• Characteristics of hippocampal sclerosis include:
  • Cortical neuron loss
  • Loss of pyramidal cells in the hippocampus
  • Possible co-morbidity with cardiovascular disease, depression, and other brain pathology, but lacks sufficient plaques and tangles to diagnose co-morbid Alzheimer’s disease
  • Reports of dysexecutive syndrome, behavior disturbances and expressive aphasia at onset, with relatively mild impairment in other realms
  • Course often difficult to distinguish from AD
    

• To document the neuropsychological course of a woman with hippocampal sclerosis who initially presented herself for research as a normal elderly volunteer


• To demonstrate the presence of early executive dysfunction in a case of hippocampal sclerosis


 

• W.H. was an 82 year old woman with a 14 year education who entered the Alzheimer’s Disease Research Registry as a normal control participant.  She was residing at an assisted living establishment where she was active in many recreational and administrative activities.
• Medical, functional and behavioral evaluations and a battery of tests measuring executive functioning, language, visuospatial ability, and primary and secondary memory were administered at entry and at annual visits following the standard protocol of the CWRU/UHC Alzheimer’s Disease Research Center.
  

• Results of the longitudinal neuropsychological evaluations are shown in Table 1. At initial evaluation mildly impaired scores were noted on Porteus Maze and Verbal Fluency tests and 1 of 4 delayed recall measures, while other scores were normal in those and other domains.
• Test scores fluctuated over the next three visits but most remained within normal limits and no clear pattern of decline was evident. In Figure 1. is shown the pattern of W.H.’s performance on tests of executive functioning.
• By the 5th follow-up visit, a further slight decline in executive and language scores was noted. By the 7th follow-up visit, W.H.’s executive functioning and primary and secondary memory scores were clearly impaired, and a dementia diagnosis was made. Verbal fluency scores were also impaired; picture naming and some visuospatial scores remained within a normal range. W.H.’s ability to actively function in her roles as volunteer cashier and exercise leader had also diminished, as had her i nitiation and enjoyment of leisure activities.
• Autopsy was performed 2 months after the 8th follow-up visit. At post mortem examination, hippocampal sclerosis was diagnosed.

Neuropsychological Test Scores	Entry	1st follow-up	2nd follow-up	3rd follow-up	4th follow-up	5th follow-up	6th follow-up	7th follow-up	8th follow-up
Porteus Mazes	12.5*	13	11.5*	11.5*	11*	11.5*		10**	8**
Plan of Search	9	13	9	9	9	9		7*	10
Tinkertoy		9	9	6*	10	9		7	5**
Judgment	7	6	7	6
Cognitive Estimation Unusual Responses	0		0	1	1	0		4***	6***
Trail-making B (/10)	15.4*	17.7**	20.6***	20.9***	30.0***	23.3***	24.4***	30.0***	30.0***
Controlled Word Retrieval - (FAS)	28	24	17*	19	26	16*		14*
CERAD Verbal Fluency (Animals)	17	9**	12*	19	10*	13*	12*	7**	4***
Picture Naming	37	39	35	39	39	38		37	39
CERAD Boston Naming	15	15	15	15	15	14	15	14	13*
Token Test (errors)	1		0	3***	2**	2**		2**
Visual Design Copy	31		31		32			29*
CERAD Constructional Praxis	11	9**	9**	10*	10*	10*	10*	9**	9**
Form Assembly	12		12	11	11	11		12	10*
Gollin Incomplete Picture Test	1.5	1.8	1.2	1.6	1.5	1.9		1.6	2.3*
Nonverbal Sequencing Span	15*	18	16	17	16	15*		15*	15*
Visual Verbal Sequencing Span	17	16	19	17	17	16		16	15*
Divided Attention Test	47	37**	38*	43	40	39*		29***
Easy Prose Immediate Recall	7.5	11.5	9.5	10	9	7*		5.5**	3***
Easy Prose Delayed Recall	7	8	4*	5*	7*	0***		2.5***	0***
CERAD Word List Memory	21	14	18	21	17	13**	12**	9***	6***
CERAD Word List Delayed Recall	4*	5	5	6	4*	4*	3**	1***	2**
CERAD Word List Recognition	20	19	20	20	19	19	19	18*	17**
Picture Naming Recall	10*	9*	11	13	11	11		9*	3**
CERAD Constructional Praxis Recall	11	7	5*	6*	7	8	3**	3**	2**
MMSE	28	29	28	28	25	26	23	19	20
CERAD Short Blessed	0	0	0	0	2	2	2	13	6.5
* 1 S.D. below age 76+ mean ** 2 S.D. below age 76+ mean *** 3 S.D. below age 76+ mean

DISCUSSION AND CONCLUSIONS

• In this case of hippocampal sclerosis, the full dementia syndrome was preceded for seven years by mild, fluctuating cognitive problems
• Signs of executive dysfunction were present prior to diagnosis, with disturbances of planning and working memory.