Fronto-temporal lobar degeneration: logopaenic variant primary progressive aphasia

Case contributed by Dr Brad Hayhow

Presentation

Retired 70 year old tradesman presenting with 2 year history of progressive memory, speech and language problems.

Patient Data

Age: 70 years
Gender: Male

Mental State Examination

  • Neatly groomed and attired gentleman wearing spectacles.
  • Warm and friendly demeanour.
  • Good eye contact, easy rapport.
  • Mood “happy”, affect cheerful, reactive and congruent.
  • Speech effortful and halting with marked and frequent word-finding difficulty, occasional bursts of fluency marked by the use of circumlocutions and word approximations.
  • Goal-directed thought form.
  • No delusional beliefs or suicidal thoughts.
  • No hallucinations.
  • Alert and orientated.
  • Preserved insight into cognitive deficits.
  • Judgement intact.

Neuropsychological tasks

Modality: Diagram

Bedside Cognitive Profile (NUCOG)

  • Attention: 9/20
  • Visuoconstructional: 17/20
  • Memory: 10/20
  • Executive Function: 11.5/20
  • Language: 8/20
  • Total: 55.5/100

Marked asymmetric atrophy of the frontal pole of the left temporal lobe as well as the left frontal opercular region. The left fusiform and inferior temporal gyri are particularly atrophic with dilated temporal horn. Associated mild increased subcortical FLAIR signal. The rest of the brain, including the hippocampi demonstrate mild involutional change - slightly exaggerated for age.   

No intra-cranial mass lesions, acute haemorrhages or collections.  No acute or established infarcts.  Mild bi-hemispheric chronic white matter ischaemic change. Small anterior falcine calcification.

SPECT perfusion studies show severe perfusion reduction in the left temporal lobe with mild perfusion reduction extending into the left frontal and parietal cortex. Right temporal lobe perfusion uninvolved. Posterior cingulate perfusion preserved.

OVERALL IMPRESSION

The severe perfusion reduction involving the left temporal lobe is consistent with semantic dementia.

Case Discussion

Structural and functional neuroimaging suggests the diagnosis of advanced fronto-temporal dementia.

The history, mental state examination and cognitive assessment of this patient are most in keeping with a language variant fronto-temporal dementia.

The diagnosis of logopaenic variant primary progressive aphasia is favoured over (1) progressive non-fluent aphasia (PNFA) due to the patient's preserved semantics and grammar, and (2) semantic dementia due to the patients striking preservation of object recognition and single word repetition.

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Case Information

rID: 22365
Case created: 27th Mar 2013
Last edited: 20th Feb 2016
Inclusion in quiz mode: Included

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