Familial cases of early-onset prominent frontal lobe dysfunction associated with epilepsy have not been reported to date. We report a mother and her only daughter with incapacitating behavioral manifestations of frontal lobe dysfunction and epilepsy of variable severity. The possibility of a hitherto undescribed genetic condition is discussed.
Key words:
frontal lobe; genetics; behavioral; neuropsychiatry; epilepsies; partial; heredity
| Cognitive domains / specific tests* | Mother - at age 28§¶ | Mother - at age 49¶ | Daughter - at age 28§ | |
|---|---|---|---|---|
| Memory - Wechsler Memory Scale - Revised (SD) | Verbal Memory I | -0.8 | -1.6 | -1.8 |
| Verbal Memory II | -1.3 | -1.8 | -0.9 | |
| Visual Memory I | 0.2 | -3.6 | -0.7 | |
| Visual Memory II | -3.2 | -3.8 | -1.1 | |
| Verbal learning - Rey Auditory Verbal Learning Test (SD) | Verbal Learning I | -1.0 | -1.7 | -1.7 |
| Verbal Learning II | -2.1 | -1.1 | -1.3 | |
| Intelligence - Wechsler Adult Intelligence Scale (raw scores and corresponding categories) | Arithmetic | 7 / low average | 7 / low average | 6 / low average |
| Digit Span | 11 / average | 11 / average | 7 / low average | |
| Similarities | 11 / average | 9 / average | 10 / average | |
| Comprehension | 10 / average | 6 / low average | 4 / low | |
| Block Design | 8 / low average | 7 / low average | 8 / low average | |
| Picture Completion | 8 / low average | 10 / average | 11 / average | |
| Picture Arrangement | 8 / low average | 9 / average | 9 / average | |
| Digit Symbol | 7 / low average | 7 / low average | 7 / low average | |
| Attention and processing speed - Trail Making Test (SD) | Test A | -3.9 | -16.5 | -2.7 |
| Test B | -3.2 | -12.3 | -7.9 | |
| Executive function | Wisconsin Card Sorting Test (raw score) | 3 out of 6 | 3 out of 6 | 0 out of 6 |
| Visuospatial ability | Rey-Osterrieth Complex Figure Test (SD) | 0.2 | -8.0 | -2.6 |
| Hooper Visual Organization Test (category) | superior | very superior | N/A | |
| Language | Boston Naming Test (SD) | 1.1 | 0.5 | 0.9 |
