A 60 year old man with Progressive Dementia and sillly behavior

Progressive dementia may be due to a variety of causes (toxic, metabolic, vascular, genetic, infectious, neoplastic (cancerous), neurodegenerative and inflammatory. A specific type of behavioural problem known as a frontal syndrome may co-exist with memory problems in certain forms of dementia.

Progressive Dementia Slideshow transcript

Slide 2: • 60 year old man • R handed • Married + 2 children • Admitted electively with complaints of cognitive decline and gait difficulty for the past 2 ½ years

Slide 3: History (collateral) • 2 ½ years prior to admission patient had a RTA ( unclear how) without head injury • From that point family became aware of cognitive changes: - Apathy - Memory disturbance

Slide 4: History 2 • Over following months cognitive complaints exacerbated • Unable to find way around familiar places • Urinary incontinence • Began to develop behavioural changes especially “jokiness” with inappropriate comments

Slide 5: History 3 • About 8 months prior to admission family noticed gait disturbance and other movements becoming difficult - Standing from sitting - Turning over in bed • In addition cognitive aspects continued to deteriorate, 6 months ago had to close shop • Kept returning to shop and believed someone was trying to steal from him • Overeating and weight gain • Referred for elective admission to investigate cause of progressive dementia.

Slide 6: History 4 • Patient / family deny: – Step-wise deteriorations – Myoclonus – Language disorder – Headache – Falls

Slide 7: Past history • Hyperlipidemia • Denies: – Diabetes – Smoking – HTN – IHD – Alcoholism • Treated with aspirin only

Slide 8: Examination • General examination unremarkable • Fully conscious • Cranial nerves: – Mild facial asymmetry ( R side weak) – Otherwise intact, no gaze or visual disturbance

Slide 9: Examination 2 • Motor: – Tone increased on R side (“ paratonia”) – Power 5/5 x 4 – Reflexes increased, more on right – Bilateral pyramidal signs

Slide 10: Examination 3 • Sensory examination unremarkable • No cerebellar signs • Romberg –ve • Gait intact • Movement apraxia – Difficulty changing postion without any weakness

Slide 11: Cognitive 1 • MMSE 16 / 30 - Lack of orientation in time - Partial lack of orientation in space -Stimulus-bound responses - Disturbance of concentration - Disturbance of STM - Occasional “ silly responses”

Slide 12: Cognitive 2 • Occipital – Mild visual object agnosia (L OT) – No - Field defects - Other defects of higher visual function

Slide 13: Cognitive 3 • Parietal – Topographagnosia ( R PO) – Ideomotor apaxia (L) – Constructional apraxia (R) – NO: – Neglect – Astereognosis / agraphaesthesia

Slide 14: Cognitive 4 • Temporal – Semantic paraphasias – Disturbed time perception

Slide 15: Cognitive 5 • Frontal: • Release signs – Witzelsucht – Tactlessness – PMR bilaterally – Abulia – Pout – Lack of attention – No grasp – Stimulus-bound ( reverse grasping) – Concrete thinking – Difficulty with abstraction – Perseveration

Slide 16: Blood work-up prior to admission • FBC normal • Bioch normal • ESR 38 • TSH 1.29 ( antithyroglobulin negative) • B1 / B12 / Folate normal • ANA / ENA /VDRL all negative

Slide 17: Investigations • FBC normal • Bioch normal • ESR 68 • TSH 1.79 ( antithyroglobulin negative) • B12 / Folate normal • LP:- TP 597 - no cells • EEG normal

Slide 18: Brain MRI

Slide 21: Paper Summary • FTD is a clinical syndrome • Behavioral or Language presentation ( or combination ) • Pathologically heterogeneous progressive dementia

Slide 29: S tr u c tu r a l Im a g in g • Magnetic resonance imaging (MRI) scans indicate that both primary progressive aphasia (PPA) and FTD patients show frontotemporal atrophy • In PPA patients the focus of atrophy is in the left temporal lobe • Focus of atrophy is mainly in R frontal lobe in “FTD” patients • In AD , the mesial temporal lobes are specifically atrophic in

Slide 32: Paper Summary • Overlap between FTD, PSP, CBD Clinical FTD Clinical CBD Pathologically FTD Pathologically CBD

Slide 33: Clinicopathological Heterogeneity In 32 autopsied cases of the CBS pathology: CBD 18 Alzheimer’s disease 3 Pick’s disease 2 PSP 6 Dementia lacking distinctive histology 2 Creutzfeldt–Jakob disease 3

Slide 34: MRI Findings in “ corticobasal syndrome” • Asymmetrical cortical atrophy, especially frontoparietal • Asymmetrical atrophy in the basal ganglia, lateral ventricles, and cerebral peduncles • Atrophy of the middle or posterior segment of the corpus callosum • Signal changes in the putamen • Hyperintense subcortical signal changes in motor and somatosensory cortex

Slide 36: C o n c lu s io n s An increased plasma homocysteine level is a strong, independent risk factor for the development of dementia and Alzheimer’s disease. (N Engl J Med 2002;346:476-83.) Above independent of any association with vascular dementia Out of 111 subjects: - AD 78 - VD 11 - “ non AD degenerative dementias” 11 - other 6

Slide 39: Case report • 62 year old with 3 year history of - apathy - confusion - paranoid delusions, loss of social graces - no localising signs - frontal release signs - cognitive syndrome - attention ( + STM) - perseveration - preserved language- progressive dementia

Slide 40: CADASIL MRI

Slide 41: CADASIL skin biopsy

Slide 44: Summary 1. FTD (clinically, cortical atrophy, w.matter) 2. CBS (asymmetric rigidity, parietal signs, overlap with FTD, frequent w. matter signs, bulk of syndrome frontal) 3. Vascular aetiologies should be excluded - “ stroke in the young” esp. homocysteine - ? Skin biopsy - ? Angio 4. If above all negative consider brain biopsy 5. Classical “ white matter diseases ” very unlikely due to extensive cortical involvement both clinically and radiologically to explain progressive dementia.

Slide 45: Progressive Dementia

Submitted to AskTheNeurologist.Com in 2007 Author Anon.

Ask The Neurologist about Progressive Dementia