Neudle.com: dementia hereditary

Differential
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abducens nerve paralysis

abulia

aceruloplasminemia

achilles tendon, enlarged

acoustic nerve

acquired immunodeficiency syndrome

acquired immunodeficiency syndrome myelopathy

acromicria

activities of daily living

activities of daily living scale

Addison's disease

adrenoleukodystrophy

adrenoleukodystrophy, adult onset

adrenoleukodystrophy, carrier

adrenomyeloneuropathy

adult polyglucosan body disease

adult-onset leukodystrophy, with neuroaxonal spheroids

advances in neurology

adverse drug reaction

affect, inappropriate

alcohol, blood level of

alcohol, neurologic complications with

alcoholic blackout

alcoholic coma

alcoholic dementia

alcoholic polyneuropathy

alcoholic withdrawal states, DT's, convulsions, etc.

altered states of consciousness

alternating hemiplegia

alternating hemiplegia of childhood

alternating rapid movement

alternating rapid movement, impaired

aluminum

Alzheimer's disease

Alzheimer's disease, age of onset

Alzheimer's disease, conjugal

Alzheimer's disease, diagnosis of

Alzheimer's disease, early onset

Alzheimer's disease, familial

Alzheimer's disease, familial, late onset

Alzheimer's disease, heterogeneity of

Alzheimer's disease, incidence

Alzheimer's disease, misdiagnosis

Alzheimer's disease, pathogenesis

Alzheimer's disease, preclinical

Alzheimer's disease, risk factors in

Alzheimer's disease, treatment of

aminoacidopathies

ammonia

amnesia

amyloid angiopathy, cerebral

amyloid angiopathy, cerebral, Dutch type

amyloid angiopathy, hereditary cystatin C

amyloid beta protein

amyloid imaging

amyloid plaques

amyotrophic lateral sclerosis

amyotrophic lateral sclerosis, diagnosis of

amyotrophic lateral sclerosis, differential diagnosis

amyotrophic lateral sclerosis, epidemiology of

amyotrophic lateral sclerosis, etiology of

amyotrophic lateral sclerosis, familial

amyotrophic lateral sclerosis, guamian type of

amyotrophic lateral sclerosis, Parkinson-dementia-complex

amyotrophic lateral sclerosis, prognosis

amyotrophic lateral sclerosis, treatment of

anatomy of

anemia

anemia, megaloblastic

anesthesia, general

aneuploidy

aneurysm

aneurysm, intracranial

angiography, cerebral

anticholinergic drugs

anticholinesterase

anticonvulsants, selection of

aphasia, progressive, primary

apraxia, constructional

areflexia

arm weakness

Arnold Chiari malformation

asterixis, unilateral

asymptomatic

ataxia

ataxia telangiectasia

atherosclerosis, premature

auditory and vestibular pathways

auditory evoked brainstem potentials

autism

autonomic dysfunction

basal ganglia, calcification of

basal ganglia, degeneration

basal ganglia, infarction

basal ganglia, lesion of

basal ganglia, lesion, bilateral

basilar artery occlusion

behavior, combative

behavioral disorder

Behcet's syndrome

benign essential tremor

blood dyscrasias, neurologic findings with

blood transfusion

bone marrow transplantation

bone pain

botulinum toxin

bovine spongiform encephalopathy

brain transplantation

calcification, intracranial

camptocormia

Canavan's disease

carbon monoxide poisoning

carcinoma of pancreas

cardiomyopathy

carotid artery disease

carotid artery occlusion, bilateral

CAT scan

CAT scan, abnormal

CAT scan, contrast enhanced

CAT scan, emission

CAT scan, emission, abnormal

CAT scan, false negative

catalepsy

cataplexy

cataracts

caudate nucleus, atrophy

caudate nucleus, lesion of

caudate nucleus, lesion of, bilateral

cell fusion test

central nervous system, infection of

central pontine myelinolysis

cerebellar ataxia, children

cerebellar ataxia, children, differential diagnosis of

cerebellar ataxia, hereditary

cerebellar atrophy, primary

cerebellar atrophy, secondary

cerebellar degeneration

cerebellar lesion

cerebellar plaques, amyloid

cerebellar vermis

cerebellum, disease of

cerebral autosomal dominate arteriopathy with subcortical infarction and leukoencephalopathy

cerebral autosomal recessive arteriopathy with subcortical infarction and leukoencephalopathy

cerebral cortex

cerebral cortical atrophy

cerebral edema

cerebral edema, vasogenic

cerebral glucose metabolism

cerebral infarction

cerebral infarction, subcortical

cerebral ischemia

cerebral vasculature, calcification

cerebral venous thrombosis

cerebral venous thrombosis, deep

cerebrospinal fluid

cerebrospinal fluid, abnormal

cerebrospinal fluid, amonia

cerebrospinal fluid, elevated protein of

cerebrospinal fluid, glutamine

cerebrospinal fluid, lactic acid concentration

cerebrospinal fluid, pressure low

cerebrospinal fluid, protein of

cerebrotendinous xanthomatosis

cerebrovascular accident

cerebrovascular accident, familial occurrence

cerebrovascular accident, infancy and childhood

cerebrovascular accident, mimics

cerebrovascular accident, multiple

cerebrovascular accident, non atherosclerotic cause of

cerebrovascular accident, nonarterial territory

cerebrovascular accident, recurrent

cerebrovascular accident, young adult

cerebrovascular disease

ceruloplasmin, serum

Charcot-Marie-Tooth

chemotherapy, CNS treatment and complications with

chenodeoxycholic acid

cherry red spot

cherry red spot-myoclonus syndrome

choline acetyltransferase

chromosomal abnormality

chronic progressive external ophthalmoplegia

cigarette smoking

cingulate gyrus

cingulate island sign

cirrhosis

clindamycin

Clinical Pathologic Conference(C.P.C.)

clonus

clubbing of fingers

coat-hanger pain

cobalamin C deficiency

coenzyme Q10 deficiency

color vision, impaired

coma

complications

compression neuropathy

copper metabolism, abnormal

corneal dystrophy

corneal transplant

corpus callosum

corpus callosum, atrophy of

corpus callosum, lesion of

corpus callosum, thinning

cortical blindness

cortical blindness, transient

cortical infarction

cost

cranial neuropathy, multiple

Creutzfeldt-Jakob disease, genetic

crying, pathologic

cultured skin fibroblasts

degenerative diseases of CNS

delay in diagnosis

delusion

dementia

dementia, age at onset

dementia, cerebrovascular disease causing

dementia, childhood

dementia, diagnostic evaluation of

dementia, differential diagnosis of

dementia, familial

dementia, frontal lobe type

dementia, frontotemporal

dementia, presenile

dementia, rapidly progressive

dementia, screening for

dementia, subcortical

dementia, thalamic

dementia, transmissible

demyelinating disease

dentate nuclei

dentate nuclei, lesion of

dentatorubral-pallidoluysian atrophy

depression

developmental milestones

developmental milestones, loss of

developmental retardation

differential diagnosis

difficulty climbing stairs

diplopia

disability rating scale, neurological

disease modifying agents

disinhibition-dementia-parkinsonism-amyotrophic complex

dyskinesia, drug induced

dyssynergia cerebellaris myoclonica

dystonia

dystonia, cervical

dystonia, focal

dystonia, post traumatic

dystonia, treatment of

ears of the Lynx MR sign

echolalia

edema, pedal

electroencephalogram

electroencephalogram, abnormalities of

electroencephalogram, pediatric patients

electroencephalogram, periodic complexes

electroencephalogram, video monitoring with

encephalitis, Japanese

encephalitis, viral

encephalomalacia

encephalopathy

encephalopathy, metabolic

encephalopathy, neonatal

encephalopathy, progressive

enzyme, defect

epidemiology of neurology

episodic neurologic deficits

executive dysfunction

exercise intolerance

exome sequencing

extrapyramidal

eye movement, disorders of

Fabry's disease

facial expression abnormality

facial nerve palsy, bilateral

familial hemiplegic migraine

fasciculation

fatal familial insomnia

fatigue

fatty acid, elevated plasma content

feeding disorder

ferritin, elevated

ferritinemia

fetal alcohol syndrome

fever

fibrillations

finger nose finger test

Fragile-X associated tremor/ataxia-syndrome

fragile-X syndrome

fragile-X syndrome, carrier

Friedreich's ataxia

frontal lobe, anatomy and physiology

frontal lobe, atrophy

frontal lobe, lesion of

frontal lobe, pathologic signs of

frontotemporal dementia, behavioral variant

fundus, abnormality of

Gaucher's disease, adult onset

gaze palsy

gaze palsy, supranuclear

genetic diagnosis, prenatal

genetic linkage

genetic neurologic disorders

genetic screening

genetic testing

genu of corpus callosum

Gerstmann-Straussler-Scheinker disease

Gilles de la Tourette syndrome

globus pallidus, lesion of

globus pallidus, lesion of, bilateral

glutamic acid

glutamine

glutaric acidemia

glycogen storage disease

gout

granular osmiphilic material

Hallervorden Spatz disease

Hallervorden Spatz disease, late onset

hallucination

hallucination, visual

hearing loss, bilateral

hearing problems in children

hemochromatosis, primary

hepatic encephalopathy

hepatic failure

hepatitis

hepatolenticular degeneration(Wilson's disease)

hepatolenticular degeneration(Wilson's disease), presymptomatic

hepatolenticular degeneration, non-Wilsonian

hepatomegaly

hepatosplenomegaly

heralding manifestation

herpes simplex encephalitis

herpes simplex encephalitis, differential diagnosis of

Huntington's chorea, genetic counselling

Huntington's chorea, late onset

Huntington's chorea, misdiagnosis of

Huntington's disease, children

hydrocephalus, exvacuo

hyperpigmentation of skin

hyperpyrexia, CNS disorder causing

hypotension, neurologic causes of

hypotension, systemic

hypothalamus, disturbance of

hypothermia

hypothermia, causes of

hypoxic encephalopathy

iatrogenic neurologic disorders

inappropriate antidiuretic(A.D.H.)hormone, CNS involvement with

inappropriate behavior

inattention

inborn errors of metabolism

inclusion bodies

inclusion bodies, eosinophilic cytoplasmic

inclusion bodies, eosinophilic intranuclear

inclusion bodies, intracytopasmic

inclusion bodies, intranuclear

incontinence, fecal

incoordination

infantile spasm

inflexibility, mental

insight, loss

insomnia

intellectual deficit

intellectual deficit, treatable causes of

intellectual deterioration

intelligence quotient

internet

intestinal pseudoobstruction

intracerebral hemorrhage

intracerebral hemorrhage, familial

intracerebral hemorrhage, lobar

intracerebral hemorrhage, recurrent

intracerebral hemorrhage, young adult

intrinsic hand muscles, wasting of

Jakob-Creutzfeldt disease

Jakob-Creutzfeldt disease, cerebellar variant

Jakob-Creutzfeldt disease, conjugal

Jakob-Creutzfeldt disease, medical precaution with

Jakob-Creutzfeldt disease, variant

Jakob-Creutzfeldt disease, young adult

Kayser-Fleischer ring

Kearns-Sayre syndrome

kinesia paradoxica

Kluver-Bucy syndrome

Korsakoff's psychosis

laminar necrosis, cortical

laminectomy

laminectomy, lumbar

language disorder in adults

Leber's hereditary optic neuropathy

leg weakness, bilateral

Leigh's disease

Leigh's disease, adult variety

lenticular nucleus, lesion of, bilateral

lethargy

leukemia, neurologic findings assoc.with

leukocyte enzyme abnormality

leukodystrophy

leukodystrophy, pigmented orthochromatic

leukoencephalopathy

leukoencephalopathy, adult onset, sporadic

leukoencephalopathy, differential diagnosis

leukoencephalopathy, hereditary diffuse

leukopenia

level of consciousness, decreased

Lewy body

Lewy body disease, diffuse

libido, decreased

life expectancy

limbic system

limbic-predominant age-related TDP-43 encephalopathy

linear lesion

lipid storage disorder of CNS

lipomembranous polycystic osteodysplasia

livedo reticularis

liver biopsy

liver disease

liver function enzymes

liver transplantation

lobar atrophy

Lorenzo's oil

lymphoma involving CNS

lysosomal storage disease

macrocephaly

magnetic susceptibility

malformation, vascular

malformation, vascular, cerebral

mania

manic-depressive

maple syrup urine disease

marche a petits pas

Marinesco-Sjogren syndrome

masked facies

medical-legal aspects of neurology

MELAS syndrome

memory, defect of recent

memory, impairment of

meningeal enhancement

meningismus

meningitis, carcinomatous

meningoencephalopathy

meningomyelitis

meningovascular syphilis

mental retardation

mental status, abnormal

MERRF syndrome

mesial temporal lobe

metabolic disorder, primary

metachromatic leukodystrophy

metachromatic leukodystrophy, adult onset

metachromatic leukodystrophy, juvenile

methotrexate

methylhydrazine derivatives

methylmalonic acidemia

metronidazole

microangiopathy, brain

microcephaly

micrographia

microhemorrhage, intracerebral

middle cerebellar peduncle

middle cerebellar peduncle, lesion

middle cerebellar peduncle, lesion, bilateral

migratory lesion pattern

mild cognitive impairment

mimics

Minamata disease

Mini Mental Status Examination

misdiagnosis

mitochondrial disease

mitochondrial encephalomyopathy

MNGIE syndrome

molecular genetics

mongolism

monoamine oxidase inhibitors

movement disorder, extrapyramidal

movement disorder, hyperkinetic

MRI, CAT scan compared to

MRI, complications with

MRI, contrast enhanced

MRI, diffusion weighted

MRI, disappearing lesion on

MRI, paramagnetic effect

MRI, punctate pattern

MRI, serial

MRI, spinal cord

MRI, spine

MRI, susceptibility weighted

MRI, T1 weighted high signal foci

MRI, target sign

MRS

multiple sclerosis

multiple sclerosis, cognitive presenttion

multiple sclerosis, differential diagnosis of

multiple sclerosis, familial

multiple sclerosis, misdiagnosis

multiple system atrophy

muscle biopsy

muscle pain

muscle stiffness

muscle wasting, diffuse

muscle weakness

muscle weakness, proximal

muscular dystrophy

muscular dystrophy, Duchenne

mutism

myasthenia gravis, differential diagnosis

myasthenia gravis, misdiagnosis of

myelitis

myelitis, longitudinal

myoclonus, stimulus sensitive

myopathy

myopathy, alcoholic

myopathy, mitochondrial

neoplasm, intracranial

neoplasm, primary of CNS

nerve biopsy

nerve conduction studies

neuroaxonal dystrophy

neuroaxonal dystrophy, infantile

neuroaxonal dystrophy, juvenile

neuroaxonal leukodystrophy

neurocutaneous disease

neuroendocrinology

neurofibrillary degeneration

neurofibromatosis 1

neurofibromatosis 2

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

neuronal ceroid-lipofuscinosis

neuronal intranuclear inclusion disease

neurons

neuroophthalmology

neuropathology

neuropathology, brain

neuropathy

neuropathy, amyloid

neuropathy, ataxia, retinitis pigmentosa

neuropathy, hereditary peripheral

neuropathy, peripheral

next-generation sequencing

obsessive-compulsive disorder

occipital lobe

occipital lobe, lesion of

occupational neurologic disorders

ocular motility, disorders of

old age, neurology of

opened mouth

ophthalmoplegia

ophthalmoplegia, progressive external

optic atrophy

optic atrophy, hereditary

optic neuropathy

optokinetic nystagmus, abnormal

orthostatic hypotension

osmotic demyelination syndrome

overlap syndrome

owl's eye sign of spinal cord

pain

pain, abdominal

pain, increased response

palatal myoclonus

palilalia

pallido-ponto-nigral degeneration

palmoplantar keratoderma

pancytopenia

paranoia

paraparesis

paraparesis, familial spastic

Parkinson disease, arteriosclerotic

Parkinson disease, atypical

Parkinson disease, dementia with

Parkinson disease, diagnosis

Parkinson disease, differential diagnosis of

Parkinson disease, drug induced

Parkinson disease, dystonia with

Parkinson disease, familial

Parkinson disease, freezing phenomena in

Parkinson disease, misdiagnosis

Parkinson disease, pathogenesis of

Parkinson disease, rapid progression

Parkinson disease, surgical treatment of

Parkinson disease, treatment of

Parkinson disease, young onset

Parkinsonism multiple-system atrophy

Parkinsonism syndrome

Parkinsonism-dementia complex

paroxysmal hemiplegia

paroxysmal neurologic deficits

PAS positive

PAS positive material in the brain

patient information and support

phenylketonuria, adult onset

pheochromocytoma

photosensitivity, skin

Pick's disease

pigmentary retinopathy

Pittsburgh Compound B

pituitary, hormones of

pleocytosis of cerebrospinal fluid

poison, mercury

poison, neurologic problems with

polycystic lipomembranous osteodysplasia

polyglucosan body

polyglucosan body disease

polymerase chain reaction

polyneuropathy

pons, lesion of

pontocerebellar atrophy

porphyria

port wine nevus

postoperative neurologic complications

postural abnormality

practice guidelines

precipitating factors

preclinical

pregnancy, neurologic complications in

prenatal diagnosis by amniocentesis

presenilin-1 gene

presenilin-2 gene

prevention of neurologic disorders

primary lateral sclerosis

progressive myoclonic epilepsy

progressive neurologic disorder

progressive pallidum atrophy

progressive subcortical gliosis

progressive supranuclear palsy

protein 14-3-3, cerebrospinal fluid

pseudoxanthoma elasticum

psychiatric disorder

psychiatric manifestations of brain tumors

psychiatric problems in neurologic disorders

psychological testing

psychomotor retardation

pursuit eye movements, abnormal

putamen, lesion of

putamen, lesion of, bilateral

pyramidal

pyramidal tract

pyramidal tract dysfunction

pyruvate metabolism, abnormality of

quadriparesis

quadriplegia

radiation encephalopathy, delayed

radiation therapy, CNS treatment and complications with

radiculopathy

ragged-red fibers

rapid onset dystonia parkinsonism

rapidly progressing neurologic illness

rash

real-time quaking-induced conversion

recombinant DNA

recurrent

reflex sympathetic dystrophy

release phenomena

remote effect of cancer on the nervous system

renal failure

renal stones

research

respiratory depression

salivation, excessive

Schilder's disease

schizophrenia

scoliosis

scoliosis, neurologic association with

seizure, laughing as manifestation

seizure, teenager

senile plaques

sensorineural hearing loss

single photon emission computed tomography

skin, biopsy

skin, darkening of

skin, lesions in neurologic disorders

sleep pathology and physiology

slit lamp examination

slow virus infection of CNS

small vessel disease

small vessel disease, cerebral

SNCA duplication

Sneddon's syndrome

snout reflex

somatosensory evoked potentials

spastic diplegia

spastic paraplegia, type 11

spasticity

speech disorder

speech disorder, childhood

spinal cord, infarction of

spinal cord, lesion of

spinal cord, pathologic exam of

spinal muscular atrophy

spinocerebellar ataxia

spinocerebellar ataxia type 1

spinocerebellar ataxia type 17

spinocerebellar ataxia type 7

spinocerebellar degeneration

spinopontine atrophy, dominant

splenomegaly

spongy degeneration of brain

spontaneous remission

sports medicine, neurology of

steroid therapy, CNS treatment and complications with

stimulation, deep brain

storage disease of CNS

striatonigral degeneration

striatonigral degeneration, infantile

striatum, lesion of

striatum, lesion of, bilateral

stridor

striopallidodentate calcifications, familial idiopathic

strokelike episodes

stuporous

Sturge-Weber syndrome

stuttering

subarachnoid hemorrhage

symmetric brain lesions

syncope

syphilis, meningomyelitis

syphilis, neurologic complications with

systemic illness

systemic lupus erythematosus

tachycardia

tandem gait, ataxic

tapetoretinal degeneration

tau protein

Tay-Sachs disease

TDP-43 proteinopathy

temporal lobe, atrophy

temporal lobe, lesion

temporal lobe, lesion, bilateral

testicular atrophy

thalamic tumors

thalamic tumors, bilateral

thalamus

thalamus, atrophy of

thalamus, infarction of

thalamus, infarction, bilateral

thalamus, lesion of

thalamus, lesion of-bilateral

thiamine

thromboangiitis obliterans cerebri

tongue, fasciculations of

tonic foot response

torticollis

torticollis, post traumatic

toxins, nervous system

transient ischemic attack

transient ischemic attack, recurrent

transient neurologic deficit

trauma

treatment of neurologic disorder

tremor

tremor, intention

tremor, post traumatic

tremor, resting

tremor, wing beating

trientine dihydrochloride

trinucleotide repeats

urea-cycle enzymopathies

uremia

urinary frequency

urinary incontinence

urine test for metabolic disorders

urine, dark

vanishing white matter

vasculopathy

venous thrombosis, non-cerebral

ventricular enlargement

vertigo

vertigo, episodic

very long chain fatty acids

vibratory sensation, abnormal

vinblastine

vincristine neurotoxicity

violent behavior

viral infection, CNS

vision, failure of in childhood

visual acuity, decreased

visual evoked response

visual fields, constricted

visual loss

visual loss, progressive

visual loss, slow

visuospatial disturbance

vocal cord paralysis

Von Hippel Lindau

walking frame

walking, difficulty with

weakness

weakness, acute

weakness, focal

weakness, progressive

weight loss

Wernicke's aphasia

Wernicke's encephalopathy

wheelchair

white matter disease

white matter disease, subcortical

whole genome sequencing

wide based gait

Wolff-Parkinson-White syndrome

word-finding difficulty

Showing articles 50 to 100 of 3870 << Previous Next >>

Degenerative Diseases of the Nervous System, Alzheimer Disease
Adams & Victors Principles of Neurology, Chp 39, pg 1063, Ropper, A.H.,et al, 2014

Degenerative Diseases of the Nervous System, Familial Dementia with Spastic Paraparesis
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Psychosis in an Adolescent Girl: A Common Manifestation in Niemann-Pick Type C Disease
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Mystery Case: A Young Boy with Myoclonic Jerks
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Clinical Reasoning: A Woman with Rapidly Progressive Apraxia
Neurol 80:e162-e165, Pressman, P.,et al, 2013

Sturge-Weber Syndrome and Port-Wine Stains Caused by Somatic Mutation in GNAQ
NEJM 368:1971-1979, Shirley, M.,et al, 2013

The Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) Scale
Stroke 43:2871-2876, Pescini, F.,et al, 2012

Cerebral Amyloid Angiopathy
eMedicine, Jan, Menon, R.S., 2012

Clinical and Biomarker Changes in Dominantly Inherited Alzheimers Disease
NEJM 367:795-804,864, Bateman, R.J.,et al, 2012

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

Differential Diagnosis of Bilateral Abnormalities of the Basal Ganglia and Thalamus
RadioGraphics 31:5-30, Hegde,A.N.,et al, 2011

Huntingtons Disease
BMJ 341:34-40, Novak,M.J. &Tabrizi,S.J., 2010

Bilateral Thalamic Lesions
AJR 192:W53-W62, Smith,A.B.,et al, 2009

Association of Parental Dementia With Cognitive and Brain MRI Measures in Middle-Aged Adults
Neurol 73:2071-2078, 2054, Debette,S.,et al, 2009

Clinicopath Conf., Adult Cerebral Form of X-Linked Adrenoleukodystrophy
NEJM 360:171-181, Case 1-2009, 2009

Genetic Aspects of Alzheimer Disease
The Neurologist 15:80-86, Williamson,J.,et al, 2009

Diagnosis and Therapy in Neuromuscular Disorders: Diagnosis and New Treatments in Mitochondrial Diseases
JNNP 80:943-953, Rahman,S. &Hanna,M.G., 2009

Biochemical Markers in Persons With Preclinical Familial Alzheimer Disease
Neurol 71:85-92,78, Ringman,J.M.,et al., 2008

Insights into the Dynamics of Hereditary Diffuse Leukoencephalopathy with Axonal Spheroids
Neurol 71:925-929, Van Gerpen,J.A.,et al, 2008

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

Tuberous Sclerosis Complex: A Tale of Two Genes
Neurol 70:904-905, Nass,R. &Crino,P.B., 2008

MR Imaging of Familial Creutzfeldt-Jakob Disease: A Blinded and Controlled Study
AJNR 29:1638-1643, Fulbright,R.K.,et al, 2008

Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts & Leukoencephalopathy (CADASIL)
UpToDate (www.uptodate.com) Aug 21, Dichgans,M.,et al., 2007

Variability of Age at Onset in Siblings With Familial Alzheimer Disease
Arch Neurol 64:1743-1748, G�mez-Tortosa,E.,et al, 2007

Neuroimaging Findings in Human Prion Disease
JNNP 78:664-670, Macfarlane,R.G.,et al, 2007

CT and MR Imaging of Neuroaxonal Leukodystrophy Presenting as Early-Onset Frontal Dementia
AJNR 27:1037-1039, Mascalchi,M.,et al, 2006

Young-Onset Dementia, A Practical Approach to Diagnosis
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Migraine and Cerebral White Matter Lesions
The Neurologist 11:19-29, Gladstone,J.P. &Dodick,D.W., 2005

Genetic Factors in Alzheimer's Disease
NEJM 352: 862-863, 884, Bird, T., 2005

The Genetic Causes of Basal Ganglia Calcification, Dementia, and Bone Cysts
Neurol 64:1502-1507, Klunemann,H.H.,et al, 2005

Familial Multiple Sclerosis and Other Inherited Disorders of the White Matter
The Neurologist 10:201-215, Kalman,B. &Leist,T.P., 2004

Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy (CADASIL)
Pract Neurology 4:50-55, Razvi,S.S.M. &Muir,K.W., 2004

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

Identification of New Presenilin Gene Mutations in Early-Onset Familial Alzheimer Disease
Arch Neurol 60:1541-1544,1521, Tedde,A.,et al, 2003

Alzheimer's Disease and Parkinson's Disease
NEJM 348:1356-1364, Nussbaum,R.L. &Ellis,C.E., 2003

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

Clinicopath Conf, Neuronal Ceroid Lipofuscinosis, Late-Onset Infantile Subtype
NEJM 347:672-680, Case 27-2002, 2002

Familial Dementia With Lewy Bodies
Arch Neurol 59:1622-1630, Tsuang,D.W.,et al, 2002

A Practical Approach to the Diagnosis and Management of MELAS: Case Report and Review
The Neurologist 8:302-312, Thambisetty,M.,et al, 2002

Adult-Onset Leukoencephalopathy with Vanishing White Matter Presenting with Dementia
Ann Neurol 50:665-668, Prass,K.,et al, 2001

Phenylketonuria Presenting in Adulthood as Progressive Spastic Paraparesis With Dementia
JNNP 71:795-797, Kasim,S.,et al, 2001

Linkage of Familial Amyotrophic Lateral Sclerosis with Frontotemporal Dementia to Chromosome 9q21-q22
JAMA 284:1664-1669, Hosler,B.A.,et al, 2000

Familial Occipital Calcifications, hemorrhagic Strokes, Leukoencephalopathy, Dementia, and External Carotid Dysplasia
Neurol 55:1661-1667, Iglesias,S.,et al, 2000

Autosomal Dominant Diffuse Leukoencephalopathy with Neuroaxonal Spheroids
Neurol 54:463-468, van der Knaap,M.S.,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

De Novo Mutation in the Notch3 Gene Causing CADASIL
Ann Neurol 47:388-391, Joutel,A.,et al, 2000

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

Adult-Onset MELAS Presenting as Herpes Encephalitis
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