A 60 year old man with Progressive Dementia and sillly behavior

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A 60 year old man with Progressive Dementia and “silly behavior”:- Case Discussion

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A 60 year old man with Progressive Dementia and sillly behavior

  1. 1. Progressive Dementia and “silly behavior” Submitted to AskTheNeurologist.Com in 2007 Author Anon.
  2. 2. <ul><li>60 year old man </li></ul><ul><li>R handed </li></ul><ul><li>Married + 2 children </li></ul><ul><li>Admitted electively with complaints of cognitive decline and gait difficulty for the past 2 ½ years </li></ul>
  3. 3. History (collateral) <ul><li>2 ½ years prior to admission patient had a RTA ( unclear how) without head injury </li></ul><ul><li>From that point family became aware of cognitive changes: </li></ul><ul><ul><ul><li>- Apathy </li></ul></ul></ul><ul><ul><ul><li>Memory disturbance </li></ul></ul></ul>
  4. 4. History 2 <ul><li>Over following months cognitive complaints exacerbated </li></ul><ul><li>Unable to find way around familiar places </li></ul><ul><li>Urinary incontinence </li></ul><ul><li>Began to develop behavioural changes especially “jokiness” with inappropriate comments </li></ul>
  5. 5. History 3 <ul><li>About 8 months prior to admission family noticed gait disturbance and other movements becoming difficult </li></ul><ul><li>- Standing from sitting </li></ul><ul><li>- Turning over in bed </li></ul><ul><li>In addition cognitive aspects continued to deteriorate, 6 months ago had to close shop </li></ul><ul><li>Kept returning to shop and believed someone was trying to steal from him </li></ul><ul><li>Overeating and weight gain </li></ul><ul><li>Referred for elective admission </li></ul>
  6. 6. History 4 <ul><li>Patient / family deny: </li></ul><ul><ul><li>Step-wise deteriorations </li></ul></ul><ul><ul><li>Myoclonus </li></ul></ul><ul><ul><li>Language disorder </li></ul></ul><ul><ul><li>Headache </li></ul></ul><ul><ul><li>Falls </li></ul></ul>
  7. 7. Past history <ul><li>Hyperlipidemia </li></ul><ul><li>Denies: </li></ul><ul><ul><li>Diabetes </li></ul></ul><ul><ul><li>Smoking </li></ul></ul><ul><ul><li>HTN </li></ul></ul><ul><ul><li>IHD </li></ul></ul><ul><ul><li>Alcoholism </li></ul></ul><ul><li>Treated with aspirin only </li></ul>
  8. 8. Examination <ul><li>General examination unremarkable </li></ul><ul><li>Fully conscious </li></ul><ul><li>Cranial nerves: </li></ul><ul><ul><li>Mild facial asymmetry ( R side weak) </li></ul></ul><ul><ul><li>Otherwise intact, no gaze or visual disturbace </li></ul></ul>
  9. 9. Examination 2 <ul><li>Motor: </li></ul><ul><ul><li>Tone increased on R side (“ paratonia”) </li></ul></ul><ul><ul><li>Power 5/5 x 4 </li></ul></ul><ul><ul><li>Reflexes increased, more on right </li></ul></ul><ul><ul><li>Bilateral pyramidal signs </li></ul></ul>
  10. 10. Examination 3 <ul><li>Sensory examination unremarkable </li></ul><ul><li>No cerebellar signs </li></ul><ul><li>Romberg –ve </li></ul><ul><li>Gait intact </li></ul><ul><li>Movement apraxia </li></ul><ul><ul><li>Difficulty changing postion without any weakness </li></ul></ul>
  11. 11. Cognitive 1 <ul><li>MMSE 16 / 30 </li></ul><ul><ul><li>Lack of orientation in time </li></ul></ul><ul><ul><li>Partial lack of orientation in space </li></ul></ul><ul><ul><li>-Stimulus-bound responses </li></ul></ul><ul><ul><li>- Disturbance of concentration </li></ul></ul><ul><ul><li>Disturbance of STM </li></ul></ul><ul><ul><li>Occasional “ silly responses” </li></ul></ul>
  12. 12. Cognitive 2 <ul><li>Occipital </li></ul><ul><ul><li>Mild visual object agnosia (L OT) </li></ul></ul><ul><ul><li>No </li></ul></ul><ul><ul><ul><ul><li>Field defects </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Other defects of higher visual function </li></ul></ul></ul></ul>
  13. 13. Cognitive 3 <ul><li>Parietal </li></ul><ul><ul><li>Topographagnosia ( R PO) </li></ul></ul><ul><ul><li>Ideomotor apaxia (L) </li></ul></ul><ul><ul><li>Constructional apraxia (R) </li></ul></ul><ul><ul><li>NO: </li></ul></ul><ul><ul><ul><ul><li>Neglect </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Astereognosis / agraphaesthesia </li></ul></ul></ul></ul>
  14. 14. Cognitive 4 <ul><li>Temporal </li></ul><ul><ul><li>Semantic paraphasias </li></ul></ul><ul><ul><li>Disturbed time perception </li></ul></ul>
  15. 15. <ul><li>Frontal: </li></ul><ul><ul><li>Witzelsucht </li></ul></ul><ul><ul><li>Tactlessness </li></ul></ul><ul><ul><li>Abulia </li></ul></ul><ul><ul><li>Lack of attention </li></ul></ul><ul><ul><li>Stimulus-bound </li></ul></ul><ul><ul><li>Concrete thinking </li></ul></ul><ul><ul><li>Difficulty with abstraction </li></ul></ul><ul><ul><li>Perseveration </li></ul></ul>Cognitive 5 <ul><li>Release signs </li></ul><ul><ul><li>PMR bilaterally </li></ul></ul><ul><ul><li>Pout </li></ul></ul><ul><ul><li>No grasp </li></ul></ul><ul><ul><li>( reverse grasping) </li></ul></ul>
  16. 16. Blood work-up prior to admission <ul><li>FBC normal </li></ul><ul><li>Bioch normal </li></ul><ul><li>ESR 38 </li></ul><ul><li>TSH 1.29 ( antithyroglobulin negative) </li></ul><ul><li>B1 / B12 / Folate normal </li></ul><ul><li>ANA / ENA /VDRL all negative </li></ul>
  17. 17. Investigations <ul><li>FBC normal </li></ul><ul><li>Bioch normal </li></ul><ul><li>ESR 68 </li></ul><ul><li>TSH 1.79 ( antithyroglobulin negative) </li></ul><ul><li>B12 / Folate normal </li></ul><ul><li>LP: - TP 597 </li></ul><ul><ul><li>- no cells </li></ul></ul><ul><li>EEG normal </li></ul>
  18. 18. Brain MRI
  19. 21. Paper Summary <ul><li>FTD is a clinical syndrome </li></ul><ul><li>Behavioral or Language presentation </li></ul><ul><li>( or combination ) </li></ul><ul><li>Pathologically heterogeneous </li></ul>
  20. 29. <ul><li>Structural Imaging </li></ul><ul><li>Magnetic resonance imaging (MRI) scans indicate that both primary progressive aphasia (PPA) and FTD patients show frontotemporal atrophy </li></ul><ul><li>In PPA patients the focus of atrophy is in the left temporal lobe </li></ul><ul><li>Focus of atrophy is mainly in R frontal lobe in “FTD” patients </li></ul><ul><li>In AD , the mesial temporal lobes are specifically atrophic in </li></ul>
  21. 32. Paper Summary <ul><li>Overlap between FTD, PSP, CBD </li></ul><ul><ul><li>Clinical FTD Clinical CBD </li></ul></ul><ul><ul><li>Pathologically FTD Pathologically CBD </li></ul></ul>
  22. 33. <ul><li>Clinicopathological Heterogeneity </li></ul><ul><li>In 32 autopsied cases of the CBS pathology: </li></ul><ul><li>CBD 18 </li></ul><ul><li>Alzheimer’s disease 3 </li></ul><ul><li>Pick’s disease 2 </li></ul><ul><li>PSP 6 </li></ul><ul><li>Dementia lacking distinctive histology 2 </li></ul><ul><li>Creutzfeldt–Jakob disease 3 </li></ul>
  23. 34. MRI Findings in “ corticobasal syndrome” <ul><li>Asymmetrical cortical atrophy, especially frontoparietal </li></ul><ul><li>Asymmetrical atrophy in the basal ganglia, lateral </li></ul><ul><li>ventricles, and cerebral peduncles </li></ul><ul><li>Atrophy of the middle or posterior segment of the corpus callosum </li></ul><ul><li>Signal changes in the putamen </li></ul><ul><li>Hyperintense subcortical signal changes in motor and somatosensory cortex </li></ul>
  24. 36. Conclusions 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
  25. 39. Case report <ul><li>62 year old with 3 year history of </li></ul><ul><ul><li>- apathy </li></ul></ul><ul><ul><li>- confusion </li></ul></ul><ul><ul><li>- paranoid delusions , loss of social graces </li></ul></ul><ul><ul><li>- no localisin g signs </li></ul></ul><ul><ul><li>- frontal release signs </li></ul></ul><ul><ul><li>- cognitive syndrome - attention ( + STM) </li></ul></ul><ul><ul><li>- perseveration </li></ul></ul><ul><ul><li>- preserved language </li></ul></ul>
  26. 40. CADASIL MRI
  27. 41. CADASIL skin biopsy
  28. 44. Summary <ul><li>FTD (clinically, cortical atrophy, w.matter ) </li></ul><ul><li>CBS (asymmetric rigidity, parietal signs, overlap with FTD, frequent w. matter signs, bulk of syndrome frontal ) </li></ul><ul><li>Vascular aetiologies should be excluded </li></ul><ul><ul><li>- “ stroke in the young” esp. homocysteine </li></ul></ul><ul><ul><li>- ? Skin biopsy </li></ul></ul><ul><ul><li>- ? Angio </li></ul></ul><ul><li>If above all negative consider brain biopsy </li></ul><ul><li>Classical “ white matter diseases ” very unlikely due to extensive cortical involvement both clinically and radiologically </li></ul>
  29. 45. Submitted to AskTheNeurologist.Com in 2007 Author Anon.

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