TABLE 1.
Demographic and clinical features
| Case # | Sex | Age at onset | Total Disease duration (years) | Presenting symptoms at the time of dementia onset | Additional symptoms during the rapidly progressive clinical course | Final clinical diagnosis before death | Pathological diagnosis |
|---|---|---|---|---|---|---|---|
| 1 | F | 72 | 0.67 | Personality change | Limb apraxia and myoclonic jerks | CJD | CJD |
| 2 | F | 51 | 0.33 | Personality change, dysarthria, poor attention, hypersomnolence, and restless leg movements in sleep | Limb apraxia, myoclonic jerks and choreoathetoid movements | CJD | CJD |
| 3 | F | 80 | 0.25 | Speech difficulties and loss of episodic memory | Myoclonic jerks and excessive startle | CJD | CJD |
| 4 | M | 61 | 1.0 | Cognitive and behavioral changes and balance difficulties | Worsening of balance with falls, delusions and reduplicative para-amnesia | CJD | CJD |
| 5 | F | 41 | 0.25 | Body spasms, speech difficulties and abnormal involuntary arm movements | Worsening of balance with falls, myoclonic jerks and choreoathetoid movements | CJD | CJD |
| 6 | F | 68 | 0.42 | Loss of episodic memory | Worsening of balance with falls myoclonic jerks and hallucinations | CJD | CJD |
| 7 | F | 73 | 0.17 | Cognitive decline, balance difficulties and excessive startle | Worsening of balance with falls and stereotypical rubbing of the bed sheet. | CJD | CJD |
| 8 | M | 56 | 1.0 | Cognitive and behavioral changes and difficulties with gait | Urinary incontinence, ataxia myoclonic jerks and fasciculations | CJD | CJD |
| 9 | F | 67 | 3.5 | Difficulties with gait | Impaired attention, aphasia and parkinsonism | Dementia NOS | FTLD-MND |
| 10 | M | 33 | 2.0 | Personality change | Impaired judgment and planning | FTD | FTLD-MND |
| 11 | F | 48 | 2.0 | Behavioral changes and dysarthria | Cognitive changes and paranoia | FTD vs. AD | FTLD-MND |
| 12 | F | 52 | 2.5 | Paranoid behaviors and dysarthria | Cognitive changes | FTD-MND | FTLD-MND |
| 13 | F | 50 | 2.0 | Personality change | Aphasia and dysarthria | FTD-MND | FTLD-MND |
| 14 | M | 85 | 2.5* | Memory loss, difficulty putting words together and wandering at night | Incontinence of urine, reduced personal hygiene, fluctuations and RBD | DLB | DLBD |
| 15 | M | 72 | 3.0* | Increasingly confused and disoriented, agitated, word finding difficulties and hallucinations | Fluctuations throughout the daytime, shuffling gait, falls | DLB | DLBD |
| 16 | F | 81 | 3.0* | Increasingly confused with short term memory loss | Hallucinations, parkinsonism | CJD | DLBD |
| 17 | F | 79 | 3.5 | Word finding pauses in speech | Cognitive impairment, dystonia and apraxia of limb movement | CBD | Tauopathy (PSP) |
| 18 | M | 83 | 1.5 | Gait difficulties with falls and choreoathetoid movements | Cognitive impairment and apraxia of speech | CJD | Tauopathy (PSP) |
| 19 | M | 54 | 3.5 | Personality change | Parkinsonism and stiffness of muscles | FTD | Tauopathy (CBD) |
| 20 | M | 63 | 3.5 | Cognitive problems with confusion and obsessive behaviors | Parkinsonism and stiffness of muscles | FTDP | Tauopathy (CBD) |
| 21 | M | 72 | 3.0 | Impaired judgment and confusion | Fluctuating course, RBD and hallucinations | Dementia NOS | AD |
| 22 | M | 74 | 1.2 | Gait difficulties, cognitive impairment and light headedness on standing | Hallucinations and RBD | DLB | AD |
AD = Alzheimer's disease; CJD= Creutzfeldt-Jakob disease; FTD = Frontotemporal dementia; FTDP = Frontotemporal dementia with parkinsonism; FTD-MND = frontotemporal dementia with motor neuron disease; FTLD-MND = frontotemporal lobar degeneration with motor neuron degeneration; DLB = dementia with Lewy bodies; DLBD = Diffuse Lewy body disease; PSP = progressive supranuclear palsy; CBD = corticobasal degeneration; RBD = rapid eye movement sleep behavior disorder
• Total disease duration is based on the time of onset being recognized as the transient postoperative- or illness- associated confusion, and not as the time of onset of the dementia.