Herbert F. Durwen

the amygdala's contribution to memory—a study on two patients with Urbach-wiethe disease

The consequences of primary amygdaloid damage on memory performance are described in terms of neuropsychological, CT, MRI and PET results of two patients, a brother and a sister. Both had circumscribed, bilaterally symmetrical damage confined to the amygdaloid region, while the hippocampal formation and other brain structures were intact. PET-imaging furthermore revealed an overall decrease in glucose metabolism which was particularly apparent at the cingular and thalamic levels. Although neither patient was amnesic, both showed memory impairments in selective tests. In one patient these impairments were more pronounced and they were accompanied by marked affective-emotional fluctuations. Our results suggest that the amygdaloid region is a bottle-neck structure that confers an affective flavour to memories, thereby enhancing the probability of their long term storage.

Retrograde amnesia after traumatic injury of the fronto-temporal cortex.

An industrial manager had severe retrograde and variable but usually mild anterograde amnesia four years after a head injury. MRI showed damage of both temporal poles and the lateral portion of the right prefrontal cortex. The prefrontal and temporal cortical damage on the right side extended deeply into the white matter while the temporal cortical damage on the left side was much smaller. There was an additional left temporo-parietal lesion. The patient was of average intelligence. His attention, short term memory and learning ability were average or somewhat below average. His old memories were severely affected for the personal-episodic domain and less so for semantic remote memory abilities. Therefore an anatomical dissociation between anterograde and retrograde amnesia is possible at the anterior temporal regions, possibly interacting with the prefrontal cortex; these regions seem necessary for the retrieval of old episodic memories.

Right temporofrontal cortex as critical locus for the ecphory of old episodic memories.

A 54 year old patient of average intelligence with a severe and enduring loss of old autobiographical memories after herpes simplex type 1 infection is described. She was tested with a comprehensive neuropsychological battery two years after the infection. Special emphasis was laid on examining different aspects of retrograde memory. The neurological examination involved MRI and SPECT. Brain damage was found mainly in the right temporofrontal region, but minor left sided damage to this region seems possible. The patient was in the normal or slightly subnormal range for all tested anterograde memory functions, but manifested severe retrograde memory deficits with respect to episodic old memories and more moderate deficits in tests of general knowledge (semantic old memories). It is concluded that the ecphory of old autobiographical memories relies heavily on an activation of the right lateral temporofrontal junction area, but that probably only some complementary left hemispheric damage to these regions will lead to major and persistent retrograde amnesia. Alternatively, the disconnection between major prefrontal and posterior cortical regions may provide a basis for retrograde amnesia.

Searching for the anatomical basis of retrograde amnesia

Abstract The case of a patient with profound retrograde and minor anterograde amnesia is described and used to discuss the kind of brain damage which will most likely result in persistent retrograde amnesia as the principal symptom. The patient was an industrial manager who had fallen off a horse four years prior to the present neuropsychological and neuroradiological investigation. MRI examination revealed an injury to both temporal poles and to the latero-ventral portion of the right prefrontal cortex. The prefrontal and temporal cortical damage on the right side deeply invaded the white matter while the temporal cortical damage on the left side was much smaller; here, however, portions of the temporo-parietal transition zone were affected as well. The patient was of average intelligence. His attention, short-term memory, and learning ability were average or somewhat below average. His old memories were severely affected in the personal-episodic domain, and much less so in that of semantic remote memory...

Impaired episodic memory retrieval in a case of probable psychogenic amnesia

A patient with severe, selective retrograde amnesia for personal material diagnosed as probable psychogenic amnesia, was investigated intensively neuropsychologically with cranial computed tomography (CCT), magnetic resonance imaging (MRI), and single photon emission tomography (SPECT). The patient was of average intelligence and memory with no anterograde amnesia. No evidence for structural brain damage was detected in CCT and MRI. SPECT, performed about 3 weeks after the onset of symptoms, demonstrated reduced perfusion in right temporal and frontal areas, that is, in areas which have been suggested as critical for episodic memory retrieval. To study episodic memory retrieval, positron-emission-tomography (PET) blood flow (rCBF) measurements were performed 6 months after the onset of symptoms. During episodic memory retrieval bilateral neuronal activations were observed in the precuneus, the lateral parietal and the right dorsolateral and polar prefrontal cortex. Compared to the results of previous functional imaging studies on episodic memory retrieval, our findings suggest an underlying functional disturbance of brain areas previously demonstrated to be involved in episodic memory retrieval.

Retrograde amnesia for world knowledge and preserved memory for autobiographic events. A case report.

A patient (PC) with severe and chronic retrograde amnesia for world knowledge (tested with famous events and famous faces), but unimpaired autobiographical memory is described. The 64-year-old man had traumatic brain injury four years prior to the present evaluation. Current brain imaging showed principally damage involving the infero-lateral prefrontal and the lateral temporal regions of the left-hemisphere. PC was of average intelligence, had no depression and only minor language problems, but manifested some additional anterograde memory deficits and performed subaverage in various frontal lobe-sensitive tests. Patient PC represents one of the very few cases with a preserved retrograde episodic and an impaired retrograde knowledge system, showing a dissociation between preserved retrieval of autobiographical events and amnesia for nonpersonal famous events. It is hypothesized that the sparing of autobiographical memories can be linked to the integrity of the right frontal and temporo-polar cortices.

Left hemispheric neuronal heterotopia: A PET, MRI, EEG, and neuropsychological investigation of a university student

A 21-year-old left-handed medical student had a prominent unilateral cerebral cortical malformation due to an ontogenetic migration disorder. We performed neuropsychological studies, EEG, T1 and T2-weighted and proton-density MRI, and positron emission tomography (PET) (under both the resting condition and neuropsychological activation). Neuropsychological testing revealed normal intelligence and generally normal memory functioning but selective deficits in tests of verbal fluency and spatial-figural relationships. Proton-density and T2-weighted MRI revealed extensive left cortical heterotopia that included parts of the Wernicke area. PET under the resting condition revealed a small interhemispheric difference with slightly reduced glucose metabolism in the left temporoparietal cortical zone. An activation PET (with the patient performing a verbal fluency test) resulted in a normal overall increase in metabolism but marked deviations in cortical areas. The highest activity changes were in the Broca and Wernicke areas of the right hemisphere, and there was very little activation in those regions of the left hemisphere that were expected to respond well to the activation–the temporal, parietal, and temporo-occipital cortical zones. We conclude that there can be large compensations for unilateral heterotopia.

Ein Fall von Urbach-Wiethe-Erkrankung

Bei der sogenannten Urbach-Wiethe-Erkrankung handelt es sich um eine lipoide Proteinose, die erstmals im Jahre 1908 von Siebermann als generalisierte Hyper-keratose der Haut unter Einbeziehung der Mucosa beschrieben wurde. Die erste umfassende Darstellung dieses Krankheitsbildes stammt von Urbach und Wiethe aus dem Jahre 1929. Anfanglich galt die Erkrankung als umschriebene Dermatose mit zusatzlicher Beteiligung der oralen Mucosa und des Larynx. Im Laufe der Jahre wurde jedoch deutlich, das es sich bei diesem Syndrom um eine Multi-System-Er-krankung handelt, die zu Veranderungen an Augen, Magen, Rektum, Vagina, Leber, Pankreas und Gehirn fuhren kann. Es handelt sich um ein sehr seltenes und gutartiges, autosomal-rezessiv vererbtes Krankheitsbild.