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Biomedical subjects

M Koutroumanidis

Publications and source records attributed to M Koutroumanidis.

33 records · Page 2Linked to original sources

Interictal 18FDG PET findings in temporal lobe epilepsy with déjà vu.

The authors studied the functional anatomy of the déjà vu (DV) experience in nonlesional temporal lobe epilepsy (TLE), using interictal fluorine-18 fluorodeoxyglucose PET in 14 patients with and 17 patients without DV. Several clinical conditions, such as age at PET study, side of ictal onset zone, and dominance for language, were no different between the two groups. The patients with DV showed significant relative reductions in glucose metabolism in the mesial temporal structures and the parietal cortex. The findings demonstrate that ictal DV is of no lateralizing value. They further suggest that temporal lobe dysfunction is necessary but not sufficient for the generation of DV. Extensive association cortical areas may be involved as part of the network that integrates this distinct experience.

Adult↗

The variants of reading epilepsy. A clinical and video-EEG study of 17 patients with reading-induced seizures.

We present the clinical and electrographic data of 17 patients with reading-induced seizures documented with ictal video-EEG studies during provocation with language related tasks. The median age at onset was 15 years (range 11-22 years) and the male:female ratio was 2.4. Fourteen patients had no spontaneous seizures of any type while the remaining three had infrequent generalized tonic-clonic seizures during nocturnal sleep. Two distinct electroclinical ictal patterns were confirmed on video-EEG analysis. (i) Fifteen patients had reading-induced jerks which invariably involved the region of the jaw but also included the upper limbs in five of them. Ictal EEG discharges were noted in 12 patients; these were brief but varied in terms of morphology and spatial distribution, with a clear tendency for left-sided predominance. All but one of these patients had similar myoclonic seizures induced by linguistic activities other than reading, the phenomenon probably justifying the term 'language-induced epilepsy'. Some patients had evidence of transient cognitive impairment associated with the reading-induced jaw or limb jerks. Three patients had a sibling with reading epilepsy but there was no other family history of epileptic seizures. (ii) Two patients had reading-provoked paroxysmal alexia without motor symptoms, associated with prolonged focal ictal EEG abnormalities. Reading provoked a subclinical, continuous and reproducible EEG activation over the left posterior temporal area. We propose that ictogenesis in reading or language-induced epilepsy is based on the reflex activation of a hyperexcitable network that subserves the function of speech and extends over multiple cerebral areas on both hemispheres. The parts of this network responding to the stimulus may, secondarily, drive the relative motor areas producing the typical regional myoclonus. This network hyperexcitability can be genetically determined and its clinical expression is age-related.

Adolescent↗

Positron emission tomography in partial epilepsies: the clinical point of view.

Epilepsy research using positron emission tomography (PET) has advanced our understanding of the pathophysiology and neurochemical correlates of both focal and generalized epilepsies, but from the clinical viewpoint its major contribution has been in the presurgical evaluation of patients with medically intractable partial seizures. Depending on the tracer used, PET may provide information on regional cerebral blood flow and glucose metabolism, and the binding of specific ligands to receptors that are thought to be related to the genesis and propagation of epileptic activity. In this communication, we discuss the diagnostic yield, limitations and perspectives of 18F-fluorodeoxyglucose (FDG) and 11C-flumazenil (FMZ) PET in partial epilepsies. The current evidence regarding the pathophysiology of the focal changes is also presented, with an emphasis on issues which must be carefully addressed for effective and reliable clinical research.

Brain↗

Clinical value of "ictal" FDG-positron emission tomography and the routine use of simultaneous scalp EEG studies in patients with intractable partial epilepsies.

PURPOSE: EEG is widely used during positron emission tomography (PET) to confirm the interictal state of the patient and assist in scan interpretation when a seizure occurs. Ictal scans usually reflect mixed interictal-ictal-postictal metabolic activity as seizures are brief in comparison to the 30-min uptake period of the tracer. We wished to determine whether routine EEG is justified and if seizures commonly affect the diagnostic information of the PET scan. METHODS: We examined the PET scans of 6 of 236 outpatients with intractable epilepsy with clinical and electrical evidence of a seizure during tracer uptake. We performed semi-quantitative analysis in 2 patients who had "ictal" and control interictal scans. RESULTS: Patients with single seizures lasting 23 s to 4 min [four complex partial seizures (CPS) and one absence seizure (AS)] had focal hypometabolism concordant with results of other investigations. One patient with complex partial status had irregular cortical uptake and focal hypometabolism, but the site of the ictal focus could not be confirmed. CONCLUSIONS: In this group of patients, seizures occurred infrequently during tracer uptake. The interpretation of the PET scan when single seizures occurred did not appear to be influenced by the continuous scalp EEG (CSEEG) recordings. The value of routine CSEEG in outpatients treated with medication should be reappraised, with potential cost savings. In rare circumstances in which a true ictal study occurs (complex partial status, epilepsia partialis continua, and repetitive CPS), PET scanning may be inconclusive and repeat interictal scanning should be pursued.

Adolescent↗

Typical absence status in adults: diagnostic and syndromic considerations.

PURPOSE: To study the electroclinical features of typical absence status (TAS) in adults with syndromes of idiopathic generalized epilepsies (IGEs). METHODS: Twenty-one patients with one or more spells of TAS were identified among 136 consecutive adult patients with IGEs. All patients with TAS had comprehensive electroclinical investigations and EEG or video-EEG recorded absences. RESULTS: TAS occurred in 24.4% of 86 patients who had IGEs with typical absences alone or in combination with other seizures presisting in adult life. The prevalence of TAS appeared to be syndrome related, ranging from as high as 57.1% in perioral myoclonia with absences and 46.2% in "phantom" absences with GTCS to as low as 6.7% in juvenile myoclonic epilepsy. A varying degree of impairment of cognition was the cardinal clinical symptom shared in all TAS, but corresponding syndromes of IGE were often betrayed by other symptoms such as eyelid or perioral myoclonia. In phantom absences with GTCS, TAS was more numerous (p < or = 0.05) and more frequently the first overt seizure type (p = 0.006) than in any other IGE. Only in the syndrome of eyelid myoclonia with absences, TAS was always situation related, mainly as a result of antiepileptic drug discontinuation. CONCLUSIONS: The clinical EEG semiology and prevalence of TAS appear to be syndrome related with the highest prevalence in the syndromes of perioral myoclonia with absences and phantom absences with GTCS (p = 0.0024).

Adult↗

Interictal regional slow activity in temporal lobe epilepsy correlates with lateral temporal hypometabolism as imaged with 18FDG PET: neurophysiological and metabolic implications.

OBJECTIVES: The phenomenon of interictal regional slow activity (IRSA) in temporal lobe epilepsy and its relation with cerebral glucose metabolism, clinical data, MRI, and histopathological findings was studied. METHODS: Interictal 18F-fluorodeoxyglucose positron emission tomography (FDG PET) was performed under continuous scalp EEG monitoring in 28 patients with temporal lobe epilepsy not associated with intracranial foreign tissue lesions, all of whom subsequently underwent resective surgery. Regions of interest (ROIs) were drawn according to a standard template. IRSA was considered lateralised when showing a 4:1 or greater ratio of predominance on one side. RESULTS: Sixteen patients (57%) had lateralised IRSA which was always ipsilateral to the resection and of maximal amplitude over the temporal areas. Its presence was significantly related to the presence of hypometabolism in the lateral temporal neocortex (p=0.0009). Logistic regression of the asymmetry indices for all measured cerebral regions confirmed a strong association between IRSA and decreased metabolism of the posterior lateral temporal neocortex only (p=0.009). No significant relation could be shown between slow activity and age at onset, duration of the epilepsy, seizure frequency, and MRI evidence for hippocampal atrophy. Furthermore, IRSA was not specifically related to mesial temporal sclerosis or any other pathology. CONCLUSIONS: Interictal regional slowing in patients with temporal lobe epilepsy not associated with a mass lesion is topographically related to the epileptogenic area and therefore has a reliable lateralising, and possibly localising, value. Its presence is irrelevant to the severity or chronicity of the epilepsy as well as to lateral deactivation secondary to neuronal loss in the mesial temporal structures. Although slow EEG activity is generally considered as a non-specific sign of functional disturbance, interictal regional slowing in temporal lobe epilepsy should be conceptualised as a distinct electrographic phenomenon which is directly related to the epileptogenic abnormality. The strong correlation between interictal regional slowing and lateral temporal hypometabolism suggests in turn that the second may delineate a field of reduced neuronal inhibition which can receive interictal and ictal propagation.

Adolescent↗

Chronic relapsing multifocal sensory-motor neuropathy with conduction block.

A 47 years old man had 13 episodes of relapsing and remitting sensory-motor neuropathy involving the upper limbs over the last 20 years. All but the last episode resolved spontaneously within 2 months. Neurophysiology revealed multifocal motor and sensory conduction block in the upper limbs with normal terminal latencies. CSF analysis was normal and anti-GM1 antibodies were not detected. There was a dramatic clinical improvement after intravenous immunoglobulin treatment. This case represents an unusual multifocal variant of chronic inflammatory demyelinating neuropathy.

Action Potentials↗

Idiopathic generalised epilepsy in adults manifested by phantom absences, generalised tonic-clonic seizures, and frequent absence status.

OBJECTIVES: To describe the clinical and EEG features of adult patients with very mild absences, late onset generalised tonic clonic seizures, and frequent absence status. METHODS: Patients were referrals to a clinic for epilepsies. They all had clinical assessment and EEG, video EEG, or both for documentation of absences. RESULTS: Of 86 adults with idiopathic generalised epilepsies and EEG/video-EEG documented absences, 13 patients showed similar clinico-EEG features with: (a) "phantom absences" consisting of mild ictal impairment of cognition associated with brief (3-4 s), generalised 3-4 Hz spike/multiple spike and slow wave discharges; (b) infrequent, mainly late onset, generalised tonic clonic seizures, and (c), absence status which occurred in six of them either in isolation or terminating with generalised tonic clonic seizures. None of the patients had myoclonic jerks or photosensitivity. Two patients were father and daughter and another patient had a family history of infrequent generalised tonic clonic seizures. CONCLUSION: It seems that this is an idiopathic generalised epilepsy syndrome in adults which has not been previously recognised.

Adolescent↗

Fixation-off-sensitive epilepsy with absences and absence status: video-EEG documentation.

A 33-year-old woman of borderline normal intelligence had catamenial absence status epilepticus and absences since early teens, diagnosed as temporal lobe epilepsy. Clinical and video-EEG evaluation documented that she suffers from a rare epileptic condition manifested with fixation-off sensitivity, absences, absence status, and infrequent generalized tonic-clonic seizures.

Adult↗

Reduced brain stem excitability in mitochondrial myopathy: evidence for early detection with blink reflex habituation studies.

Blink reflex (BR) was studied in 17 patients with histochemically and genetically confirmed mitochondrial myopathy (MM). Fourteen patients had chronic progressive external ophthalmoplegia (CPEO) associated with a mild to moderate craniosomatic myopathy without any symptoms or signs of central nervous system (CNS) involvement, 2 myoclonic epilepsy with ragged red fibers syndrome, and 1 Kearns-Sayre syndrome. The mean latencies of the early (R1) and late (R2) responses were prolonged (P < 0.01 and P < 0.001, respectively), and the corresponding amplitudes decreased (P < 0.001). Increased habituation of the reflex was clearly observed in 10 out of 14 patients tested (71.4%), 9 of whom presented CPEO. These findings suggest that the brain stem reticular network is in a state of basal inhibition which is presumably due to a subclinical impairment of the cerebral cellular metabolism. Multimodal evoked potentials revealed abnormalities suggestive of CNS involvement in 7 out of 17 patients (41.2%), 4 of whom had CPEO. These observations document the validity of BR in detecting clinically silent brain stem impairment in patients with apparently pure MM and provide important clues for a further understanding of the underlying pathophysiology.

Adolescent↗

Ophthalmologic examination in the diagnosis of Proteus syndrome.

PURPOSE: To describe the clinical features of Proteus syndrome, a rare recently recognized hamartoneoplastic malformation, with emphasis on the ocular findings. METHODS: Complete physical and ocular examination of two new patients with Proteus syndrome. RESULTS: The two reported cases illustrate the wide clinical polymorphism of Proteus syndrome and the overlap of its clinical manifestations with those of other overgrowth syndromes. Both patients had periorbital exostoses and epibulbar tumors. The ocular findings are compared with those in the literature. CONCLUSION: Considering the paucity of information in the ophthalmic literature, this article explores the role of the ophthalmologist in diagnosing this rare entity.

Abnormalities, Multiple↗