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Table 1 Principal characteristics of the seven subjects with Fahr’s disease

From: Bilateral strio-pallido-dentate calcinosis (Fahr’s disease): report of seven cases and revision of literature

Case Gender Age Diagnosis Risk factors Classification by Manyam (and other neuroimaging findings) Psychotic symptoms Cognitive impaiment Other Psychiatric disorders Extrapyramidal disorders Other neurologic abnormalities Response to neuroleptics
1 Male 73 Primary
Late onset
(cerebrovascular disease ?) SPD calcinosis (+ left, frontal malacic area, leukoaraiosis, cortical atrophy) Severe: well-structured paranoid delusions of robbery and conspirancy, with strong emotional involvment Severe, progressive: memory, language, orientation, calculation, attention (MMSE: 19/30; MODA: 88/100) Apathia, Bradykinesia
Later, secondary hypertonia, diskinesias, tremor, gait disturbances
2 Male 37 Secondary
Early onset
Head injury SP calcinosis Severe: poorly structured paranoid delusions of body transformation and greatness, auditory hallucinations, aggressiveness Mild: language, comprehension, abstract reasoning, visual-motor ability.
(WAIS: below lower limit)
Flattening of affectivity Late, secondary (facial and limb diskinesias) Amimia, fatuous expression Poor
3 Male 54 Primary
Late onset
  SP calcinosis (+ mild cerebellar atrophy) Absent Mild: memory Anxious-depressive syndrome focused on health problems Absent - -
4 Female 48 Secondary
Early onset
Head injuries, Hyperparathyroidism SP calcinosis (+ mild cerebellar atrophy) Absent Mild: attention, memory, logical reasoning, language, visual-spatial skills. Anxious-depressive syndrome Absent Inexhaustible glabellar reflex -
5 Female 63 Primary
Late onset
  SPD + occipital calcinosis (+ frontal leukoaraiosis; left ponto-cerebellar cistern meningioma) Absent Very mild: sustained and divided attention Mild focus on health condition Absent - -
6 Female 73 Secondary
Late onset
Hyperparathyroidism SP calcinosis (worsened at follow-up with development of moderate atrophy, leukoaraiosis and left parietal cortical infarction) Progressive: visual hallucinations, then aggressiveness, paranoid delusions Progressive: memory, orientation. (initial MMSE: 24.7/30, then progressive worsening). Major depression Early, progressive (worsening tremor of superior limbs, then gait disturbances) Palmo-mental reflex Partial
7 Female 82 Secondary
Late onset
Hyperparathyroidism, previous stroke? SPD calcinosis (+ diffuse atrophy) Progressive: visual and auditory hallucinations, then aggressiveness Progressive, severe: memory, orientation, attention (MMSE 19.5/30) Depression Early (diffuse hypertonia, resting and intentional tremor, gait disturbances) Inexhaustible glabellar reflex, bilateral Babinski reflex Good
  1. Legend: MMSE mini mental state examination, MODA Milan overall dementia assessment, SP strio-pallidal, SPD strio-pallido-dentate, WAIS Wechsler Adult Intelligence Scale