Episodic memory in transient global amnesia.
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Biomedical subjects
Publications and source records attributed to A Z Zeman.
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OBJECTIVES: To clarify the clinical and neuropsychological aspects of transient epileptic amnesia (TEA) based on 10 personally studied cases as well as review of 21 previously published cases; and to propose tentative diagnostic criteria for the diagnosis of TEA. METHODS: All 10 patients and informants underwent a standardised clinical interview. The radiological and neurophysiological (EEG) data were also reviewed in all cases. The diagnosis of transient epileptic amnesia was made on the basis of the following criteria: (1) there was a history of recurrent witnessed episodes of transient amnesia; (2) cognitive functions other than memory were judged to be intact during typical episodes by a reliable witness; (3) there was evidence for a diagnosis of epilepsy. This evidence was provided by either (a) wake or sleep EEG, or (b) the co-occurrence of other seizure types (if their roughly concurrent onset or close association with episodes of transient amnesia suggested a connection), or (c) a clear cut response to anticonvulsant therapy, or by a combination of these three factors. In addition all patients were administered a comprehensive neuropsychological test battery designed to assess verbal and non-verbal anterograde memory and retrograde memory for famous personalities and personal events. Their results were compared with those of 25 age and IQ matched normal controls. RESULTS: TEA usually begins in later life, with a mean age of 65 years in this series. Episodes are typically brief, lasting less than one hour, and recurrent, with a mean frequency of three a year. Attacks on waking are characteristic. Repetitive questioning occurs commonly during attacks. The anterograde amnesia during episodes is, however, often incomplete so that patients may later be able to "remember not being able to remember". The extent of the retrograde amnesia during attacks varies from days to years. Most patients experience other seizure types compatible with an origin in the temporal lobes, but transient amnesia is the only manifestation of epilepsy in about one third of patients. Epileptiform abnormalities arising from the temporal lobes are most often detected on interictal sleep EEG. Despite normal performance on tests of anterograde memory, many patients complain of persistent interictal disturbance of autobiographical memory, involving a significant but variable loss of recall for salient personal episodes. The epochs affected may predate the onset of epilepsy by many years. CONCLUSIONS: TEA is an identifiable syndrome and comprises episodic transient amnesia with an epileptic basis, without impairment of other aspects of cognitive function. Future studies should consider the question of whether TEA reflects ictal activity or a postictal state, and the mechanism of the persistent autobiographical amnesia. It is hypothesised that the latter may result in part from impairment of very long term memory consolidation as a result of epileptic activity in mesial temporal structures.
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Synthesis of oligoclonal IgG within the central nervous system is a well established feature of multiple sclerosis. The occurrence of oligoclonal IgG in the serum of patients with multiple sclerosis has received little attention. We detected such a serum response in 20/45 consecutive patients (44%, 95% CI 30-59%) but in only 3/41 age- and sex-matched healthy controls (p < 0.01). We present qualitative and quantitative evidence that this oligoclonal IgG has a systemic origin. The plasma cell clones responsible for the serum response are often also represented in the intrathecal compartment. In a further study of the clinical significance of serum oligoclonal bands, in 80 patients, their presence was associated with elevated levels of intrathecal synthesis, increasing age, later disease onset and the presence of serum autoantibodies. These findings add to the evidence that there is a systemic immune disturbance in multiple sclerosis.
OBJECTIVES: To determine whether oligoclonal band (OCB) negative multiple sclerosis is a reliable diagnosis and, if so, whether it has a distinctive prognosis. METHODS: Retrospective and matched prospective comparison of the clinical and laboratory features of patients with clinical definite multiple sclerosis with and without intrathecal synthesis of oligoclonal IgG. RESULTS: Thirty four patients were identified with apparent OCB negative clinically definite multiple sclerosis. The results of oligoclonal banding proved to have been equivocal in 14 of 34; the clinical diagnosis of multiple sclerosis was questionable in 8 of 34. The remaining 12 patients with "true" OCB negative multiple sclerosis were significantly less disabled than matched OCB positive controls. Re-examination of CSF-serum pairs from six OCB negative patients showed that three remained OCB negative while three showed evidence of intrathecal synthesis of OCBs. CONCLUSIONS: OCB negative clinically definite multiple sclerosis is rare and should be diagnosed with caution; in unequivocal cases it seems to have a relatively benign prognosis.
We describe a case of lymphocytic meningitis following insertion of a porcine dermis implant to repair an operative dural defect. Histology of the excised implant revealed local abscess formation with a granulomatous reaction. Oligoclonal Immunoglobulin G, part of which could be removed by absorbtion with the porcine dermis, was present in the patient's cerebrospinal fluid, and, to a less marked degree, in his serum. The cerebrospinal fluid glucose was markedly depressed. An unusual hypersensitivity reaction to the porcine implant was considered the most likely explanation for this meningitic illness. The patient went on to make a full recovery following excision of the implant.
Seventy four patients with clinically definite multiple sclerosis were studied by using polyacrylamide gel electrophoresis of cerebrospinal fluid to assess blood-brain barrier function. Blood-brain barrier impairment was associated with recent clinical relapses of multiple sclerosis and worsened across a spectrum from the relapsing-remitting type of multiple sclerosis to secondary and primary progressive disease. The association between blood-brain barrier impairment and primary progressive disease is particularly interesting in the light of recent evidence that focal gadolinium enhancement on MRI is relatively unusual in patients with this disease.
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The diagnosis of CSF rhinorrhoea on clinical grounds alone can be difficult. We describe how the use of non-invasive electrophoretic analysis of nasal secretions for tau protein (asialotransferrin) helped in the management of cases where the existence of a CSF leak was in doubt. Patients were thus saved unnecessary invasive investigations or surgery. A modification of the method of analysis, which improves diagnostic accuracy, is described.
Transient global amnesia is an alarming but benign disorder of middle age, which prevents both the formation of new memories, and the retrieval of recently formed ones, for some hours. Research over the last 10 years has shed light on the epidemiology, neuropsychology, functional anatomy and differential diagnosis of this distinctive condition, and clarified its management.