure, neonatal
seizure, paradoxical
seizure, photosensitive
seizure, psychomotor-temporal lobe
seizure, tonic-clonic
seizure, treatment of
self-mutilation
semialdehyde dehydrogenase deficiency
sensorineural hearing loss
sensory ganglia
sensory ganglia, abnormal
sensory loss
sensory loss, leg
serologic testing
short stature
Shy-Drager syndrome
sinemet
skin, biopsy
skin, darkening of
skin, lesions in neurologic disorders
sleep apnea
sleep pathology and physiology
slurred speech
smiling
sodium channel dysfunction
somatosensory evoked potentials
spastic ataxia
spastic paraplegia, type 7
spasticity
speech disorder
speech disorder, childhood
speech disorder, non aphasic
speech, delayed development of
speech, loss of
Spielmeyer Vogt syndrome
spinal cord, lesion of
spinal cord, neoplasm
spinal cord, pathologic exam of
spinal muscular atrophy
spinocerebellar ataxia
spinocerebellar ataxia type 1
spinocerebellar ataxia type 10
spinocerebellar ataxia type 12
spinocerebellar ataxia type 14
spinocerebellar ataxia type 17
spinocerebellar ataxia type 2
spinocerebellar ataxia type 28
spinocerebellar ataxia type 3/Machado Joseph disease
spinocerebellar ataxia type 5
spinocerebellar ataxia type 6
spinocerebellar ataxia type 7
spinocerebellar ataxia type 8
spinocerebellar degeneration
spinopontine atrophy, dominant
splenium of corpus callosum
splenomegaly
spongy degeneration of brain
staggering
startle myoclonus
status epilepticus
steatorrhea
stereotaxic surgery
stereotyped behavior
stereotypy
steroid therapy, CNS treatment and complications with
storage disease of CNS
strabismus
stress, emotional
striatonigral degeneration
striatonigral degeneration, infantile
striopallidodentate calcifications, familial idiopathic
strokelike episodes
stuttering
subcortical U fibers
suck, poor
sudden death
suicide
superior cerebellar peduncle
symmetric brain lesions
syncope
syndactyly
syphilis, neurologic complications with
systemic illness
tachycardia
tandem gait, ataxic
tantrum
tauopathy
teeth, abnormal
teeth, number of in infants
teeth, wide-spaced
telangiectases
temper tantrums
temporal lobe, lesion
term infant
testicular enlargement
thalamus, lesion of
thalamus, lesion of-bilateral
thrombocytopenia
tinnitus
titubation
tongue, protrusion of
topiramate
toxins, nervous system
transient neurologic deficit
trazodone
treatment of neurologic disorder
tremor
tremor, cerebellar
tremor, intention
tremulousness
trinucleotide repeats
trisomes
trisomy 9p
tubulopathy
undiagnosed
Unverricht-Lundborg disease
upgaze, paralysis of
urea-cycle enzymopathies
urinary frequency
urinary incontinence
urinary urgency
urine test for metabolic disorders
Usher's syndrome
vertigo
vertigo, episodic
vertigo, treatment of
vestibular areflexia
vestibulopathy
vibratory sensation, abnormal
vinblastine
vincristine neurotoxicity
viral isolation
vision, failure of in childhood
visual acuity, decreased
visual field defect
visual fields, constricted
visual loss
visual loss, progressive
visual loss, slow
visuospatial disturbance
vitamin A
vitamin deficiency
vitamin E
vitamin E deficiency
vitamin K
vitamin supplementation
vitiligo
vocal cord paralysis
voice, abnormality of
Von Hippel Lindau
walking frame
walking, difficulty with
walking, difficulty with in dark
war
weakness
weakness, generalized
weakness, progressive
weaning from respirator, failure to
web sites
weight loss
Western immunoblot test
wheelchair
white matter disease
whole genome sequencing
wide based gait
Wolfram syndrome
work loss
workup
wrist drop
writing
xanthoma, tendon
xeroderma pigmentosa
X-linked bulbospinal neuronopathy
x-linked intellectual deficit
x-linked mental retardation
Showing articles 50 to 100 of 3337 << Previous Next >>

Degenerative Diseases of the Nervous System, Olivopontocerebellar Atrophy
Adams & Victors Principles of Neurology, Chp 39, pg 1106, Ropper, A.H.,et al, 2014

Degenerative Diseases of the Nervous System, Machado-Joseph-Azorean Disease
Adams & Victors Principles of Neurology, Chp 39, pg 1107, Ropper, A.H.,et al, 2014

Degenerative Diseases of the Nervous System, Multiple System Atrophy
Adams & Victors Principles of Neurology, Chp 39, pg 1095, Ropper, A.H.,et al, 2014

Psychosis in an Adolescent Girl: A Common Manifestation in Niemann-Pick Type C Disease
Child Adolesc Pych Ment Health 8:20, Wouters,S.,et al, 2014

Clinical Reasoning: A Woman with Rapidly Progressive Apraxia
Neurol 80:e162-e165, Pressman, P.,et al, 2013

The Autosomal Recessive Cerebellar Ataxias
NEJM 366:636-646, Anheim,M.,et al, 2012

GFAP Mutations, Age at Onset, and Clinical Subtypes in Alexander Disease
Neurol 77:1287-1294, Prust, M.,et al, 2011

Clinicopathologic Conference, Kufs Disease (Autosomal Dominant) Parry Type Neuronal Ceroid Lypofuscinosis
NEJM 364:1062-1074, Case 8-2011, 2011

Clinical and Genetic Spectrum of Mitochondrial Neurogastrointestinal Encephalomyopathy
Brain 134:3326-3332, Garone, C.,et al, 2011

The Hot Cross Bun Sign in the Patients with Spinocerebellar Ataxia
Eur J Neurol 16:513-516, Lee, Y.-C.,et al, 2009

Epilepsy, Ataxia, Sensorineural Deafness, Tubulopathy, and KCNJ10 Mutations
NEJM 360:1960-1970, Bockenhauer,D.,et al, 2009

Clinical Spectrum of Ataxia-Telangiectasia in Adulthood
Neurol 73:430-437, Verhagen,M.M.M.,et al, 2009

Fragile X-Associated Tremor/Ataxia Syndrome: An Aging Face of the Fragile X Gene
Arch Neurol 65:19-25, Amiri,K.,et al, 2008

A 23-Year-Old Man With Seizures and Visual Deficit
Neurol 70:73-78, Boustany,R.-M.,et al, 2008

Downbeat Nystagmus: Aetiology and Comorbidity in 117 Patients
JNNP 79:672-677, Wagner,J.N.,et al, 2008

Angelman Syndrome Revisited
Neurologist 13:305-312, Paprocka,J.,et al, 2007

Primary Episodic Ataxias:Diagnosis, Pathogenesis and Treatment
Brain 130:2484-2493, Jen, J.C.,et al, 2007

Clinical Spectrum of Mutations in SCN1A Gene: Severe Myoclonic Epilepsy in Infancy and Related Epilepsies
Epilepsy Res 70S:S223-S230, Fujiwara,T., 2006

Epilepsy Syndromes in Infancy
Pediatr Neurol 34:253-263, Korff,C.M. &Nordii,D.R.,Jr., 2006

Late-Onset Metachromatic Leukodystrophy: Genotype Strongly Influences Phenotype
Neurol 67:859-863, Rauschka,H.,et al, 2006

Fragile X Premutation With Atypical Symptoms at Onset
Arch Neurol 63:1135-1138, Cellini,E.,et al, 2006

Phenotypic Spectrum Associated with Mutations of the Mitochondrial Polymerase y Gene
Brain 129:1674-1684, Horvath, R.,et al, 2006

Mitochondrial DNA Polymerase-y and Human Disease
Hum Mol Genet 15:R244-R252, Hudson, G.,et al, 2006

Autonomic Dysfunction in Machado-Joseph Disease
Arch Neurol 62:630-636, Yeh,T-H.,et al, 2005

The Clinical and Genetic Spectrum of Spinocerebellar Ataxia 14
Neurol 64:1258-1260, Chen,D-H.,et al, 2005

The Parkinsonian Phenotype of Spinocerebellar Ataxia Type 2
Arch Neurol 61:35-38, Lu,C.,et al, 2004

Impact of Presymptomatic Genetic Testing for Hereditary Ataxia and Neuromuscular Disorders
Arch Neurol 61:875-880, Smith,C.O.,et al, 2004

Clinical Spectrum of Episodic Ataxia Type 2
Neurol 62:17-22, Jen,J.,et al, 2004

Clinical Features and Neuropathology of Autosomal Dominant Spinocerebellar Ataxia (SCA17)
Ann Neurol 43:367-375, Rolfs,A.,et al, 2003

Clinical Spectrum of Succinic Semialdehyde Dehydrogenase Deficiency
Neurol 60:1413-1417, Pearl,P.L.,et al, 2003

Clinical Features and ATTCT Repeat Expansion in Spinocerebellar Ataxia Type 10
Arch Neurol 59:1285-1290, Grewal,R.P.,et al, 2002

Spinocerebellar Ataxia Type 10 is Rare in Populations Other Than Mexicans
Neurol 58:983-984, Matsuura,T.,et al, 2002

Fragile X Premutation Carriers: Characteristic MR Imaging Findings of Adult Male Patients with Progressive Cerebellar and Cognitive Dysfunction.
AJNR 23:1757-1766, Brunberg,J.A.,et al, 2002

Spinocerebellar Ataxia Type 2 Presenting as Familial Levodopa-Responsive Parkinsonism
Ann Neurol 50:812-815, Shan,D.,et al, 2001

Recessive Ataxia With Ocular Apraxia
Arch Neurol 58:201-205,173, Barbot,C.,et al, 2001

SCA-12: Tremor with Cerebellar and Cortical Atrophy is Associated with a CAG Repeat Expansion
Neruol 56:299-303,287, O'Hearn,E.,et al, 2001

Genetic Testing in Spinocerebellar Ataxias
Arch Neurol 58:191-195, Tan,E. &Ashizawa,T., 2001

Gluten Sensitivity in Sporadic and Hereditary Cerebellar Ataxia
Ann Neurol 49:540-543, Bushara,K.O.,et al, 2001

Evolution of Sporadic Olivopontocerebellar Atrophy Into Multiple System Atrophy
Neurol 55:527-532, Gilman,S. et al, 2000

Spinocerebellar Ataxia Type 8
Neurol 55:649-657, Day,J.W. et al, 2000

Niemann-Pick Disease Type C: Two Cases and an Update
Movement Disorders 15:1199-1203, Uc,E.Y.,et al, 2000

Inborn Errors of Metabolism as a Cause of Neurological Disease in Adults: An Approach to Investigation
JNNP 69: 5-12, Gray,R.G.F. et al, 2000

N-Acetylcysteine Therapy for Unverricht-Lundborg Disease
Neurol 52:426-427, Selwa,L.M., 1999

Molecular Basis of the Neurodegenerative Disorders
NEJM 340:1970-1980, Martin,J.B., 1999

Clinical and MRI Findings in Spinocerebellar Ataxia Type 5
Neurol 53:1355-1357, Stevanin,G.,et al, 1999

Prader-Willi and Angelman Syndromes
Medicine 77:140-151, Cassidy,S.B.&Schwartz,S., 1998

Clinical Usefulness of Magnetic Resonance Imaging in Multiple System Atrophy
JNNP 65:65-71, Schrag,A.,et al, 1998

CAG Repeat Number Correlates with the Rate of Brainstem and Cerebellar Atrophy in Machado-Joseph Disease
Neurol 51:882-884, Abe,Y.,et al, 1998

Incidence of Dominant Spinocerebellar and Friedreich Triplet Repeats Among 361 Ataxic Families
Neurol 51:1666-1671, Moseley,M.L.,et al, 1998

Ion Channels and Neurological Disease:DNA Based Diagnosis is Now Possible,and Ion Channels May be Important in Common Paroxysmal Disorders
JNNP 65:427-431, Hanna,M.G.,et al, 1998



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