Diagnosis, Treatment and Rehabilitation of Non-Alzheimer Dementias

78 Pages·2012·6.78 MB·English

by  Chiadi U. Onyike

Chiadi U. Onyike
Johns Hopkins University
Division of Geriatric Psychiatry and Neuropsychiatry
Disclosure:

 PI of the Baltimore site of the NAM-53 Multicenter trial of
memantine for frontotemporal dementia
 Discussion of medicines pertains to “off-label” use
Objectives:

 Examine phenotype(s), epidemiology and pathobiology
 Review evaluation and diagnosis
 Discuss treatments and rehabilitative interventions
 Is it “memory loss”?
 Is it “old age”?
How is it different

from Alzheimer
disease?
Types of dementia
Most common cause between ages 50-65;
Alzheimer disease, AD 33% of dementia below age 65
Cerebrovascular disease, CVD Genetic varieties particularly
Frontotemporal dementias, FTD 1:1 or greater ratio FTD:AD below age 50
Dementia with Lewy bodies Uncommon before age 65
Traumatic brain injury, TBI Follows head trauma
HIV/AIDS dementia Prevalence has decreased since HAART
Mostly associated with nutritional
Alcohol-related dementia deficienceis
Huntington disease, HD Among the commonest below age 35
Prion dementias Generally rare beyond age 65
Multiple sclerosis, MS
Normal Pressure Hydrocephalus
McMurtay et al, 2006
Generally non-amnestic

Disorders of temperament and conduct

Motor features: parkinsonism, apraxia,

ataxia, tremor, gait disorder
Mid-life onset common

 Insidious deterioration
 Behavioral disinhibition and asocial
behavior
 Inertia and apathy
 Loss of empathy/sympathy
 Perseveration, stereotypies and
compulsions
 Hyperorality and altered eating habits
 Executive dysfunction
 Focal abnormalities on brain imaging
 Tau, progranulin or FUS mutations
Sources: Rascovskyet al., 2011
 A technician of 55 had difficulty findings words, which in a few years evolved to
dysfluency, repetitiousness, stereotypies and echolalia. In the 2nd year, his work
efficiency deteriorated due to his poor comprehension and reasoning, so he was placed
on disability leave. He was childlike, impulsive and unfeeling. He insisted on the same TV
shows. His manners coarsened – examples include indiscrimmatelyapproaching
strangers, eating out of a serving bowl, jumping queues, abruptness in conversations. He
was restless - biking, swimming laps and running 6.5 miles each day. His wife arranged
his “volunteering” at a local nursing home, where he made rounds with maintenance
crews all day long. She noted that his awareness of tiredness seemed impaired.
 On examination 30 months ago, he was pleasant and proper. Depression was not
evident, and he did not have euphoria, psychosis or paranoia. Speech was mildly non-
fluent. Verbal fluency was impaired. MMSE 29 (3MS 96). Brain MRI showed right
temporal atrophy. A year later he developed marked preference for sweets, along with
foraging and overeating. Two years later he was admitted to residential care. At the last
visit 6 months ago, MMSE score was 27.
 Progressive coarsening of conduct
 Literalness and rigidity
 Indifference
 Impulsions and compulsions
 Sweet tooth and hyperphagia
 Impaired perception of body states/functions
 Verbal and motor stereotypies
 Hyperkinesis
 Motor phenomena
 Focal atrophy on MRI
 MMSE 29!
 Agrammatism
 Effortful, halting speech with sound
errors and distortions
 Impaired comprehension of syntactically
complex sentences
 Impaired confrontation naming
 Impaired word comprehension
 Impaired object knowledge
 Surface dyslexia and/or dysgraphia
 Impaired word retrieval
 Impaired repetition of sentences and
phrases
 Phonological errors in spontaneous
speech
Sources: Gorno-Tempiniet al., 2011