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Scientific Meeting of the Flemish Society of Neurology-Psychiatry held in Aalst, May 9th 1987
Scientific meeting of the Flemish Society of Neurology-Psychiatry held in Aalst, May 9th 1987. Epilepsy, mental retardation and ichtiosis: A case report. M . B . Lenders, P. de Wit, P. Castelein, L. Schuddinck (Zoersel) We report a 6-year-old boy who was admitted to the hospital in a status epilepticus. There was cyanosis, alternating gazing of the eyes with parcellar myoclonias. He is a healthy child of nonconsanguinous parents. The pregnancy was uneventful and there were no perinatal problems. There is no history of epilepsy. Since childhood he has a very dry, scaling skin, especially on the legs and recurrent asthmatiform bronchitis. He has a younger brother met ichtiosis. In the maternal family there is epilepsy: an aunt with secundary generalised epilepsy, a cousin with petit mal epilepsy. The mother has bitemporal spikes but no clinical epilepsy. In the paternal family there are several members with asthma and/or atopic eczema. The clinical examination the day after admission shows a small boy with hypertelorism and a very dry scaling skin. Abdomen and cor are normal. On lung auscultation there is some wheezing. The neurological examination is normal. Further investigations were done: routine blood and urine laboratory tests were normal; aminoacids in urine was normal, there was no elevated phytanic acid. The electrophysiology: the EEG shows a petit mal epilepsy; the evoked potentials (VEP, BAEP, SSEP) are normal. Ophthalmologic examination is normal: no retinitis pigmentosa or cataract. On audiometry there is a discrete loss of hearing right for the high frequences. CT-scan of the cerebrum is normal. Skin biopsy: X-linked ichtiosis. Intellectual quotient in february '86: 101; in febru-
ary '87: 71. Our patient presents with petit mal epilepsy, X-linked ichtiosis and mental retardation. Neurological and dermatological symptoms, e.g. phakomatosis, are specific for neurocutaneous diseases. There is also a large group that presents with ichtiosis and varying neurological problems. When ichtiosis and mental retardation are involved, one has to consider different syndromes. In the Sj6gren-Larssen Syndrome there is also a spastic paraplegia. In the Refsum disease (high phytanic acidataxia, hypertrophic polyneuropathy and retinitis pigmentosa are included. In the Rud Syndrome ichtiosis and mental retardation are associated with epilepsy whereas in the Netherton Syndrome there is a fourth symptom, trichorrhexis invaginata (bamboo hair). There are a lot more syndromes that present with ichtiosis and mental retardation, like the Passwell Syndrome, Senter Syndrome, but these are less frequent. According to the symptoms our patient presented, the diagnosis of Rud Syndrome was made. The major symptoms are epilepsy, mental retardation and ichtiosis. It is a genetic heterogenous syndrome with a varying degree of mental retardation and "epilepsy" which is sometimes only characterized by paroxisms on the EEG. Rud originally described a patient with also hypogonadism, hyperchromic anemia and polyneuritis. Nystagmus, anosmia, neurosensory deafness and recurrent airway infections are also mentioned but less frequently. Probably this syndrome will be better described when more features are available.
Infarctions of the thalamic region: Identification of five clinical pictures and corresponding C T-findings. G. V a n h o o r e n , I. D e h a e n e , M. van Zandijcke, M. M a r c h a u (Brugge) Infarctions in the thalamic area give rise to specific, clinical pictures. They can be correlated to typical hypodensities on CT-scan. Anterior choroidal artery territory infarction 298
causes contralateral hemiparesis, visual field defects and hemineglect. On CT-scan a laterothalamic hypodensity, extending in the capsula interna is noted. The thalamogeniculate artery irrigates the posterolateral thalamus, and infarction in this area causes pure sensory stroke. The
tuberothalamic infarction gives rise to longlasting neuropsychological deficit and is seen on CT pictures as a hypodensity in the anterolateral thalamus. A paramedian hypodensity in the posterior thalamic region is caused by occlusion of the thalamoperforating artery. A transient distur-
bance of consciousness with memory deficit and vertical eye movement abnormalities are observed. As an illustration, we presented four patients. We are looking for the fifth infarction type, in the anterior nucleus region, expected to be seen as an anteromedial hypodensity on CT-scan.
The syndrome of the anterior choroidal artery.
J. D e Bleecker, J. de R e u c k , G. Vingerhoets, W. Ceusters, and E. Thiery (Ghent) The complete clinical picture after infarction in the anterior choroidal artery territory combines the triad of hemiplegia, hemianesthesia, and homonymous hemianopia. CT and NMR examination reveal an area of reduced density in the posterior limb of the internal capsula, sparing the thalamus medially, and reaching the top of the globus pallidus laterally. Incomplete clinical forms are due to frequent individual differences in the vascular supply of this artery,
the hemianopia being the least constant symptom. A classic case of leftsided anterior choroidal artery syndrome is presented, with clear demonstration of the infarcted territory by CT scan and NMR. Attention is drawn to the speech disturbances as well as to the visual neglect. Neuro-ophthalmologic examination reveals dysfunction of the cortico-pontine projections. Neuropsychological testing is compatible with a left thalamofrontal syndrome. The clinical syndrome is correlated to the vascular anatomy of the infarct.
Pseudotumoral intracerebral hematoma due to cerebral amyioid angiopathy.
F. van Calenbergh, R. D o m , J. Goffin, C. Plets, and R. van den Bergh (Leuven) We describe the case history of a 72-year-old woman, who presented with progressive mental deterioration, headache and a mild left hemiparesis. These symptoms had an insidious onset and evolved over five weeks. CT scan and angiography were suggestive of a right parietal brain tumor, with a small hemorrhagic component. During the operation, we also had the impression of a gliomatous tumor with a central necrotic cyst. Pathological examination, how-
ever, showed an old hematoma with a fibrous capsule and neovascularisation. In the wall of these vessels, amyloid was revealed by Congo red staining. Small leptomeningeal vessels showed the same changes. The pseudotumoral or "chronic expanding" intracerebral hematoma (ICH) has only recently received special interest in the neurosurgical literature. As far as we know, our patient is the first, in whom cerebral amyloid angiopathy was found as an aetiological factor in this type of ICH.
Statistical analysis of 153 cases of meningitis.
J. de Koninck, and J. de R e u c k (Ghent) A retrospective study of patients with meningitis treated at the University Hospital of Ghent during the period 1980-1985 is presented.
Patients with immunodeficiency or C.S.F.leakage were excluded. A predominance of males was found for aseptic as well as for bacterial meningitis. The highest incidence of aseptic meningitis was seen in the age group between 299
20 and 30 years, while the occurrence of bacterial meningitis was less frequent in that age group. A summerpeak, typical for enteroviral infections was found for the majority of cases of aseptic meningitis. During this summerpeak a smaller percentage of patients received antibiotics before admission. Pre-adrnission antibiotic treatment was responsible for a negative bacterial C.S.F. culture in all cases and had also a
slight influence on the Wlc. count in the C.S.F. Pneumococcus meningitis appeared to be the most frequent and the most dangerous form in our adult population with 55% occurrence of convulsions in contrast to 3% for the other types of meningitis. The length of hospital stay was not significantly longer for bacterial meningitis than for aseptic meningitis and was not influenced by the pre-admission antibiotic therapy.
"La meningite du charcutier". W. Ceusters, Ph. van O o t e g h e m , A. V a k a e t , G. Claeys, J. Verschraegen, and J. de R e u c k ( G h e n t ) A case of an acute, purulent meningitis caused by Streptococcus suis type II is presented. This agent is known since 1959 when it was first isolated from septicaemic pigs. Since 1968 it has been recognized as a pathogenic agent for man,
causing septicaemia and meningitis. About 75% of the patients with meningitis develop an irreversible sensorineural hearing loss. Making the exact diagnosis can be a problem because most available commercial biochemical kits allow only to classify the agent as Streptococcus viridans. Except for the hearingloss our patient was cured succesfully with 12 gram of ampicilline daily.
Neurological complications of mononucleosis infectiosa. M. van O r s h o v e n and F. van Orshoven (Aalst). Following an influenzal illness with fever and cervical adenopathy, a 26-year-old young man developed disturbances of speaking, writing and balance, and vomiting. Serology proved the clinical diagnosis of mononucleosis infectiosa (M. I.): a positive Paul & Bunnell reaction, positive IgG (1:620) and IgM antibodies against Ebstein-Barr Viral Capsid Antigen (EBV-CA). The neurological examination demonstrated a bilateral cerebellar syndrome with long tract signs (piramidal reflexes in the legs with Chaddock reflexes in extension). CT of the brain, EEG and evoked potentials (VEP - B A E R SSEP n. medianus and n. saphenus) were normal. Lumbar puncture showed a clear liquid with 4 leucocytes, normal protein (32 mg%) with normal agarelectroforesis and
eiectrofocusing. EBV-CA IgM and IgG in the CSF were negative. Neurologic symptoms resolved in 3 months. Neurological complications of MI are well known: besides a cerebellar syndrome, a serous meningitis, encephalitis, epilepsy, myelitis, Guillain-Barr6 syndrome, and a cranial or peripheral mononeuritis have been described. Sometimes the infectious manifestations of MI may be absent. The prognosis is usually good. The pathological mechanism is uncertain: both the hypothesis of a direct viral agression of the nervous system and the possibility of a postinfectious allergic reaction have been postulated. Our observations would rather favour the second hypothesis. Anyway, examination of the specific EBV serology is worthwhile when confronted with a young person with neurological symptoms.
Poliomyelitis in an airline pilot and the need for vaccination of travellers. P. G o u b a n , H. Carton, H. Taelman, E. D r u y t s - V o e t s , J. D e Smyter (Leuven, Antwerp, Brussels) 300
Since the widespread use of polio vaccination, poliomyelitis has virtually disappeared from industrialized countries, but rare localized
outbreaks and sporadic cases still occur. A number of individuals in these widely immunized communities lack protective immunity against one or more poliovirus types. Such persons are not exposed due to the lack of circulation of poliovirus in these populations (herd im-
munity). Nevertheless, they are at high risk of paralytic poliomyelitis when they travel to endemic areas. We present a case which illustrates this point and emphasizes the importance of polio vaccination for travellers to developing countries.
Polyneuropathy due to lithium toxicity.
F. Piessens, B. M e y e r , and V. van den Bergh (Duffel, Leuven) A 50-year-old woman developed a severe hypertonic-hypokinetic extrapyramidal syndrome with muscle rigidity following addition of 1000 mg Haloperidol to a maintenance dose of Lithium carbonate 600 rag. Serum lithium level at that moment was within therapeutic range. One week after withdrawal of these drugs the hypertonic-hypokinetic
syndrome was replaced by dyskinetic movements, myoclonic jerks and head tremor. At the same time a flacid quadriparesis with absent tendon reflexes in the lower limbs became apparent. Electrodiagnostic studies suggested axonal lesions. Recovery of the polyneuropathy was very slow and still is incomplete after 3 months. As suggested by this and other studies serum lithium level may be a reliable parameter of neurotoxicity. A possible interaction between haloperidol and lithium is discussed.
Wilson's disease.
M. Marchau, T.U. H o o g e n r a a d , M. van Zandijcke, and I. D e h a e n e (Brugge, Utrecht) Wilson's disease is an autosomal recessive inherited disease. The major symptoms are due to deposits of copper in liver, brain and cornea. The urinary excretion of copper is elevated. Ceruloplasmin, the serum enzyme that binds copper, is defectively synthesized according to
some authors. Others state that the primary defect is intrahepatic and concerns the copper metabolism. This is followed by an impairment of ceruloplasmin synthesis. The neurologic signs are extrapyramidal (tremor, dysphagia and dysarthria, rigidity). With the use of CT scan and NMR typical abnormalities can be demonstrated: generalized atrophy of brain and brain stem, hypodensities in the basalganglia and cerebellar nuclei.
Wilson's disease: Treatment of the paradoxal reaction on penicillamine with oral zinc sulfate.
T.U. H o o g e n r a a d (Utrecht, Brugge)
and
M.
Marchau
A paradoxal reaction on penicillamine in Wilson's disease is the worsening of neurological signs and symptoms, although a good cupriuresis is found. It is one of the most feared complications of penicillamine therapy of Wilson's disease. Three patients with extrapyrami-
dal symptoms due to Wilson's disease were deteriorating shortly after the penicillamine therapy started. All three improved after stopping penicillamine and administration of oral zinc sulfate. In patients with neurologic symptoms due to hereditary copper toxicosis one should choose treatment with oral zinc sulfate.
301
Somatosensory evoked potentials in patients with Huntington's disease. E. Triau, M. Malfroid, R. D o m , F. Baro (Leuven) We studied the use of somatosensory evoked potentials in 25 patients with Huntington's Chorea, compared with a control group of healthy volunteers. On the response to median stimulation, no significant alteration of N20 latency was found, whereas there was a significant
decrease in amplitude of the primary cortical response N20/P25 in Huntington patients, in comparison to the control group. Our results confirm the findings of previous reports. Possible underlying pathological changes are discussed. The use of somatosensory evoked potentials on subjects at risk for Huntington's disease is still under investigation.
Single fibre EMG versus repetitive nerve stimulations in Myasthenia gravis. R. Mercelis, P. de Jonghe, J. Gheuens, and J.J. Martin ( A n t w e r p e n ) We performed single fibre EMG of the extensor digitorum communis (EDC) in 25 patients with defective neuromuscular transmission (23 myasthenia gravis, 1 congenital myasthenia, 1 Eaton-Lambert myasthenic syndrome). The study was considered abnormal if an increased jitter was found in 10% or more of the potential pairs or if the mean jitter of all potential pairs was increased. In most of the patients, repetitive nerve stimulations were done in the abductor digiti minimi (ADM) and in the trapezius muscle. Records were made at 3/sec before and until 4 minutes after 30 sec of maximal voluntary activation of the muscle. A decrement of 8% or
more between the 1st and the 4th or the 5th response was taken as abnormal. The "doublestep" test with ischemia of the forearm was not done. Singel fibre EMG was abnormal in all but 2 patients. One of these patients was in clinical remission of ocular myasthenia gravis. She had an increased jitter in 1 out of 20 potential pairs and the mean jitter was on the border of normality. The second patient with normal jitter in the EDC suffered from unilateral ptosis and had an increased jitter in the orbicularis oculi. Repetitive nerve stimulations were abnormal in 7 out of 23 patients in the ADM and in 11 out of 21 in the trapezius. Single fibre EMG is a valuable aid in the detection of abnormality of neuromuscular transmission.
Evaluation of computer assisted EEG-analysis in clinical practice. P. de Wit (Zoersel) Numerical analysis of EEG signals can be performed with a Personal Computer based EEGAnalysis-System. The communication deals with some clinical aspects of mathematical
302
models used in the computer system. The use of quantitative EEG-analysis in combination with visual analysis increases the value of EEG examination in clinical situation and is encouraging for further development.
ary '87: 71. Our patient presents with petit mal epilepsy, X-linked ichtiosis and mental retardation. Neurological and dermatological symptoms, e.g. phakomatosis, are specific for neurocutaneous diseases. There is also a large group that presents with ichtiosis and varying neurological problems. When ichtiosis and mental retardation are involved, one has to consider different syndromes. In the Sj6gren-Larssen Syndrome there is also a spastic paraplegia. In the Refsum disease (high phytanic acidataxia, hypertrophic polyneuropathy and retinitis pigmentosa are included. In the Rud Syndrome ichtiosis and mental retardation are associated with epilepsy whereas in the Netherton Syndrome there is a fourth symptom, trichorrhexis invaginata (bamboo hair). There are a lot more syndromes that present with ichtiosis and mental retardation, like the Passwell Syndrome, Senter Syndrome, but these are less frequent. According to the symptoms our patient presented, the diagnosis of Rud Syndrome was made. The major symptoms are epilepsy, mental retardation and ichtiosis. It is a genetic heterogenous syndrome with a varying degree of mental retardation and "epilepsy" which is sometimes only characterized by paroxisms on the EEG. Rud originally described a patient with also hypogonadism, hyperchromic anemia and polyneuritis. Nystagmus, anosmia, neurosensory deafness and recurrent airway infections are also mentioned but less frequently. Probably this syndrome will be better described when more features are available.
Infarctions of the thalamic region: Identification of five clinical pictures and corresponding C T-findings. G. V a n h o o r e n , I. D e h a e n e , M. van Zandijcke, M. M a r c h a u (Brugge) Infarctions in the thalamic area give rise to specific, clinical pictures. They can be correlated to typical hypodensities on CT-scan. Anterior choroidal artery territory infarction 298
causes contralateral hemiparesis, visual field defects and hemineglect. On CT-scan a laterothalamic hypodensity, extending in the capsula interna is noted. The thalamogeniculate artery irrigates the posterolateral thalamus, and infarction in this area causes pure sensory stroke. The
tuberothalamic infarction gives rise to longlasting neuropsychological deficit and is seen on CT pictures as a hypodensity in the anterolateral thalamus. A paramedian hypodensity in the posterior thalamic region is caused by occlusion of the thalamoperforating artery. A transient distur-
bance of consciousness with memory deficit and vertical eye movement abnormalities are observed. As an illustration, we presented four patients. We are looking for the fifth infarction type, in the anterior nucleus region, expected to be seen as an anteromedial hypodensity on CT-scan.
The syndrome of the anterior choroidal artery.
J. D e Bleecker, J. de R e u c k , G. Vingerhoets, W. Ceusters, and E. Thiery (Ghent) The complete clinical picture after infarction in the anterior choroidal artery territory combines the triad of hemiplegia, hemianesthesia, and homonymous hemianopia. CT and NMR examination reveal an area of reduced density in the posterior limb of the internal capsula, sparing the thalamus medially, and reaching the top of the globus pallidus laterally. Incomplete clinical forms are due to frequent individual differences in the vascular supply of this artery,
the hemianopia being the least constant symptom. A classic case of leftsided anterior choroidal artery syndrome is presented, with clear demonstration of the infarcted territory by CT scan and NMR. Attention is drawn to the speech disturbances as well as to the visual neglect. Neuro-ophthalmologic examination reveals dysfunction of the cortico-pontine projections. Neuropsychological testing is compatible with a left thalamofrontal syndrome. The clinical syndrome is correlated to the vascular anatomy of the infarct.
Pseudotumoral intracerebral hematoma due to cerebral amyioid angiopathy.
F. van Calenbergh, R. D o m , J. Goffin, C. Plets, and R. van den Bergh (Leuven) We describe the case history of a 72-year-old woman, who presented with progressive mental deterioration, headache and a mild left hemiparesis. These symptoms had an insidious onset and evolved over five weeks. CT scan and angiography were suggestive of a right parietal brain tumor, with a small hemorrhagic component. During the operation, we also had the impression of a gliomatous tumor with a central necrotic cyst. Pathological examination, how-
ever, showed an old hematoma with a fibrous capsule and neovascularisation. In the wall of these vessels, amyloid was revealed by Congo red staining. Small leptomeningeal vessels showed the same changes. The pseudotumoral or "chronic expanding" intracerebral hematoma (ICH) has only recently received special interest in the neurosurgical literature. As far as we know, our patient is the first, in whom cerebral amyloid angiopathy was found as an aetiological factor in this type of ICH.
Statistical analysis of 153 cases of meningitis.
J. de Koninck, and J. de R e u c k (Ghent) A retrospective study of patients with meningitis treated at the University Hospital of Ghent during the period 1980-1985 is presented.
Patients with immunodeficiency or C.S.F.leakage were excluded. A predominance of males was found for aseptic as well as for bacterial meningitis. The highest incidence of aseptic meningitis was seen in the age group between 299
20 and 30 years, while the occurrence of bacterial meningitis was less frequent in that age group. A summerpeak, typical for enteroviral infections was found for the majority of cases of aseptic meningitis. During this summerpeak a smaller percentage of patients received antibiotics before admission. Pre-adrnission antibiotic treatment was responsible for a negative bacterial C.S.F. culture in all cases and had also a
slight influence on the Wlc. count in the C.S.F. Pneumococcus meningitis appeared to be the most frequent and the most dangerous form in our adult population with 55% occurrence of convulsions in contrast to 3% for the other types of meningitis. The length of hospital stay was not significantly longer for bacterial meningitis than for aseptic meningitis and was not influenced by the pre-admission antibiotic therapy.
"La meningite du charcutier". W. Ceusters, Ph. van O o t e g h e m , A. V a k a e t , G. Claeys, J. Verschraegen, and J. de R e u c k ( G h e n t ) A case of an acute, purulent meningitis caused by Streptococcus suis type II is presented. This agent is known since 1959 when it was first isolated from septicaemic pigs. Since 1968 it has been recognized as a pathogenic agent for man,
causing septicaemia and meningitis. About 75% of the patients with meningitis develop an irreversible sensorineural hearing loss. Making the exact diagnosis can be a problem because most available commercial biochemical kits allow only to classify the agent as Streptococcus viridans. Except for the hearingloss our patient was cured succesfully with 12 gram of ampicilline daily.
Neurological complications of mononucleosis infectiosa. M. van O r s h o v e n and F. van Orshoven (Aalst). Following an influenzal illness with fever and cervical adenopathy, a 26-year-old young man developed disturbances of speaking, writing and balance, and vomiting. Serology proved the clinical diagnosis of mononucleosis infectiosa (M. I.): a positive Paul & Bunnell reaction, positive IgG (1:620) and IgM antibodies against Ebstein-Barr Viral Capsid Antigen (EBV-CA). The neurological examination demonstrated a bilateral cerebellar syndrome with long tract signs (piramidal reflexes in the legs with Chaddock reflexes in extension). CT of the brain, EEG and evoked potentials (VEP - B A E R SSEP n. medianus and n. saphenus) were normal. Lumbar puncture showed a clear liquid with 4 leucocytes, normal protein (32 mg%) with normal agarelectroforesis and
eiectrofocusing. EBV-CA IgM and IgG in the CSF were negative. Neurologic symptoms resolved in 3 months. Neurological complications of MI are well known: besides a cerebellar syndrome, a serous meningitis, encephalitis, epilepsy, myelitis, Guillain-Barr6 syndrome, and a cranial or peripheral mononeuritis have been described. Sometimes the infectious manifestations of MI may be absent. The prognosis is usually good. The pathological mechanism is uncertain: both the hypothesis of a direct viral agression of the nervous system and the possibility of a postinfectious allergic reaction have been postulated. Our observations would rather favour the second hypothesis. Anyway, examination of the specific EBV serology is worthwhile when confronted with a young person with neurological symptoms.
Poliomyelitis in an airline pilot and the need for vaccination of travellers. P. G o u b a n , H. Carton, H. Taelman, E. D r u y t s - V o e t s , J. D e Smyter (Leuven, Antwerp, Brussels) 300
Since the widespread use of polio vaccination, poliomyelitis has virtually disappeared from industrialized countries, but rare localized
outbreaks and sporadic cases still occur. A number of individuals in these widely immunized communities lack protective immunity against one or more poliovirus types. Such persons are not exposed due to the lack of circulation of poliovirus in these populations (herd im-
munity). Nevertheless, they are at high risk of paralytic poliomyelitis when they travel to endemic areas. We present a case which illustrates this point and emphasizes the importance of polio vaccination for travellers to developing countries.
Polyneuropathy due to lithium toxicity.
F. Piessens, B. M e y e r , and V. van den Bergh (Duffel, Leuven) A 50-year-old woman developed a severe hypertonic-hypokinetic extrapyramidal syndrome with muscle rigidity following addition of 1000 mg Haloperidol to a maintenance dose of Lithium carbonate 600 rag. Serum lithium level at that moment was within therapeutic range. One week after withdrawal of these drugs the hypertonic-hypokinetic
syndrome was replaced by dyskinetic movements, myoclonic jerks and head tremor. At the same time a flacid quadriparesis with absent tendon reflexes in the lower limbs became apparent. Electrodiagnostic studies suggested axonal lesions. Recovery of the polyneuropathy was very slow and still is incomplete after 3 months. As suggested by this and other studies serum lithium level may be a reliable parameter of neurotoxicity. A possible interaction between haloperidol and lithium is discussed.
Wilson's disease.
M. Marchau, T.U. H o o g e n r a a d , M. van Zandijcke, and I. D e h a e n e (Brugge, Utrecht) Wilson's disease is an autosomal recessive inherited disease. The major symptoms are due to deposits of copper in liver, brain and cornea. The urinary excretion of copper is elevated. Ceruloplasmin, the serum enzyme that binds copper, is defectively synthesized according to
some authors. Others state that the primary defect is intrahepatic and concerns the copper metabolism. This is followed by an impairment of ceruloplasmin synthesis. The neurologic signs are extrapyramidal (tremor, dysphagia and dysarthria, rigidity). With the use of CT scan and NMR typical abnormalities can be demonstrated: generalized atrophy of brain and brain stem, hypodensities in the basalganglia and cerebellar nuclei.
Wilson's disease: Treatment of the paradoxal reaction on penicillamine with oral zinc sulfate.
T.U. H o o g e n r a a d (Utrecht, Brugge)
and
M.
Marchau
A paradoxal reaction on penicillamine in Wilson's disease is the worsening of neurological signs and symptoms, although a good cupriuresis is found. It is one of the most feared complications of penicillamine therapy of Wilson's disease. Three patients with extrapyrami-
dal symptoms due to Wilson's disease were deteriorating shortly after the penicillamine therapy started. All three improved after stopping penicillamine and administration of oral zinc sulfate. In patients with neurologic symptoms due to hereditary copper toxicosis one should choose treatment with oral zinc sulfate.
301
Somatosensory evoked potentials in patients with Huntington's disease. E. Triau, M. Malfroid, R. D o m , F. Baro (Leuven) We studied the use of somatosensory evoked potentials in 25 patients with Huntington's Chorea, compared with a control group of healthy volunteers. On the response to median stimulation, no significant alteration of N20 latency was found, whereas there was a significant
decrease in amplitude of the primary cortical response N20/P25 in Huntington patients, in comparison to the control group. Our results confirm the findings of previous reports. Possible underlying pathological changes are discussed. The use of somatosensory evoked potentials on subjects at risk for Huntington's disease is still under investigation.
Single fibre EMG versus repetitive nerve stimulations in Myasthenia gravis. R. Mercelis, P. de Jonghe, J. Gheuens, and J.J. Martin ( A n t w e r p e n ) We performed single fibre EMG of the extensor digitorum communis (EDC) in 25 patients with defective neuromuscular transmission (23 myasthenia gravis, 1 congenital myasthenia, 1 Eaton-Lambert myasthenic syndrome). The study was considered abnormal if an increased jitter was found in 10% or more of the potential pairs or if the mean jitter of all potential pairs was increased. In most of the patients, repetitive nerve stimulations were done in the abductor digiti minimi (ADM) and in the trapezius muscle. Records were made at 3/sec before and until 4 minutes after 30 sec of maximal voluntary activation of the muscle. A decrement of 8% or
more between the 1st and the 4th or the 5th response was taken as abnormal. The "doublestep" test with ischemia of the forearm was not done. Singel fibre EMG was abnormal in all but 2 patients. One of these patients was in clinical remission of ocular myasthenia gravis. She had an increased jitter in 1 out of 20 potential pairs and the mean jitter was on the border of normality. The second patient with normal jitter in the EDC suffered from unilateral ptosis and had an increased jitter in the orbicularis oculi. Repetitive nerve stimulations were abnormal in 7 out of 23 patients in the ADM and in 11 out of 21 in the trapezius. Single fibre EMG is a valuable aid in the detection of abnormality of neuromuscular transmission.
Evaluation of computer assisted EEG-analysis in clinical practice. P. de Wit (Zoersel) Numerical analysis of EEG signals can be performed with a Personal Computer based EEGAnalysis-System. The communication deals with some clinical aspects of mathematical
302
models used in the computer system. The use of quantitative EEG-analysis in combination with visual analysis increases the value of EEG examination in clinical situation and is encouraging for further development.