Bal. Z Neurol. Sci. 13:593-596, 1992
Transient topographical amnesia Stracciari A.
Servizio di Neurologia, Policlinico S. Orsola-Malpighi, Bologna
A woman experienced three isolated episodes o f loss o f topographical memory, without overt etiology. A rare f o r m o f selective non verbal transient amnesia, similar to the more common transient global amnesia, is postulated.
Key Words: Amnesia - - topographical memory - - transient global amnesia
Introduction Topographical amnesia (TA) defines the loss of topographical orientation in familiar surroundings, due to failure to utilize known landmarks for finding the right direction [2, 5, 7]. Mostly observed in confusional states and in global dementias, it may occur selectively in right posterior hemisphere damage and is usually permanent. I observed a patient, who experienced three episodes of selective TA, without any other neurological or neuropsychological deficit, who recovered completely. There was no known or detectable etiology.
Case report A 64 year old healthy righthanded woman experienced two brief episodes of loss of orientation in known surroundings in 1987 and 1988. Each episode lasted a few minutes, during which the patient experienced the strange feeling of not being able to find her way, even though she was walking in familiar places. However, given the shortness of the episodes and their complete remission, the patient did not take any notice of them. In January 1990 she had a third, more prolonged attack, described as follows: "On Saturday in the afternoon, I went to the S. Pietro Cathedral [in Bologna] to hear mass. I entered the church from the main door. At the end of the mass I went out, passing through a side door, as I usually do, because the bus stop is not far from this exit. But when I left the church, I suddenly lost my way and turned left, not right, as I should. So I began to walk in the opposite direction, recognizing the l~'~.~ed 26 March 1992 - Accepted 30 May 1992
surroundings but not being able to orientate myself. I continued to walk for about 30 minutes, without finding my way, even though I knew the buildings and the streets around me. I was very worried about this. I asked someone for help and the person showed me the fight way, but, despite this, I could not find it. Finally, 30-40 minutes later, my disorientation began to clear up and I quickly grasped how to reach the fight direction". The patient was admitted one month after this episode. Apart from the abovementioned attacks, her medical history was unremarkable. General and neurological examination was normal. EEG, cerebrovascular ultrasound tomography, cerebral CT and MRI were unimpressive. A 99m Tc-HMPAO cerebral SPECT revealed mild diffuse abnormalities, more pronounced on the right occipital region (Fig. 1). Neuropsychological examination (Table 1) ruled out mental deterioration (Brief Mental Deterioration Battery Index [9] = 0.94). Memory was assessed by means of digit and spatial span, verbal and spatial learning, Rey Auditory 15 Words, Rey Complex Figure, Immediate and Delayed Visual Memory, Story Recall, Paired Associate Learning, Serial Position Curve (for details see [5, 9, 14]). These gave normal results, except for a slight delay on verbal and spatial learning and a flat first trait of the Serial Position Curve. The Elithorn Perceptual Maze test was within normal range [14]. On visual maze learning test [5, 7] the criterion was reached after 13 attempts. Streets and buildings on a map were located correctly except for one error of right/left inversion. The mental representation of real itineraries was spared. No further episodes or other neurological disturb593
The halian Journal of Neurological Sciences
ances were reported during a year of follow-up. A cerebral SPECT performed 12 months later was unchanged. Discussion
Fig. 1. Horizontal slices ~/' SPECT study. Descending brain cuts. Right hemisphere appears on the left side. The arrow indicates the right occipital hypoperfusion.
This woman experienced three episodes of selective loss of topographical orientation, not accompanied by a disorder of consciousness, global amnesia, or other neurological disturbances. The loss of topographical orientation, usually characterized by defective route finding, is a disorder known for a century [15], which has been given a number of names in the literature, including: 'topographical amnesia', 'pure topographical disorientation', 'spatial disorientation', 'visual disorientation', 'loss of topographical familiarity', 'topographical agnosia', [1-3, 6, 7, 10, 12]. This reflects the difficulty of attributing the disorder to a single, definite pathogenetic mechanism. In general, disorders of spatial orientation are related
TABLE 1. N e u r o p s y c h o l o g i c a l testing. Case
Normal mean
BMDB* [ 9 ] Rey's 15 Words Barrage
Visual Memory Simple Analogies Final result OTHER TESTS [5, 14] Digit span
- - short term - - long term - - time - - score - - errors - - immediate - - delayed
- - forward - - backward Supra-span Verbal Learning (digits)/span Corsi's Cube Supra-span Spatial Learning/span Rey's Complex Figure - - copy - - recall Paired Associated Learning Story Recall - - immediate - - delayed Serial Position Curve - - primacy effect - - recency effect Visual M~.e Learning Elithorn's Perceptual Maze
39 7 90 7 7 16 13 12 0:94 5 3 4.8 4 4.2
(SD)
41.9 (9.0) 8.6 (3.0) 64.4 (23.7) 11.7 (1.8) 0.6 (1.2) 19.6 (2.5) 11.6 (2.7) 15.4 (3.4) 2.0 (0.7) 5.6 3.9 2.6 4.9 1.9
(6.9) (1.0) (1.6) (0.8) (1.2)
32 10 11 6 10
30.0 15.0 13.8 11.4 13.2
(5.2) (6.7) (2.6) (3.6) (5.0)
1 14 13 21
8.4 (4.5) 13.8 (4.4) 13.6 26.2 (5.1)
Brief Mental Deterioration Battery [9] ** Personal data except for Visual Maze Learning test [5] and Elithorn's Perceptual Maze test [14]
594
values**
Stracciari A.: Transient topographical amnesia
to defects of exploration, perception, cognition and memory [6]. A specific defect of topographical orientation is usually ascribed to two main mechanisms [1, 3, 6, 10, 11]: 1) a visual-perceptual deficit (topographical agnosia) and 2) a m n e s i c deficit (topographical amnesia). The former is said to be characterized by failure to recognize familiar places, while a deficit of topographical memory refers to a situation in which the patient loss his/her way in a well known environment, being able to recognize buildings and places, but not to utilize them as useful landmarks to orient himself. This seems to have occurred in my patient. Concerning the anatomical sites crucial for this disorder, topographical memory appears to be a function of the right hemisphere and in particular the hippocampal system, the occipital lobe and the posterior parietal lobe [6]. Usually TA is accompanied by other neurological and neuropsychological signs and symptoms, resulting in permanent deficits. Occasionally, episodes of temporary loss of topographical memory have been reported. In 1981 Aimard et al. [1] reported on two patients with transient topographical disorientation (obs. 1 and 2), with accompanying symptoms such as visual field deficit (both), planotopokinesia and constructional apraxia (case 2), language disorder and prosopagnosia (case 1). An ischemic etiology was postulated. In 1983 Moretti et al. [13] reported the case of a woman who experienced three episodes of pure transient TA, supposed to be caused by a transient ischemia of the right retrorolandic area; they raised the question of transient selective amnesias as partial forms of transient global amnesia (TGA). This is a well recognized clinical entity, so named by Fisher and Adams in 1964 [8], still of unknown nature, attributed to a transient bilateral hippocampal dysfunction. It is a temporary memory disorder, consisting in a sudden anterograde amnesia, with repetitive quer-
ies, accompanied by a retrograde amnesia of variable duration, without any other neurological or neuropsychological signs or symptoms. In TGA, the term global defines a deficit covering all modalities of memory (verbal, spatial, visual, etc.). Others have postulated the possibility of selective transient amnesias as disorders close to TGA. In 1986, Damasio et al. reported a case of selective transient amnesia confined to verbal material, with sparing of non verbal memory, named transient partial amnesia (TPA) [4]. They attributed this event to a temporary dysfunction of the left rostral temporal lobe, with normal functioning of the right. My case resembles that one, the non verbal memory being impaired and the verbal memory spared and may reasonably be defined as TPA with selective impairment of topographical memory, or transient topographical amnesia (TTA). A transient blackout of the right hemisphere structures crucial for memory is a possible explanation. Given the absence of evident etiology, the transience of the episodes, the good outcome with only a mild residual memory 'frailty' detected by n e u r o p s y c h o l o g i c a l examination, TTA should be considered very close to TGA. Even TGA is probably not so 'global', because some aspects of memory (remote memory, procedural memory) are spared. Why selective transient amnesias are so rarely reported compared to the more common TGA is not clear. One may suppose that little attention is paid to this disorder by patients ot physicians, who consider it not dangerous, a bizarre psychic phenomenon, as probably occurred in the past for TGA. A more careful search for such episodes, in the wide context of confusional/amnesic states in the elderly, may disclose similar cases more frequently and reveal whether they are really partial forms of TGA, with the same characteristics of benignity and good prognosis.
Acknowledgment: I am indebted to Professor Anna Mazzucchi, who first critically read the manuscript, encouraging its publication.
Sommario Viene riportato il caso di una paziente che ha presentato tre episodi di perdita transitoria della memoria topografica, senza una chiara eziologia. Si ipotizza una rara forma di amnesia transitoria selettiva non verbale, analoga alla piit comune amnesia globale transitoria.
Address reprint requests to: Dr. Andrea Stracciari Servizio di Neurologia Policlinico S. Orsola-Malpighi via Albertoni, 15 - 40138 Bologna. 595
The Italian Journal of Neurological Sciences
References [1] AJMARD G., VIGHETTO A., CONFAVREUX C., DEVIC M.: La d~sorientation spatiale. Rev. Neurol. (Paris) 137, 2:97-111, 1981. [2] BENTON A.L.: Disorders of spatial orientation. In: Handbook of Clinical Neurology, vol. 3, Vinken PJ and Bruyn GM, eds., North Holland Publishing Company, Amsterdam, pp. 212-228, 1969. [3] BRAIN W.R.: Visual disorientation with special reference to lesions of the right cerebral hemisphere. Brain 64:244-272, 1941. [4] DAMAS!O A.R., GRAFF-RADFORD N.R., DAMASIO H.: Transient partial amnesia. Arch. Neurol. 40:656-657, 1983. [5] DE RENZI C.: Le amnesie. In: Neuropsicologia Clinica, Franco Angeli editore, Milano, pp. 199246, 1980. [6] DE RENZl E.: Disorders of spatial orientation. In: Handbook of Clinical Neurology, vol. 1 (45): Clinical Neuropsychology, JAM Frederiks, ed., Flsevier Science Publishers, Amsterdam, pp. 405422, 1985. [7] DE RENZ~ E., FAGLIOM P., VILLA: Topographical amnesia. J. Neurol. Neurosurg. Psychiatry 40:498505, 1977.
596
[8] FISHER C.M., ADAMS V.: Transient global amnesia. Acta Neurol. Scand. 40 (suppl. 9):1-83, 1964. [9] GALLASSI R., LENZI P., STRACCIARI A., et al.: Neuropsychological assessment of mental deterioration: purpose of a brief batter 3, and a probabilistic definition of 'normalio,' and 'non-normality'. Acta Psychiatr. Scand. 74:62-67, 1986. [10] HABIB M., SIRIGU A.: Pure topographical disorientation: a definition and anatomical basis. Cortex 23:73-85, 1987. [11] KLEIST K.: Gehirnpathologie. Barth, Leipzig, 1934. [12] LANDIS T., CUMMINGS J.L., BENSON D.F., PALMER E.P.: Loss of topographic familiariO,. An environmental agnosia. Arch. Neurol. 43:132-136, 1986. [13] MORETTI G., CAFFARRA P., PARMA M.: Transient topographical amnesia. Ital. J. Neurol. Sci. 3:361, 1983. [14] SPINNLER H., TOGNON! G.: eds. Standardizzazione e taratura di test neuropsicologici. Ital. J. Neurol. Sci. (suppl. 8), 1987. [15] WILBRAND H.: Ein Fall yon Seelenblindheit und Hemianopsia mit Sectionsbefund. Dtsch. Z. Nervenheilkd. 2:361-387, 1892.
Transient topographical amnesia Stracciari A.
Servizio di Neurologia, Policlinico S. Orsola-Malpighi, Bologna
A woman experienced three isolated episodes o f loss o f topographical memory, without overt etiology. A rare f o r m o f selective non verbal transient amnesia, similar to the more common transient global amnesia, is postulated.
Key Words: Amnesia - - topographical memory - - transient global amnesia
Introduction Topographical amnesia (TA) defines the loss of topographical orientation in familiar surroundings, due to failure to utilize known landmarks for finding the right direction [2, 5, 7]. Mostly observed in confusional states and in global dementias, it may occur selectively in right posterior hemisphere damage and is usually permanent. I observed a patient, who experienced three episodes of selective TA, without any other neurological or neuropsychological deficit, who recovered completely. There was no known or detectable etiology.
Case report A 64 year old healthy righthanded woman experienced two brief episodes of loss of orientation in known surroundings in 1987 and 1988. Each episode lasted a few minutes, during which the patient experienced the strange feeling of not being able to find her way, even though she was walking in familiar places. However, given the shortness of the episodes and their complete remission, the patient did not take any notice of them. In January 1990 she had a third, more prolonged attack, described as follows: "On Saturday in the afternoon, I went to the S. Pietro Cathedral [in Bologna] to hear mass. I entered the church from the main door. At the end of the mass I went out, passing through a side door, as I usually do, because the bus stop is not far from this exit. But when I left the church, I suddenly lost my way and turned left, not right, as I should. So I began to walk in the opposite direction, recognizing the l~'~.~ed 26 March 1992 - Accepted 30 May 1992
surroundings but not being able to orientate myself. I continued to walk for about 30 minutes, without finding my way, even though I knew the buildings and the streets around me. I was very worried about this. I asked someone for help and the person showed me the fight way, but, despite this, I could not find it. Finally, 30-40 minutes later, my disorientation began to clear up and I quickly grasped how to reach the fight direction". The patient was admitted one month after this episode. Apart from the abovementioned attacks, her medical history was unremarkable. General and neurological examination was normal. EEG, cerebrovascular ultrasound tomography, cerebral CT and MRI were unimpressive. A 99m Tc-HMPAO cerebral SPECT revealed mild diffuse abnormalities, more pronounced on the right occipital region (Fig. 1). Neuropsychological examination (Table 1) ruled out mental deterioration (Brief Mental Deterioration Battery Index [9] = 0.94). Memory was assessed by means of digit and spatial span, verbal and spatial learning, Rey Auditory 15 Words, Rey Complex Figure, Immediate and Delayed Visual Memory, Story Recall, Paired Associate Learning, Serial Position Curve (for details see [5, 9, 14]). These gave normal results, except for a slight delay on verbal and spatial learning and a flat first trait of the Serial Position Curve. The Elithorn Perceptual Maze test was within normal range [14]. On visual maze learning test [5, 7] the criterion was reached after 13 attempts. Streets and buildings on a map were located correctly except for one error of right/left inversion. The mental representation of real itineraries was spared. No further episodes or other neurological disturb593
The halian Journal of Neurological Sciences
ances were reported during a year of follow-up. A cerebral SPECT performed 12 months later was unchanged. Discussion
Fig. 1. Horizontal slices ~/' SPECT study. Descending brain cuts. Right hemisphere appears on the left side. The arrow indicates the right occipital hypoperfusion.
This woman experienced three episodes of selective loss of topographical orientation, not accompanied by a disorder of consciousness, global amnesia, or other neurological disturbances. The loss of topographical orientation, usually characterized by defective route finding, is a disorder known for a century [15], which has been given a number of names in the literature, including: 'topographical amnesia', 'pure topographical disorientation', 'spatial disorientation', 'visual disorientation', 'loss of topographical familiarity', 'topographical agnosia', [1-3, 6, 7, 10, 12]. This reflects the difficulty of attributing the disorder to a single, definite pathogenetic mechanism. In general, disorders of spatial orientation are related
TABLE 1. N e u r o p s y c h o l o g i c a l testing. Case
Normal mean
BMDB* [ 9 ] Rey's 15 Words Barrage
Visual Memory Simple Analogies Final result OTHER TESTS [5, 14] Digit span
- - short term - - long term - - time - - score - - errors - - immediate - - delayed
- - forward - - backward Supra-span Verbal Learning (digits)/span Corsi's Cube Supra-span Spatial Learning/span Rey's Complex Figure - - copy - - recall Paired Associated Learning Story Recall - - immediate - - delayed Serial Position Curve - - primacy effect - - recency effect Visual M~.e Learning Elithorn's Perceptual Maze
39 7 90 7 7 16 13 12 0:94 5 3 4.8 4 4.2
(SD)
41.9 (9.0) 8.6 (3.0) 64.4 (23.7) 11.7 (1.8) 0.6 (1.2) 19.6 (2.5) 11.6 (2.7) 15.4 (3.4) 2.0 (0.7) 5.6 3.9 2.6 4.9 1.9
(6.9) (1.0) (1.6) (0.8) (1.2)
32 10 11 6 10
30.0 15.0 13.8 11.4 13.2
(5.2) (6.7) (2.6) (3.6) (5.0)
1 14 13 21
8.4 (4.5) 13.8 (4.4) 13.6 26.2 (5.1)
Brief Mental Deterioration Battery [9] ** Personal data except for Visual Maze Learning test [5] and Elithorn's Perceptual Maze test [14]
594
values**
Stracciari A.: Transient topographical amnesia
to defects of exploration, perception, cognition and memory [6]. A specific defect of topographical orientation is usually ascribed to two main mechanisms [1, 3, 6, 10, 11]: 1) a visual-perceptual deficit (topographical agnosia) and 2) a m n e s i c deficit (topographical amnesia). The former is said to be characterized by failure to recognize familiar places, while a deficit of topographical memory refers to a situation in which the patient loss his/her way in a well known environment, being able to recognize buildings and places, but not to utilize them as useful landmarks to orient himself. This seems to have occurred in my patient. Concerning the anatomical sites crucial for this disorder, topographical memory appears to be a function of the right hemisphere and in particular the hippocampal system, the occipital lobe and the posterior parietal lobe [6]. Usually TA is accompanied by other neurological and neuropsychological signs and symptoms, resulting in permanent deficits. Occasionally, episodes of temporary loss of topographical memory have been reported. In 1981 Aimard et al. [1] reported on two patients with transient topographical disorientation (obs. 1 and 2), with accompanying symptoms such as visual field deficit (both), planotopokinesia and constructional apraxia (case 2), language disorder and prosopagnosia (case 1). An ischemic etiology was postulated. In 1983 Moretti et al. [13] reported the case of a woman who experienced three episodes of pure transient TA, supposed to be caused by a transient ischemia of the right retrorolandic area; they raised the question of transient selective amnesias as partial forms of transient global amnesia (TGA). This is a well recognized clinical entity, so named by Fisher and Adams in 1964 [8], still of unknown nature, attributed to a transient bilateral hippocampal dysfunction. It is a temporary memory disorder, consisting in a sudden anterograde amnesia, with repetitive quer-
ies, accompanied by a retrograde amnesia of variable duration, without any other neurological or neuropsychological signs or symptoms. In TGA, the term global defines a deficit covering all modalities of memory (verbal, spatial, visual, etc.). Others have postulated the possibility of selective transient amnesias as disorders close to TGA. In 1986, Damasio et al. reported a case of selective transient amnesia confined to verbal material, with sparing of non verbal memory, named transient partial amnesia (TPA) [4]. They attributed this event to a temporary dysfunction of the left rostral temporal lobe, with normal functioning of the right. My case resembles that one, the non verbal memory being impaired and the verbal memory spared and may reasonably be defined as TPA with selective impairment of topographical memory, or transient topographical amnesia (TTA). A transient blackout of the right hemisphere structures crucial for memory is a possible explanation. Given the absence of evident etiology, the transience of the episodes, the good outcome with only a mild residual memory 'frailty' detected by n e u r o p s y c h o l o g i c a l examination, TTA should be considered very close to TGA. Even TGA is probably not so 'global', because some aspects of memory (remote memory, procedural memory) are spared. Why selective transient amnesias are so rarely reported compared to the more common TGA is not clear. One may suppose that little attention is paid to this disorder by patients ot physicians, who consider it not dangerous, a bizarre psychic phenomenon, as probably occurred in the past for TGA. A more careful search for such episodes, in the wide context of confusional/amnesic states in the elderly, may disclose similar cases more frequently and reveal whether they are really partial forms of TGA, with the same characteristics of benignity and good prognosis.
Acknowledgment: I am indebted to Professor Anna Mazzucchi, who first critically read the manuscript, encouraging its publication.
Sommario Viene riportato il caso di una paziente che ha presentato tre episodi di perdita transitoria della memoria topografica, senza una chiara eziologia. Si ipotizza una rara forma di amnesia transitoria selettiva non verbale, analoga alla piit comune amnesia globale transitoria.
Address reprint requests to: Dr. Andrea Stracciari Servizio di Neurologia Policlinico S. Orsola-Malpighi via Albertoni, 15 - 40138 Bologna. 595
The Italian Journal of Neurological Sciences
References [1] AJMARD G., VIGHETTO A., CONFAVREUX C., DEVIC M.: La d~sorientation spatiale. Rev. Neurol. (Paris) 137, 2:97-111, 1981. [2] BENTON A.L.: Disorders of spatial orientation. In: Handbook of Clinical Neurology, vol. 3, Vinken PJ and Bruyn GM, eds., North Holland Publishing Company, Amsterdam, pp. 212-228, 1969. [3] BRAIN W.R.: Visual disorientation with special reference to lesions of the right cerebral hemisphere. Brain 64:244-272, 1941. [4] DAMAS!O A.R., GRAFF-RADFORD N.R., DAMASIO H.: Transient partial amnesia. Arch. Neurol. 40:656-657, 1983. [5] DE RENZI C.: Le amnesie. In: Neuropsicologia Clinica, Franco Angeli editore, Milano, pp. 199246, 1980. [6] DE RENZl E.: Disorders of spatial orientation. In: Handbook of Clinical Neurology, vol. 1 (45): Clinical Neuropsychology, JAM Frederiks, ed., Flsevier Science Publishers, Amsterdam, pp. 405422, 1985. [7] DE RENZ~ E., FAGLIOM P., VILLA: Topographical amnesia. J. Neurol. Neurosurg. Psychiatry 40:498505, 1977.
596
[8] FISHER C.M., ADAMS V.: Transient global amnesia. Acta Neurol. Scand. 40 (suppl. 9):1-83, 1964. [9] GALLASSI R., LENZI P., STRACCIARI A., et al.: Neuropsychological assessment of mental deterioration: purpose of a brief batter 3, and a probabilistic definition of 'normalio,' and 'non-normality'. Acta Psychiatr. Scand. 74:62-67, 1986. [10] HABIB M., SIRIGU A.: Pure topographical disorientation: a definition and anatomical basis. Cortex 23:73-85, 1987. [11] KLEIST K.: Gehirnpathologie. Barth, Leipzig, 1934. [12] LANDIS T., CUMMINGS J.L., BENSON D.F., PALMER E.P.: Loss of topographic familiariO,. An environmental agnosia. Arch. Neurol. 43:132-136, 1986. [13] MORETTI G., CAFFARRA P., PARMA M.: Transient topographical amnesia. Ital. J. Neurol. Sci. 3:361, 1983. [14] SPINNLER H., TOGNON! G.: eds. Standardizzazione e taratura di test neuropsicologici. Ital. J. Neurol. Sci. (suppl. 8), 1987. [15] WILBRAND H.: Ein Fall yon Seelenblindheit und Hemianopsia mit Sectionsbefund. Dtsch. Z. Nervenheilkd. 2:361-387, 1892.
