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EEG sleep in two patients with thalamic neoplastic lesions
s95 NEUROPSYCHOLOGICAL AND NEUROPHYSIOLOGICAL STUDIES IN MYOTONIC MUSCULAR DYSTROPHY (MMD). M. Mt!dicz, A.S. Santos, F. Dalmas, Crispino, C. Nin and L. Bocos (Hospital
Italiano.
Montevideo,
D. Cibils, M. De Mora,
L.
Uruguay)
Ten patients fulfilling the diagnostic criteria for MMD were studied in order to determine impairment of the CNS. A clinical neuropsychological evaluation was performed using: WAIS-R; Piaget cognitive test; memory evaluation with the Montevideo protocol; assessment of language with the Spreen-Benton battery adapted to Spanish by Mendilharzu et al.; and a praxis protocol. Results were compared with those from a healthy control group. A battery of neurophysiological tests was administered including: pattern and flash VEPs; short, medium and long latency auditory and somatosensory EPs (including cognitive P300); FFT of spontaneous EEG during different tasks. All results were analysed on a 16-channel brain mapping system and statistically compared with a normal age-matched control group. The relationship between clinical and neurophysiological features showed some evidence of CNS impairment as evidenced by frequency analysis of spontaneous EEG and amplitude and distribution of P300. Complete results will be shown at the meeting. We consider that our results provide new evidence of multisystemic pathological features in MMD, especially the CNS and it will be necessary and useful to test all MMD patients in order to differentiate between groups.
PHOTIC EVOKED EPILEPTOGENIC SPIKES IN THE JANSKY-BIELSCHOWSKY TYPE OF NEURONAL CEROID LIPOFUCSINOSIS. C. Medina, I. Sanchez (Hospital
J.P. Garraham,
enhancement of the normal early components of the VEP, but their waveform, topography, amplitude and latency are clearly distinct from any of the normal VEP components. Moreover. in 3 of our patients they were synchronous with myoclonic jerks; therefore, we interpret them as light-evoked epileptogenie spikes that should not be confused with VEP or ERG responses.
de Leon. R.C. Reisin and N. Fejerman Buenos Aires, Argentina)
We studied 7 children with the Jansky-Bielschowsky type of neuronal ceroid lipofucsinosis (JB-NCL) using conventional and computerized EEG, ERG and flash VEP. Despite complete abolition of the ERG and the VEP in every patient, we recorded in all of them a large and well-defined spike (mean latency: 55 msec; mean amplitude: 57 pV) in response to single flashes during intermittent photic stimulation (IPS). It is classically accepted that these discharges are characteristic of JB-NCL, predominate over occipital regions and are elicited in response to low frequency IPS (1-3/set). However, our investigation showed that (1) the discharges are generalized and do not always predominate over the occipital regions; (2) the discharges are elicited not only at low but also at high frequency stimulation (up to lO/sec or more in some cases). These discharges were postulated to represent an
EEG SLEEP IN TWO PATIENTS NEOPLASTIC LESIONS. G. Mennuni (Catholic
WITH
THALAMIC
and M.A. PetreNa
University,
Rome, Italy)
The present work was aimed at studying the EEG organization during night sleep in 2 patients with neoplastic thalamic lesions. In both patients CT scan and awake EEG routine was performed. The EEG sleep pattern was evaluated from 3 consecutive polygraphic recordings obtained during spontaneous night sleep. Case V.L. (male, 37 years old) - CT scan: neoplastic lesion of the medium and posterior regions of the left thalamus. sparing the anterior zone. Awake EEG recording: asymmetry of the alpha rhythm with lower voltage on the left side. EEG sleep organization: neither the morphological evaluation of the recordings nor the quantitative analysis of the sleep stages showed any impairment. Case C.F. (male, 64 years old) - CT scan: neoplastic lesions involving the entire left thalamus, the adjoining cerebral peduncle and the most rostra1 part of the mesencephalon. Awake EEG recording: polymorphic theta activity of low amplitude in the fronto-temporal regions of the left hemisphere, together with a reduced amplitude and recognition of stage 2 elements (spindles); quantitative analysis of the sleep stages showed a decrease of sleep efficiency and sleep steadiness, together with an alteration of the relative percentage of the sleep stages. These findings suggest that focal lesions of the medium and posterior regions of the thalamus do not modify sleep pattern, as in V.L. On the other hand, broader lesions, as in CF., certainly lead to a morphological impairment (poor evidence of spindles and prolonged slow activity); since a concomitant involvement of brain-stem structures was present, it is difficult to know to what extent these thalamic lesions are directly involved in quantitative changes of sleep stages.
THALAMIC MANS.
LESIONS
G. Mennuni.
M.A. Petrella, A. Azzoni and S. Mazza
(Catholic
University,
AND
Rome,
SLEEP
CHANGES
IN HU-
Italy)
We reviewed the literature concerning the relationships tween thalamic damage and sleep abnormalities in humans.
beWe
S96 selected reports of thalamic involvement, neuropathologically or neuroradiologically verified, associated to complaints of sleep disturbances (hypersomnia or insomnia), possibly supported by polygraphic recordings. Somnolence and/or hypersomnia are frequently observed in those cases with concomitant extrathalamic involvement (mesencephalic mostly); when such a condition is present in “pure” documented thalamic lesions, then the paramedian zone of thalamus (intralaminar nuclei) is unilaterally or, more frequently, bilaterally affected. The majority of the authors assumed that the hypersomnia reported in these thalamic lesions comes from a reduction of EEG activating influences originating from the brain-stem. Moreover, we found a few cases of insomnia, in some instances polygraphically documented, caused by thalamic damage; when a neuropathological study was performed, an involvement of dorsomedial nuclei (Lugaresi et al., New Engl. J. Med., 1986. 315: 997; Little et al.. Ann. Neural., 1986, 20: 231) was observed, according to some experimental data (Marini et al., Neurosci. Lett., 1988, 85: 223). From a general point of view. it seems that the thalamus plays a role both in morphological organization of some elements (spindles) and in implementation of sleep mechanisms. Nevertheless, in order to better define the role of the thalamus in sleep organization, further experimental investigations are needed; besides. in human pathology, interesting data can be obtained from both neuroradiological and polygraphic studies. possibly integrated with neuropathological examination.
NIGHT SLEEP STUDY IN A PATIENT AFFECTED BY PICK’S DISEASE (PD) AND AMYOTROPHIC LATERAL SCLEROSIS (ALS).
EEG LATERALITY AND HYPNOSIS. I. M&zdros (Kossuth
and Za. Rev&z
LaJos University.
Debrecen.
Hungary)
It is known from the literature that the two hemispheres are differently involved in cognitive processing; the left hemisphere of right handed people is more involved in analytical processes while the right is more active in tasks of mental imagery. Our earlier results based on subjective, behavioral and EEG data demonstrate a holistic type of signal processing in those highly susceptible to hypnosis; it is subjectively easier for them to respond to imagined than verbal stimuli, their reaction time is shorter and right hemispheric EEG power is higher in the alpha and beta range. More detailed computer analysis of 16-channel EEG recorded according to the lo-20 system showed that the power is higher in the left frontal region independent of hypnotic susceptibility while the activity of the central region is equivalent. The most characteristic differences appear in the right patieto-temporal derivations: in highly susceptible subjects right hemispheric temporo-parietal area shows more alpha or beta power especially in hypnosis. It seems that the left hemispheric predominance is more rigid in less susceptible individuals while the rest have more activity in their right hemisphere but according to the task they are able to mobilize their left hemisphere without any change in their hypnotic depth.
PERIPHERAL CHILDHOOD:
NEUROPATHIES IN INFANCY AND ELECTRODIAGNOSTIC FINDINGS.
S. Mitral
G. Mennuni, M.A. Petrella,
S. Magi, S. Servidei and P. Tonali (HBpital
(Catholic
University,
Rome, Italy)
The association of Pick’s disease (PD) with amyotrophic lateral sclerosis (ALS) is a rare clinical entity. The aim of this study is to describe the sleep organization in a patient (male, 49 years old), with familial history of PD, in whom both these diseases (PD and ALS) were associated. The diagnosis was suggested by clinical symptoms and laboratory tests (neuropsychological evaluation, CT scan, EMG, EEG) and was confirmed by autopsy. Sleep study was carried out by 3 consecutive night’s sleep polygraphic recordings; sleep scoring was made according to Rechtschaffen and Kales’ criteria (1968); data from the second and third night were averaged. We observed a decrease of sleep efficiency index (SEI) and an increase of number of stage shifts; moreover, WAS0 and Sl percentages were increased, while S2 percentage was decreased. Stage 2 elements (spindles) sometimes were not clearly recognizable; REM stage was fragmented and REMs, which were quite rare, often appeared as short bursts, with decreasing voltage.
de Bic$tre. Paris, France)
Peripheral neuropathies and particularly polyneuropathies are uncommon neurologic disorders that occur in infants and children. Their identification and characterization are a difficult task and critically important diagnostic information may be provided by an electrophysiologic evaluation. Among 552 electrodiagnoses performed during the last five years, the retrospective study of the clinical records showed that 191 patients were discharged from hospital with no proven neurological disease and 51 with a diagnosed encephalopathy without neuromuscular disorder. For the 55 neuropathies in infants under one year of age the two main diagnoses were either injuries of the brachial plexus or Werdnig-Hoffmann disease for floppy infants, muscle diseases having been a rare observation (6). In children, between 1 and 17 years, excluding toxic neuropathy and systemic diseases (diabetes, chronic cholestasis, uremia) 161 patients were examined. Peripheral trauma was the main diagnosis, followed by hereditary polyneuropathy, GuillainBarre Syndrome and the group of metabolic disorders associated with childhood polyneuropathy. The particularities. if
Italiano.
Montevideo,
D. Cibils, M. De Mora,
L.
Uruguay)
Ten patients fulfilling the diagnostic criteria for MMD were studied in order to determine impairment of the CNS. A clinical neuropsychological evaluation was performed using: WAIS-R; Piaget cognitive test; memory evaluation with the Montevideo protocol; assessment of language with the Spreen-Benton battery adapted to Spanish by Mendilharzu et al.; and a praxis protocol. Results were compared with those from a healthy control group. A battery of neurophysiological tests was administered including: pattern and flash VEPs; short, medium and long latency auditory and somatosensory EPs (including cognitive P300); FFT of spontaneous EEG during different tasks. All results were analysed on a 16-channel brain mapping system and statistically compared with a normal age-matched control group. The relationship between clinical and neurophysiological features showed some evidence of CNS impairment as evidenced by frequency analysis of spontaneous EEG and amplitude and distribution of P300. Complete results will be shown at the meeting. We consider that our results provide new evidence of multisystemic pathological features in MMD, especially the CNS and it will be necessary and useful to test all MMD patients in order to differentiate between groups.
PHOTIC EVOKED EPILEPTOGENIC SPIKES IN THE JANSKY-BIELSCHOWSKY TYPE OF NEURONAL CEROID LIPOFUCSINOSIS. C. Medina, I. Sanchez (Hospital
J.P. Garraham,
enhancement of the normal early components of the VEP, but their waveform, topography, amplitude and latency are clearly distinct from any of the normal VEP components. Moreover. in 3 of our patients they were synchronous with myoclonic jerks; therefore, we interpret them as light-evoked epileptogenie spikes that should not be confused with VEP or ERG responses.
de Leon. R.C. Reisin and N. Fejerman Buenos Aires, Argentina)
We studied 7 children with the Jansky-Bielschowsky type of neuronal ceroid lipofucsinosis (JB-NCL) using conventional and computerized EEG, ERG and flash VEP. Despite complete abolition of the ERG and the VEP in every patient, we recorded in all of them a large and well-defined spike (mean latency: 55 msec; mean amplitude: 57 pV) in response to single flashes during intermittent photic stimulation (IPS). It is classically accepted that these discharges are characteristic of JB-NCL, predominate over occipital regions and are elicited in response to low frequency IPS (1-3/set). However, our investigation showed that (1) the discharges are generalized and do not always predominate over the occipital regions; (2) the discharges are elicited not only at low but also at high frequency stimulation (up to lO/sec or more in some cases). These discharges were postulated to represent an
EEG SLEEP IN TWO PATIENTS NEOPLASTIC LESIONS. G. Mennuni (Catholic
WITH
THALAMIC
and M.A. PetreNa
University,
Rome, Italy)
The present work was aimed at studying the EEG organization during night sleep in 2 patients with neoplastic thalamic lesions. In both patients CT scan and awake EEG routine was performed. The EEG sleep pattern was evaluated from 3 consecutive polygraphic recordings obtained during spontaneous night sleep. Case V.L. (male, 37 years old) - CT scan: neoplastic lesion of the medium and posterior regions of the left thalamus. sparing the anterior zone. Awake EEG recording: asymmetry of the alpha rhythm with lower voltage on the left side. EEG sleep organization: neither the morphological evaluation of the recordings nor the quantitative analysis of the sleep stages showed any impairment. Case C.F. (male, 64 years old) - CT scan: neoplastic lesions involving the entire left thalamus, the adjoining cerebral peduncle and the most rostra1 part of the mesencephalon. Awake EEG recording: polymorphic theta activity of low amplitude in the fronto-temporal regions of the left hemisphere, together with a reduced amplitude and recognition of stage 2 elements (spindles); quantitative analysis of the sleep stages showed a decrease of sleep efficiency and sleep steadiness, together with an alteration of the relative percentage of the sleep stages. These findings suggest that focal lesions of the medium and posterior regions of the thalamus do not modify sleep pattern, as in V.L. On the other hand, broader lesions, as in CF., certainly lead to a morphological impairment (poor evidence of spindles and prolonged slow activity); since a concomitant involvement of brain-stem structures was present, it is difficult to know to what extent these thalamic lesions are directly involved in quantitative changes of sleep stages.
THALAMIC MANS.
LESIONS
G. Mennuni.
M.A. Petrella, A. Azzoni and S. Mazza
(Catholic
University,
AND
Rome,
SLEEP
CHANGES
IN HU-
Italy)
We reviewed the literature concerning the relationships tween thalamic damage and sleep abnormalities in humans.
beWe
S96 selected reports of thalamic involvement, neuropathologically or neuroradiologically verified, associated to complaints of sleep disturbances (hypersomnia or insomnia), possibly supported by polygraphic recordings. Somnolence and/or hypersomnia are frequently observed in those cases with concomitant extrathalamic involvement (mesencephalic mostly); when such a condition is present in “pure” documented thalamic lesions, then the paramedian zone of thalamus (intralaminar nuclei) is unilaterally or, more frequently, bilaterally affected. The majority of the authors assumed that the hypersomnia reported in these thalamic lesions comes from a reduction of EEG activating influences originating from the brain-stem. Moreover, we found a few cases of insomnia, in some instances polygraphically documented, caused by thalamic damage; when a neuropathological study was performed, an involvement of dorsomedial nuclei (Lugaresi et al., New Engl. J. Med., 1986. 315: 997; Little et al.. Ann. Neural., 1986, 20: 231) was observed, according to some experimental data (Marini et al., Neurosci. Lett., 1988, 85: 223). From a general point of view. it seems that the thalamus plays a role both in morphological organization of some elements (spindles) and in implementation of sleep mechanisms. Nevertheless, in order to better define the role of the thalamus in sleep organization, further experimental investigations are needed; besides. in human pathology, interesting data can be obtained from both neuroradiological and polygraphic studies. possibly integrated with neuropathological examination.
NIGHT SLEEP STUDY IN A PATIENT AFFECTED BY PICK’S DISEASE (PD) AND AMYOTROPHIC LATERAL SCLEROSIS (ALS).
EEG LATERALITY AND HYPNOSIS. I. M&zdros (Kossuth
and Za. Rev&z
LaJos University.
Debrecen.
Hungary)
It is known from the literature that the two hemispheres are differently involved in cognitive processing; the left hemisphere of right handed people is more involved in analytical processes while the right is more active in tasks of mental imagery. Our earlier results based on subjective, behavioral and EEG data demonstrate a holistic type of signal processing in those highly susceptible to hypnosis; it is subjectively easier for them to respond to imagined than verbal stimuli, their reaction time is shorter and right hemispheric EEG power is higher in the alpha and beta range. More detailed computer analysis of 16-channel EEG recorded according to the lo-20 system showed that the power is higher in the left frontal region independent of hypnotic susceptibility while the activity of the central region is equivalent. The most characteristic differences appear in the right patieto-temporal derivations: in highly susceptible subjects right hemispheric temporo-parietal area shows more alpha or beta power especially in hypnosis. It seems that the left hemispheric predominance is more rigid in less susceptible individuals while the rest have more activity in their right hemisphere but according to the task they are able to mobilize their left hemisphere without any change in their hypnotic depth.
PERIPHERAL CHILDHOOD:
NEUROPATHIES IN INFANCY AND ELECTRODIAGNOSTIC FINDINGS.
S. Mitral
G. Mennuni, M.A. Petrella,
S. Magi, S. Servidei and P. Tonali (HBpital
(Catholic
University,
Rome, Italy)
The association of Pick’s disease (PD) with amyotrophic lateral sclerosis (ALS) is a rare clinical entity. The aim of this study is to describe the sleep organization in a patient (male, 49 years old), with familial history of PD, in whom both these diseases (PD and ALS) were associated. The diagnosis was suggested by clinical symptoms and laboratory tests (neuropsychological evaluation, CT scan, EMG, EEG) and was confirmed by autopsy. Sleep study was carried out by 3 consecutive night’s sleep polygraphic recordings; sleep scoring was made according to Rechtschaffen and Kales’ criteria (1968); data from the second and third night were averaged. We observed a decrease of sleep efficiency index (SEI) and an increase of number of stage shifts; moreover, WAS0 and Sl percentages were increased, while S2 percentage was decreased. Stage 2 elements (spindles) sometimes were not clearly recognizable; REM stage was fragmented and REMs, which were quite rare, often appeared as short bursts, with decreasing voltage.
de Bic$tre. Paris, France)
Peripheral neuropathies and particularly polyneuropathies are uncommon neurologic disorders that occur in infants and children. Their identification and characterization are a difficult task and critically important diagnostic information may be provided by an electrophysiologic evaluation. Among 552 electrodiagnoses performed during the last five years, the retrospective study of the clinical records showed that 191 patients were discharged from hospital with no proven neurological disease and 51 with a diagnosed encephalopathy without neuromuscular disorder. For the 55 neuropathies in infants under one year of age the two main diagnoses were either injuries of the brachial plexus or Werdnig-Hoffmann disease for floppy infants, muscle diseases having been a rare observation (6). In children, between 1 and 17 years, excluding toxic neuropathy and systemic diseases (diabetes, chronic cholestasis, uremia) 161 patients were examined. Peripheral trauma was the main diagnosis, followed by hereditary polyneuropathy, GuillainBarre Syndrome and the group of metabolic disorders associated with childhood polyneuropathy. The particularities. if