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Meige syndrome: review of 8 cases
Interhemispheric communication in visuo-verbal J. Lindeboom
(Amsterdam)
Visuo-verbal disconnection denotes a condition where visual input to the language zones of the left cerebral hemisphere is interrupted; however, even with total disconnection, visual naming is partly successful. It is argued that in such
disconnection cases the left hemisphere gains information about visual events by crossexamining the right hemisphere, and that this technique can be developed to a greater extent than has been noted in split-brain patients. This is illustrated by the results of a case study.
Scientific meeting of the Netherlands Society of Neurology held in Groningen, 31st May and 1st June 1985. The diagnosis of myasthenia gravis (MG) H.J.G.H.
Oosterhuis
tGroninge~)
A retrospective analysis was made of the factors that contributed to the delay of the diagnosis MG in 250 consecutive patients and of the factors that contributed to a false positive diagnosis in 140 patients. Of the MC-patients 66%, 78% and 91% had been diagnosed after one, two and five years respectively. A lack of suspicion of the diagnosis, or of the organic character of the symptoms was the main reason of the delay. In half of the patients seen by a neurologist the suspected diagnosis was rejected because 1) anticholinesterases failed to relieve the signs, 2) the EMG showed no decrement at 3Hz stimulation, or 3) the presence of local muscle atrophy and signs of denervation. A false positive diagnosis of MG was made in
patients with a) chronic fatique without demonstrable weakness (n=46), b) conversion syndromes (n=19), c) ocular problems (n=50) such as ptosis and or diplopia and blepharospasm and d) various neurological diseases (n=25). The non-placebo controlled and mainly subjective improvement to anticholinesterases and the EMG-decrement at to high stimulation rates (lo-20Hz) were the main reasons for a false positive diagnosis. Using the test-data of both groups, the sensitivity and specificity of the tests was catculated and the predictive value in confirming or excluding the diagnosis MG was demonstr; ted. It was emphasized that the a priori (=clinical) change on which the predictive value of all tests are dependent is the result of the neurologist’s clinical skill.
The effect of low frequency electrical stimulation on the denervated muscle - a clinical study. T. W. van Weerden,
A.M. Boonstra,
Eisma, H.J.G.H.
W.H. Oosterhuis (Groningen)
The effect of low frequency electrical stimulation (LES) in a group of 114 patients with a single or combined, total or partial lesion of the median, ulnar or peroneal nerve or brachial plexus on denervation atrophy and recovery of
the muscles was examined. The differences between the patients were evaluated by means of clinical muscle force testing, EMG, dynamometry (ulnar, median and radial nerve lesions), computer tomography (ulnar nerve lesions) and ultrasonography (median and peroneal nerve lesions). A beneficial effect of LES could not be shown.
Meige syndrome: review of 8 cases. J.W.
T.W. van Weerden Lakke (Groningen)
Snoek,
J.P.W.F.
We evaluated 236
the clinical characteristics
and
and
response to treatment of 8 patients (5 men, 3 women) with a combination of blepharospasm (Meige movements and oromandibular syndrome). Mean age at onset was 56 years
(range 45-68). Laboratory testing and evoked potential studies revealed no abnormalities. CT scans were abnormal in 3 cases: 1. marked asymmet~ of the ventric~ar system, suggesting atrophy of one caudate nucleus; 2. basal ganglia calcifications; and 3. a cerebellopontine angle tumour, which on exploration proved to be a meningioma. In the latter case no improvement in abnormal movements occurred after surgery. NMR studies, performed in 4 patients, were normal. Pharmacotherapy included the administration of dopamine antagonists, dopamine ago-
nists, anticholinergics, benzodiazepines and baclofen. Response to treatment was poor, the only exceptions being a temporary improvement with tetraben~ne and baclofen. Treatment with sodium valproate, a GABA mimetic agent, in a double-blind crossover study in 5 patients did not result in clinical improvement and no effect on CSF GABA levels was seen. Bilateral open division of branches of the facial nerve to orbicularis oculi and frontal muscles was performed in one patient, with a favourable effect on both blepharospasm and oromandibular dystonia.
Driving skills and residual mental impairments after severe head injury A.H. van Zomeren, W.H. Brouwer, J.M. Minderhoud, J.W. Snoek (Groningen) Brain damage is often assumed to have a negative effect on the patient’s fitness to drive a car, but the exact relations between residual mental impairments and driving skills are as yet unclear. Therefore, a pilot study was devised in which 9 survivors of very severe head injury, al1 of whom were driving again, were studied at an average interval of 6 years after injury. The investigation consisted of an interview, a neurological examination, a neuro-psychological test battery, a ride in an instrumented car that recorded Lateral Position Control (lanetracking ability), and the Test for Advanced Drivers as devised by the Dutch
Automobile Association A.N.W.B. The result of the latter test served as an external criterion of driving skill. In comparison with a control group, the headinjured patients performed worse on Lateral Position Control and the Test for Advanced Drivers. In addition, they had clear residual neurological and psychologi~l deficits. However, few relations were found between actual driving and neurological or psychological variables. A possible explanation for this negative finding is given in terms of a hierarchical model of car driving, in which impairments at the lowest or operational level can be compensated by taking decisions on tactical and strategical levels which decrease the probability of timepressure lower in the hierarchy.
Cisplatin-induced polyneuropathy: a clinical and electrophysiological study in 44 patients G. Tiessens Visser .
and
B. W’. Ongerboer
de
Clinical and ele~trophysiological studies - including some prospective and follow-up studies - in 44 patients suffering from Cisplatin-induced polyneuropathy are presented. Patients are subdivided in two groups: group I (n=18) receiving cyclophosphamide and cisplatin, of whom two patients also received adriamycin and group II (n=26) receiving cyclophosphamide, hexamethylmelamine, adriamycin and
cisplatin. A predominantly sensory neuropathy of varying severity showing loss of sensory modalities and reflex changes was present in all patients. Lhermitte’s sign was noted in 40%. Severe cases showed painful paresthesias, extensive sensory loss and disabling ataxia. No evident difference in character or incidence of neuropathy between the two groups was noted. Recovery was slow and, in the time observed (up to 28,5 months), incomplete. Electrophysiological results corresponded with distal axonopathy, which was confirmed by histologi237
(Amsterdam)
Visuo-verbal disconnection denotes a condition where visual input to the language zones of the left cerebral hemisphere is interrupted; however, even with total disconnection, visual naming is partly successful. It is argued that in such
disconnection cases the left hemisphere gains information about visual events by crossexamining the right hemisphere, and that this technique can be developed to a greater extent than has been noted in split-brain patients. This is illustrated by the results of a case study.
Scientific meeting of the Netherlands Society of Neurology held in Groningen, 31st May and 1st June 1985. The diagnosis of myasthenia gravis (MG) H.J.G.H.
Oosterhuis
tGroninge~)
A retrospective analysis was made of the factors that contributed to the delay of the diagnosis MG in 250 consecutive patients and of the factors that contributed to a false positive diagnosis in 140 patients. Of the MC-patients 66%, 78% and 91% had been diagnosed after one, two and five years respectively. A lack of suspicion of the diagnosis, or of the organic character of the symptoms was the main reason of the delay. In half of the patients seen by a neurologist the suspected diagnosis was rejected because 1) anticholinesterases failed to relieve the signs, 2) the EMG showed no decrement at 3Hz stimulation, or 3) the presence of local muscle atrophy and signs of denervation. A false positive diagnosis of MG was made in
patients with a) chronic fatique without demonstrable weakness (n=46), b) conversion syndromes (n=19), c) ocular problems (n=50) such as ptosis and or diplopia and blepharospasm and d) various neurological diseases (n=25). The non-placebo controlled and mainly subjective improvement to anticholinesterases and the EMG-decrement at to high stimulation rates (lo-20Hz) were the main reasons for a false positive diagnosis. Using the test-data of both groups, the sensitivity and specificity of the tests was catculated and the predictive value in confirming or excluding the diagnosis MG was demonstr; ted. It was emphasized that the a priori (=clinical) change on which the predictive value of all tests are dependent is the result of the neurologist’s clinical skill.
The effect of low frequency electrical stimulation on the denervated muscle - a clinical study. T. W. van Weerden,
A.M. Boonstra,
Eisma, H.J.G.H.
W.H. Oosterhuis (Groningen)
The effect of low frequency electrical stimulation (LES) in a group of 114 patients with a single or combined, total or partial lesion of the median, ulnar or peroneal nerve or brachial plexus on denervation atrophy and recovery of
the muscles was examined. The differences between the patients were evaluated by means of clinical muscle force testing, EMG, dynamometry (ulnar, median and radial nerve lesions), computer tomography (ulnar nerve lesions) and ultrasonography (median and peroneal nerve lesions). A beneficial effect of LES could not be shown.
Meige syndrome: review of 8 cases. J.W.
T.W. van Weerden Lakke (Groningen)
Snoek,
J.P.W.F.
We evaluated 236
the clinical characteristics
and
and
response to treatment of 8 patients (5 men, 3 women) with a combination of blepharospasm (Meige movements and oromandibular syndrome). Mean age at onset was 56 years
(range 45-68). Laboratory testing and evoked potential studies revealed no abnormalities. CT scans were abnormal in 3 cases: 1. marked asymmet~ of the ventric~ar system, suggesting atrophy of one caudate nucleus; 2. basal ganglia calcifications; and 3. a cerebellopontine angle tumour, which on exploration proved to be a meningioma. In the latter case no improvement in abnormal movements occurred after surgery. NMR studies, performed in 4 patients, were normal. Pharmacotherapy included the administration of dopamine antagonists, dopamine ago-
nists, anticholinergics, benzodiazepines and baclofen. Response to treatment was poor, the only exceptions being a temporary improvement with tetraben~ne and baclofen. Treatment with sodium valproate, a GABA mimetic agent, in a double-blind crossover study in 5 patients did not result in clinical improvement and no effect on CSF GABA levels was seen. Bilateral open division of branches of the facial nerve to orbicularis oculi and frontal muscles was performed in one patient, with a favourable effect on both blepharospasm and oromandibular dystonia.
Driving skills and residual mental impairments after severe head injury A.H. van Zomeren, W.H. Brouwer, J.M. Minderhoud, J.W. Snoek (Groningen) Brain damage is often assumed to have a negative effect on the patient’s fitness to drive a car, but the exact relations between residual mental impairments and driving skills are as yet unclear. Therefore, a pilot study was devised in which 9 survivors of very severe head injury, al1 of whom were driving again, were studied at an average interval of 6 years after injury. The investigation consisted of an interview, a neurological examination, a neuro-psychological test battery, a ride in an instrumented car that recorded Lateral Position Control (lanetracking ability), and the Test for Advanced Drivers as devised by the Dutch
Automobile Association A.N.W.B. The result of the latter test served as an external criterion of driving skill. In comparison with a control group, the headinjured patients performed worse on Lateral Position Control and the Test for Advanced Drivers. In addition, they had clear residual neurological and psychologi~l deficits. However, few relations were found between actual driving and neurological or psychological variables. A possible explanation for this negative finding is given in terms of a hierarchical model of car driving, in which impairments at the lowest or operational level can be compensated by taking decisions on tactical and strategical levels which decrease the probability of timepressure lower in the hierarchy.
Cisplatin-induced polyneuropathy: a clinical and electrophysiological study in 44 patients G. Tiessens Visser .
and
B. W’. Ongerboer
de
Clinical and ele~trophysiological studies - including some prospective and follow-up studies - in 44 patients suffering from Cisplatin-induced polyneuropathy are presented. Patients are subdivided in two groups: group I (n=18) receiving cyclophosphamide and cisplatin, of whom two patients also received adriamycin and group II (n=26) receiving cyclophosphamide, hexamethylmelamine, adriamycin and
cisplatin. A predominantly sensory neuropathy of varying severity showing loss of sensory modalities and reflex changes was present in all patients. Lhermitte’s sign was noted in 40%. Severe cases showed painful paresthesias, extensive sensory loss and disabling ataxia. No evident difference in character or incidence of neuropathy between the two groups was noted. Recovery was slow and, in the time observed (up to 28,5 months), incomplete. Electrophysiological results corresponded with distal axonopathy, which was confirmed by histologi237