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