Severe amnesia following bilateral medial temporal lobe damage occurring on two distinct occasions

Abstract A comprehensive neuropsychological assessment was performed on a 38-year-old woman with drug-resistant right temporal lobe epilepsy before temporal lobectomy, during a 2-year follow-up period, and approximately 3 years after surgery when she developed a malignant glioma in the left medial temporal lobe (MTL). Both before and after epilepsy surgery, memory function was normal. When the tumour was discovered, the patient suffered from severe retrograde and anterograde amnesia, whereas working memory and the other cognitive abilities were preserved. Compared with other cases of bilateral temporal lesion, this case is peculiar because the damage occurred on two distinct occasions. It suggests that only one MTL can allow normal memory function, or can take over the function normally subserved by a dysfunctional contralateral MTL when the dysfunction is marked and prolonged, such as in chronic epilepsy.     

Leukoencephalopathy with bilateral anterior temporal lobe cysts

Recently, several reports describing patients with a nonprogressive clinical course, increased signal in the cerebral white matter, and cystic changes in the anterior temporal lobes on magnetic resonance imaging (MRI) have appeared. To date, 25 patients with this very distinctive condition have been described. We report four new cases of this newly recognized entity. All have been identified primarily because of the distinctive MRI features consisting of the very unusual anterior temporal lobe cystic changes. The clinical features are characterized by severe, disabling, but nonprogressive mental and motor retardation. Magnetic resonance spectroscopy has shown increased myo-inositol and decreased N-acetylaspartate in the cerebral white matter. This is a distinctive, probably genetic, condition with characteristic neuroimaging and clinical features. In the appropriate clinical situation, the neuroimaging features are diagnostic.     

Outcome after temporal lobectomy in bilateral temporal lobe epilepsy

We determined how noninvasive presurgical data relate to prognosis after temporal lobectomy in patients with independent bilateral temporal lobe (IBTL) complex partial seizures on the intracranial electroencephalogram (EEG). Between 1986 and 1994, 28 patients had IBTL seizures on intracranial EEG. Fifteen of these 28 patients underwent temporal lobectomy and 13 were not offered surgery. Of the 15 patients who had surgery, 10 patients became seizure-free. Magnetic resonance imaging (MRI) and the Wada test were the only variables associated with a seizure-free outcome. Seven of 10 seizure-free patients had a lateralized Wada result or the presence of unilateral hippocampal sclerosis, whereas none of the patients with persistent seizures had either of these findings. Variables not found to be predictive of a seizure-free outcome included location of scalp interictal spikes, degree of seizure-onset laterality, presence of early epilepsy risk factor, duration of epilepsy, and full-scale intelligence quotient. We conclude that MRI and the Wada test provide information of prognostic value in patients with bilateral temporal seizures independent of intracranial EEG data.     

The nature of anterograde and retrograde memory impairment after damage to the medial temporal lobe

The study of anterograde and retrograde amnesia (AA and RA) in the laboratory and the clinic has provided important information about the structure and organization of memory. The severity of AA is usually correlated with the severity of RA. Nevertheless, variations in the expression of AA and RA have been reported, which presumably reflect variation in the locus and extent of brain damage. The relationship between AA and RA has rarely been described quantitatively in groups of patients where detailed anatomical information is available. We have quantified the severity of AA and RA for factual information in 11 memory-impaired patients with bilateral medial temporal lobe lesions, including 5 for whom detailed post-mortem neurohistological information was available. The findings describe an orderly relationship between AA and RA, such that patients with more severe AA also had more extensive RA. In addition, RA was measurable only after AA reached a substantial level of severity. This relationship between AA and RA in patients with identified medial temporal lobe lesions appears to describe a general principle, which applies to a range of etiologies, including traumatic amnesia, where the locus and extent of brain damage is less well understood. Whenever patients deviate substantially from the relationship described here, one should be alert to the likelihood that significant damage has occurred outside or in addition to the structures in the medial temporal lobe.     

Implicit representations of space after bilateral parietal lobe damage.

There is substantial evidence that the primate cortex is grossly divided into two functional streams, an occipital-parietal-frontal pathway that processes "where" and an occipital-temporal-frontal pathway that processes "what" (Ungerleider and Mishkin, 1982). In humans, bilateral occipital-parietal damage results in severe spatial deficits and a neuropsychological disorder known as Balint's syndrome in which a single object can be perceived (simultanagnosia) but its location is unknown (Balint, 1995). The data reported here demonstrate that spatial information for visual features that cannot be explicitly located is represented normally below the level of spatial awareness even with large occipital-parietal lesions. They also demonstrate that parietal damage does not affect preattentive spatial coding of feature locations or complex spatial relationships between parts of a stimulus despite explicit spatial deficits and simultanagnosia.     

Severe amnesia following a unilateral temporal lobe stroke.

A 60 year old right-handed man developed severe amnesia following a left medial temporal stroke as documented by cerebral MRI, MRA and SPECT scans. Neuropsychological evaluation 13 weeks after the stroke showed a profound retrograde amnesia characterised by memory loss for public facts and events over the previous four decades. In addition, autobiographical memory showed selective loss of personal episodic memory with relative preservation of personal semantic memory. The development of this degree of amnesia with these features following a unilateral temporal lobe lesion is unusual. The possible neuroanatomical mechanisms underlying the amnesia and how they relate to current theories of memory loss are discussed.     

Differential contributions of bilateral ventral anterior temporal lobe and left anterior superior temporal gyrus to semantic processes

Studies of semantic dementia and repetitive TMS have suggested that the bilateral anterior temporal lobes (ATLs) underpin a modality-invariant representational hub within the semantic system. However, it is not clear whether all ATL subregions contribute in the same way. We utilized distortion-corrected fMRI to investigate the pattern of activation in the left and right ATL when participants performed a semantic decision task on auditory words, environmental sounds, or pictures. This showed that the ATL is not functionally homogeneous but is more graded. Both left and right ventral ATL (vATL) responded to all modalities in keeping with the notion that this region underpins multimodality semantic processing. In addition, there were graded differences across the hemispheres. Semantic processing of both picture and environmental sound stimuli was associated with equivalent bilateral vATL activation, whereas auditory words generated greater activation in left than right vATL. This graded specialization for auditory stimuli would appear to reflect the input from the left superior ATL, which responded solely to semantic decisions on the basis of spoken words and environmental sounds, suggesting that this region is specialized to auditory stimuli. A final noteworthy result was that these regions were activated for domain level decisions to singly presented stimuli, which appears to be incompatible with the hypotheses that the ATL is dedicated (a) to the representation of specific entities or (b) for combinatorial semantic processes.     

Profound amnesia after damage to the medial temporal lobe: A neuroanatomical and neuropsychological profile of patient E. P.

E. P. became profoundly amnesic in 1992 after viral encephalitis, which damaged his medial temporal lobe bilaterally. Because of the rarity of such patients, we have performed a detailed neuroanatomical analysis of E. P.'s lesion using magnetic resonance imaging, and we have assessed his cognitive abilities with a wide range of neuropsychological tests. Finally, we have compared and contrasted the findings for E. P. with the noted amnesic patient H.M, whose surgical lesion is strikingly similar to E. P.'s lesion.     

Volumetric evidence of bilateral damage in unilateral mesial temporal lobe epilepsy

PURPOSE: We sought to analyze the contralateral volumes of the temporal pole, posterior segment of the temporal lobe, amygdala, hippocampus, and parahippocampal gyrus in patients with temporal lobe epilepsy (TLE) due to histologically proven mesial temporal lobe sclerosis (MTLS), seizure free for >or=4 years of postsurgical follow-up. METHODS: Forty-six (23 male) TLE patients, operated on between 1996 and 2001, with histopathologic diagnosis of MTLS, and a postsurgical follow-up of >or=4 years, had their temporal lobe structures manually segmented, measured, and compared with those of 23 normal volunteers, paired as groups for sex, age, and handedness. RESULTS: The mean volumes of the contralateral temporal pole, hippocampus, and parahippocampal gyrus in TLE patients were significantly lower than those in controls. CONCLUSIONS: MRI volumetric data show that the damage in TLE due to MTS may be more widespread and bilateral, even in patients with unilateral TLE by clinical and neurophysiological criteria. Our results are relevant to the discussion of epileptogenic mechanisms in TLE.     

Psychiatric aspects of temporal lobe epilepsy before and after anterior temporal lobectomy

OBJECTIVES: Psychopathology has been reported to be prevalent both before and after surgical treatment for medically intractable temporal lobe epilepsy. Individual patients were evaluated prospectively to assess the effect of anterior temporal lobectomy (ATL) on prevalence and severity of psychiatric disease. METHODS: Psychiatric status was assessed in a consecutive series of epilepsy patients before and 6 months after ATL using a structured psychiatric interview, psychiatric rating scales, and self report mood measures. RESULTS: A DSM-III-R axis I diagnosis was present in 65% of patients before and after surgery. The most common diagnoses were depression, anxiety, and organic mood/personality disorders. There was a trend for major psychiatric diagnoses to be more common in patients with right compared to left temporal lobe seizure focus, both before and after surgery. The apparent stability in the overall rate of psychiatric dysfunction concealed onset of new psychiatric problems in 31% of patients in the months shortly after surgery, and resolution of psychiatric diagnoses in 15% of patients. In the group as a whole, the severity of psychiatric symptoms was lower at 6 months postsurgery than before temporal lobectomy. CONCLUSIONS: The overall prevalence of psychiatric dysfunction was comparably high before and after ATL, but individual changes in psychiatric status and changes in severity of symptoms occurred in many patients in the 6 months after surgery.     

Fractionation of memory in medial temporal lobe amnesia

We report a comprehensive investigation of the anterograde memory functions of two patients with memory impairments (RH and JC). RH had neuroradiological evidence of apparently selective right-sided hippocampal damage and an intact cognitive profile apart from selective memory impairments. JC, had neuroradiological evidence of bilateral hippocampal damage following anoxia due to cardiac arrest. He had anomic and "executive" difficulties in addition to a global amnesia, suggesting atrophy extending beyond hippocampal regions. Their performance is compared with that of a previously reported hippocampal amnesic patient who showed preserved recollection and familiarity for faces in the context of severe verbal and topographical memory impairment [VC; Cipolotti, L., Bird, C., Good, T., Macmanus, D., Rudge, P., & Shallice, T. (2006). Recollection and familiarity in dense hippocampal amnesia: A case study. Neuropsychologia, 44, 489-506.] The patients were administered experimental tests using verbal (words) and two types of non-verbal materials (faces and buildings). Receiver operating characteristic analyses were used to estimate the contribution of recollection and familiarity to recognition performance on the experimental tests. RH had preserved verbal recognition memory. Interestingly, her face recognition memory was also spared, whilst topographical recognition memory was impaired. JC was impaired for all types of verbal and non-verbal materials. In both patients, deficits in recollection were invariably associated with deficits in familiarity. JC's data demonstrate the need for a comprehensive cognitive investigation in patients with apparently selective hippocampal damage following anoxia. The data from RH suggest that the right hippocampus is necessary for recollection and familiarity for topographical materials, whilst the left hippocampus is sufficient to underpin these processes for at least some types of verbal materials. Face recognition memory may be adequately subserved by areas outside of the hippocampus.     

The temporal pattern of retrograde amnesia in Korsakoff's disease

A multiple-choice questionnaire about well-known public events of the past 50 years, grouped with respect to time, was administered to 11 alcoholic patients with Korsakoff's disease and to 50 normal individuals of comparable age and background. Normal persons perform quite well on questions of the recent past and fall off only slightly when tested for more remote memories. In contrast, patients with Korsakoff's disease do very poorly in remembering recent events but improve to normal levels when memories become more remote. This confirms classical clinical observation and raises some important points about the mechanism of memory.     

Acquired hippocampal damage after temporal lobe seizures in 2 infants

Two infants developed unilateral hippocampal swelling on magnetic resonance imaging after prolonged seizures of temporal origin. Subsequent images suggested hippocampal sclerosis. The first child had febrile status epilepticus with exanthem subitum and developed refractory complex partial seizures. Histological findings after temporal lobectomy confirmed hippocampal sclerosis but also revealed sequelae of a focal encephalitis and microdysgenesis of the hippocampus. The second child had signs of brain dysgenesis, but acquired hippocampal damage affecting each side successively was documented by serial magnetic resonance imaging. These cases illustrate that different clinical conditions combining preexisting and acquired pathological characteristics can lead to hippocampal sclerosis.     

Factors related to successful antiepileptic drug withdrawal after anterior temporal lobectomy for medial temporal lobe epilepsy.

OBJECTIVE: To assess the rate of successful antiepileptic drug (AED) discontinuation, prognostic factors and proper time of AED withdrawal after surgery for medial temporal lobe epilepsy (MTLE). METHODS: We reviewed 171 consecutive patients who underwent resective surgery for MTLE. All patients were followed up for more than two postoperative years. AEDs were slowly tapered with an individualized schedule for each patient. Outcome status was determined from medical records and telephone interviews. RESULTS: 41.2% of patients experienced no seizure recurrence. 34.5% discontinued medication without seizure recurrence for more than 2 years at final assessment. Multivariate analysis revealed that an age greater than 30 years at surgery and postoperative AED reduction before 10 months increased the risk of recurrence [hazard ratio (HR) 2.1, 95% confidence interval (CI) 1.1-3.9 and HR 2.5, CI 1.1-5.8]. CONCLUSIONS: Resective surgery for MTLE brings seizure remission without AED to one-third of patients. Postoperative AED tapering is recommended after at least 10 months. Younger age at surgery is a good predictive factor of remission after MTLE surgery.     

Isolated prolonged retrograde amnesia.

We describe a patient who had isolated retrograde amnesia of 1-year duration without anterograde amnesia after recovery from encephalitis. Single photon emission computed tomography using 123I-IMP revealed that left temporal lobe abnormality. We postulate that the learning ability for new information and recalling ability for old information involve different neural structures, and there is a possible relationship between isolated retrograde amnesia and left temporal dysfunction.     

双侧颞叶癫(癎)的立体定向外科治疗

目的 探讨双侧海马-杏仁核复合体毁损术治疗双侧颞叶癫(癎)的疗效.方法 回顾性分析5例双侧颞叶癫(癎)病人的临床资料,复杂部分性发作中的自动症1例,部分性继发全身性癫(癎)4例.均行深部电极引导下机器人辅助定位双侧海马-杏仁核复合体毁损术.结果 随访1～2.5年,按Engel分级:Ⅰ级2例;Ⅱ、Ⅲ、Ⅳ级各1例.术后MRI显示:双侧海马-杏仁核复合体区无严重的结构性破坏.智商、心算速度、符号数字配对、划消、数字记忆广度、指扣试验等神经心理学检查指标手术前后差异均无统计学意义(P＞0.05).结论 双侧颞叶癫(癎)无法实施切除性手术,而立体定向外科治疗可减少癫(癎)发作,且并未造成严重认知功能障碍,是一种值得尝试的外科治疗手段.