Cognitive consequences of coexisting temporal lobe developmental malformations and hippocampal sclerosis.
Explore the source record for details and available documents.
Cognition Disorders↗
Biomedical subjects
R S Briellmann
Publications and source records attributed to R S Briellmann.
Occurrence of hippocampal sclerosis: is one hemisphere or gender more vulnerable?
PURPOSE: We analyzed a large group of patients investigated for suspected seizures to test whether gender or side are important factors in the origins of hippocampal sclerosis (HS). METHODS: We studied 996 consecutive patients (48% men, 52% women) by using standard hippocampal T2-relaxometry methods. RESULTS: HS was associated with a highly abnormal T2 time (< or =113 ms). Categoric analysis showed that hippocampal T2 time was independent of gender and side. T2 time was bilaterally normal in 81% of men and in 79% of women; it was unilaterally abnormal in 15% of both men and women; and bilaterally abnormal in 4% of men and in 6% of women. Highly abnormal T2 relaxometry, suggesting HS, occurred with equal frequency in men and women and on the right and left sides. Quantitative analysis of hippocampal T2 times showed values not differing significantly between men and women or between the right and left hemispheres. There was no significant interaction between gender and side. CONCLUSIONS: In patients with seizure disorders, hippocampal T2 relaxometry is not different in adult men and women and in the right and left hemispheres.
Adult↗
Twins with different temporal lobe malformations: schizencephaly and arachnoid cyst.
The etiology and relationships between different forms of malformations of cortical development are poorly understood. Schizencephaly is generally regarded as unrelated to arachnoid cysts. As part of a systematic study of epilepsy in twins we observed a monozygotic twin pair discordant for temporal lobe epilepsy where the twin with epilepsy had unilateral temporal schizencephaly and periventricular heterotopia. The twin without epilepsy had an arachnoid cyst in the same temporal lobe. Although an incidental association is possible, this observation, together with occasional reports of schizencephaly and arachnoid cysts within one individual, suggests a shared pathogenic mechanism. Schizencephaly can be caused by both genetic and acquired factors. We propose that our observations in this twin pair are best explained by a genetic factor present in both twins, with an additional environmental insult resulting in schizencephaly in only one of the pair.
Adult↗
Hemicranial volume deficits in patients with temporal lobe epilepsy with and without hippocampal sclerosis.
PURPOSE: In patients with refractory temporal lobe epilepsy, studies have suggested volume deficits measured by MRI of brain structures outside the epileptogenic hippocampus. Hippocampal sclerosis (HS) is a frequent, but not obligate, finding in such patients. The present study examines the influence of the presence of HS on quantitative magnetic resonance imaging (MRI) measurements. METHODS: We analyzed 47 patients and 30 controls by quantitative MRI, including intracranial volume (ICV), hemicranial volume, hippocampal volume (HCV), and T2 relaxometry. MRI results were compared with histological findings in the resected temporal lobe. RESULTS: Histology documented HS in 35 patients (HS group) and other findings in 12 patients (no-HS group). In both groups, the hemicranial volume ipsilateral to the epileptogenic focus was significantly smaller than on the contralateral side (p < 0.004). The HCV on both sides was smaller in the HS group compared with patients without HS (p < or = 0.004). Unilateral hippocampal atrophy and increased T2 value were found in 71% of patients with HS, and bilaterally normal HCV and T2 value were found in 67% of patients without HS. CONCLUSIONS: The smaller hemicranial volume on the focus side, irrespective of the presence or absence of HS suggests a different pathogenic mechanism for the additional hemicranial volume deficit, compared to HS itself. The contralateral HCV deficit depends on the presence of HS, indicating a pathogenic connection between damage to both hippocampi.
Adult↗
Developmental background and outcome in patients with nonepileptic versus epileptic seizures: a controlled study.
PURPOSE: This study was designed to evaluate the relevance of developmental emotional stress factors, which are considered to influence emotional functioning, as contributing factors in the development of psychogenic symptoms. METHODS: Ten patients with nonepileptic seizures (non-ESs) (frequently referred to as psychogenic seizures), in whom diagnosis had been confirmed by a placebo-infusion test (PT), and 10 control patients with complex partial seizures (ESs) were evaluated with regard to developmental background by using structured in-depth interviews performed by a single interviewer blinded to the diagnosis. In addition, outcome assessment in the non-ES group after PT was done. RESULTS: There were no significant differences between groups in developmental psychosocial stress or in any single developmental stress factor. Six months after PT, the outcome was favorable in patients with non-ES: six were seizure free, and two had a considerable reduction in frequency of seizures. CONCLUSIONS: Our research indicates the following: (a) a high incidence of developmental stress factors and functional disturbances may be found in patients with non-ESs and ESs; (b) assessment of developmental emotional stress and functional disturbances as a basis for validating the diagnosis of non-ES should be treated with caution; (c) use of PT followed by supportive information about the nonepileptic origin of the attacks may have a positive therapeutic effect or help to introduce psychotherapeutic treatment.
Adult↗
Hippocampal sclerosis studied in identical twins.
OBJECTIVE: To test both the genetic and acquired hypotheses for the etiology of hippocampal sclerosis (HS) by studying with optimized and quantitative MRI three monozygous (MZ) twin pairs in which the index twin had temporal lobe epilepsy and HS. BACKGROUND: There is conflicting evidence in the literature regarding whether HS is genetic or acquired prenatally, perinatally, or as a consequence of prolonged childhood seizures. METHODS: We compared three MZ pairs with 30 age-matched control subjects who had no history of a neurologic disorder; we also used the twins as matched samples to assess subtle differences between the affected and the unaffected twins. RESULTS: All of the affected twins had prolonged seizures with fever in early childhood, which stood out as the unique factor common to all affected twins and was absent in all the unaffected twins. HS was present in all affected twins but was absent in the unaffected twin on visual, volumetric, and T2 relaxometry criteria. Comparison of the affected twin with the co-twin revealed that intracranial volume ipsilateral to the HS was relatively small in two of three affected twins. CONCLUSIONS: The absence of HS in the unaffected twin is strong evidence against a genetic hypothesis for HS. Neither perinatal problems nor birth order were factors in determining the presence of HS. This twin study supports the notion of HS as an acquired lesion secondary to prolonged seizures in early childhood and suggests that regional abnormalities of intracranial volume are associated with HS.
Adult↗
Long-term treatment of chronic relapsing inflammatory demyelinating polyradiculoneuropathy: combination of corticosteroids, plasma exchange, and intravenous immunoglobulins.
Explore the source record for details and available documents.
Adrenal Cortex Hormones↗
Patterns of muscle activity in legs in sleep apnea patients before and during nCPAP therapy.
Nocturnal muscle activity in legs (MAL) was studied in 19 patients with sleep apnea syndrome before and during nCPAP. We distinguished periodic (P-MAL) and nonperiodic (N-MAL) as well as apnea-associated and independent MALs. N-MALs were strongly associated with apneas and disappeared during nasal continuous positive airway pressure (nCPAP). P-MALs showed a rather long intermovement interval (IMI) of about 54 s when associated with respiratory disturbances and a shorter one of about 38 s when occurring independently. In 5 patients with predominantly independent P-MALs and a short IMI before and during nCPAP an incidental coexistence of sleep apnea with periodic leg movements in sleep syndrome (PLMS) was postulated. Five patients with an equal number of apnea-associated and independent MALs before nCPAP had a long IMI which shortened under nCPAP, while the majority of MALs become independent. This could indicate a facilitating or an unmasking effect of nCPAP on PLMS. Four patients with predominantly apnea-associated MALs with a long IMI before nCPAP showed an unchanged MAL pattern under nCPAP, despite the disappearance of apneas. Since some arousals persisted in these patients, insufficient nCPAP pressure with an upper airway resistance syndrome was suspected. We conclude that when P-MALs persist under nCPAP therapy a long IMI may indicate an upper airway resistance syndrome, whereas a short IMI may indicate a coexisting or even induced PLMS.
Adult↗
Blink reflex excitability is abnormal in patients with periodic leg movements in sleep.
The excitability of the late component of the blink reflex was measured in 19 patients with periodic leg movements in sleep (PLMS) and in 18 control patients with other sleep complaints. PLMS patients were not different from controls regarding age, body mass index, and apneas and hypopneas per hour of sleep. The latencies of the R1 and R2 components of the blink reflex were not different between the groups. However, in PLMS, the excitability of the R2 response was markedly enhanced: The second R2 response to a pair of stimuli given at an interval of 500 ms was in PLMS 59.5% (range 21-87%) of the first R2 response and in controls 18.8% (range 2-79%) (p < 0.0001, Mann-Whitney U test). These results provide further evidence of an altered motor control in PLMS, which is likely to be located subcortically and possibly related to the dopaminergic striatopallidal system.
Adult↗
Autoantibody-associated sensory neuronopathy and intestinal pseudo-obstruction without detectable neoplasia.
A 60-year-old patient presenting with the typical features of progressive sensory neuronopathy and subsequent intestinal pseudo-obstruction was found to have antineuronal nuclear antibodies (ANNA-I or Anti-Hu). These findings were suggestive of a paraneoplastic syndrome, but neither clinically nor at autopsy could a neoplasm be detected. Neuropathological findings were identical with those known for carcinoma-associated forms with marked neuron loss of spinal sensory ganglia and myenteric plexus. Therefore, ANNA-I and intestinal pseudo-obstruction may in rare cases occur without detectable underlying cancer.
Antibodies, Antinuclear↗