Supplemental Online Content

Arvanitakis Z, Shah RC, Bennett DA. Diagnosis and management of dementia: review. JAMA.

doi:10.1001/jama.2019.4782

eTable 1. Atypical Features of Dementia Syndromes and Mimickers, for Which Additional

Work up May Be Needed

eTable 2. Potentially Treatable Medical Conditions Which Can Present With Cognitive

Impairment and Dementia*

eTable 3. Online Resources for Patients and Caregivers

This supplementary material has been provided by the authors to give readers additional

information about their work.

© 2019 American Medical Association. All rights reserved.

eTable 1. Atypical Features of Dementia Syndromes and Mimickers, for Which Additional Work up
May Be Needed

CATEGORY EXAMPLES OF FEATURE EXAMPLES OF CAUSES

Young-onset (e.g., significant memory Autosomal dominant forms of AD (e.g.,

decline in patient younger than age 65 PSEN1 mutations), toxins (drug-related)
Cognitive years)
problem onset Rapidly progressive course over weeks Herpes simplex meningoencephalitis, prion
and progression or months disease (e.g., Creutzfeldt-Jakob disease)
Episodic course with normal cognition Epilepsy, intermittent exposure to toxins
in between episodes (drug-related)

Seizures Space occupying brain lesion (e.g., benign

or malignant tumor, abscess), focal cortical
dysplasia
Bilateral upper motor neuron findings Frontotemporal dementia associated with
(e.g., weakness, spasticity), with or amyotrophic lateral sclerosis
Other neurologic without lower motor neuron findings
features early in (e.g., fasciculations)
the course of the
Parkinsonism, vertical gaze palsy, falls Progressive supranuclear palsy
illness, within
Asymmetric parkinsonism, cortical Corticobasal degeneration
months or 1-2
sensory loss, alien limb syndrome
years of
Gait impairment with or without falls Subdural hematoma, normal pressure
cognitive
hydrocephalus
problem
Chorea Huntington’s disease
Ataxia Alcohol abuse, cerebellar degeneration
syndrome
Severe or new-onset chronic headache Subarachnoid hemorrhage, primary cerebral
angiitis

Fever Encephalitis (e.g., viral)

Signs suggestive of metabolic disease Hepatic encephalopathy (e.g., from
(e.g., jaundice) medication side effects, alcohol, other)
Systemic signs
Dermatologic (e.g., rash) or Autoimmune disease (e.g., systemic lupus)
rheumatologic signs (e.g., joint
swelling)

© 2019 American Medical Association. All rights reserved.

eTable 2. Potentially Treatable Medical Conditions Which Can Present With Cognitive
Impairment and Dementia*

CATEGORY EXAMPLE OF CONDITION TREATMENT OPTION

Vitamin B12 deficiency B12 replacement

Hypothyroidism Thyroid replacement
Metabolic disorders
Vitamin B1 deficiency (Wernicke-Korsakoff Thiamine, alcohol cessation
syndrome)
Subdural hematoma Surgical evacuation of blood
Vascular processes
Primary cerebral angiitis Immunosuppressive drugs
Epilepsy Status epilepticus Anti-epileptic drugs
Primary in the brain (e.g., meningioma most Excision of brain tumor
commonly; others such as glioblastoma, Brain radiotherapy
astrocytoma) Treat underlying cancer (e.g.,
Tumors
Brain tumor secondary to systemic cancer chemotherapy)
(metastases)
Paraneoplastic syndrome (e.g., limbic encephalitis)
Depression Antidepressant drugs
Psychiatric disorders Bipolar disorder Lithium, antidepressant drugs
Schizophrenia Atypical neuroleptic drugs
Drug-related, single drug and polypharmacy Discontinuation or tapering of
(prescription and non-prescription, such as over- causative drug(s)
Toxins the-counter and illicit)
Heavy metal intoxication Chelating drugs
Chemotherapy (e.g., methotrexate) Discontinuation of therapy
Meningoencephalitis: syphilis, herpes simplex, Antibiotic/ anti-viral drugs
Infections HIV
Abscess: tuberculoma Drain abscess, antimicrobials
Inflammatory Temporal arteritis Corticosteroids
disorders Neurosarcoidosis Corticosteroids
Multiple sclerosis and leukoencephalopathy Immunosuppressive drugs
Hashimoto’s encephalopathy Corticosteroids
Immune disorders
Various rheumatologic diseases (e.g., systemic Immunosuppressive drugs
lupus)
Genetic disorders Wilson’s disease (hepatolenticular degeneration) D-penicillamine, trientine
Normal pressure hydrocephalus (classic triad of Shunting of cerebrospinal
Other urinary incontinence, cognitive impairment, and fluid
gait disturbance)
* Other accompanying symptoms and signs beyond cognitive impairment may be present; more detailed descriptions of
clinical features for these and other conditions is beyond the scope of this review and are found elsewhere (see Memory
Loss, Alzheimer's Disease, and Dementia: A Practical Guide for Clinicians. Elsevier, 2nd Edition (2015), by Andrew E.
Budson and Pail R. Solomon)

© 2019 American Medical Association. All rights reserved.

eTable 3. Online Resources for Patients and Caregivers

Alzheimer’s Association: https://www.alz.org (also includes a range of programs, such as a

free 24 hour hotline for caregivers, service in Spanish, a “safe-return” program for patients
who get lost, etc.)

Alzheimer's and related Dementias Education and Referral Center:

https://www.nia.nih.gov/health/about-adear-center

Dementia Friendly America: http://www.dfamerica.org/

National Institutes of Health: https://www.nia.nih.gov/health with a variety of resources,

including a manual entitled “Caring for a Person with Alzheimer’s Disease – Your easy-To-
Use Guide from the National Institute on Aging”:
https://order.nia.nih.gov/sites/default/files/2017-07/Caring_for_person_with_AD_508_0.pdf

© 2019 American Medical Association. All rights reserved.