The Gordon-Hey reflex.

Nowadays, a neurologist, even in many years of practice, rarely sees a patient with Sydenham's chorea. Things were quite different during the period +/- 1850 - +/- 1950, when 'chorea minor' was the subject of hundreds of publications. In those days, the practising neurologist stood a good chance of coming across patients with such a degree of muscular hypotonia (a characteristic feature of severe Sydenham's) that he was inclined to infer the presence of paralysis. Those instances used to be denoted as chorea mollis or chorea paralytica. According to textbooks then considered to be authoritative, any doubt regarding the diagnosis of Sydenham's chorea was largely removed if one succeeded in eliciting Gordon-Hey's reflex. This eponym turns out to be as fascinating as the question of its still largely unclarified pathophysiology. In spite of that, mention of the reflex has wholly disappeared from the textbooks of neurology since circa forty years.

[Migrane treatment in the Netherlands in the early twentieth century]. / De behandeling van migraine in Nederland in het begin van de 20e eeuw.

The treatment of migraine in the Netherlands underwent important changes in the beginning of the 20th century. Several factors played a role, including the development of analgesics (acetylsalicylic acid, phenacetin, and fampridine++) at the end of the 19th century. The introduction of phenobarbital for the treatment of epilepsy in 1912 resulted in prescription of this drug for migraine, because of the supposed similarities between both afflictions. Although ergotamine had been reported for the treatment of headache at the end of the 19th century and been recommended again during the 1920s, it appeared in the Dutch literature only at the end of the 1930s. Research abroad showed this drug to have vasoconstrictive properties in migraine, again confirming the vasogenic origin of the affliction. This is striking as vasodilating drugs had been prescribed for several years. The non-medical treatment largely remained as previously, notably the prescription of diets. The relation between migraine and anaphylaxis, based on research by the French school, was also investigated in the Netherlands and resulted in the prescription of diets to immunize against the proteins involved.

A century of Dutch neurology.

The Netherlands Society of Neurology evolved from the Society of Psychiatry founded in 1871. The name was changed into Netherlands Society of Psychiatry and Neurology (NSPN) in 1897. In the same year, the word neurology was also added to the name of the journal. The Society steadily blossomed, but in 1909 the first signs of dissatisfaction occurred: the Amsterdam Neurologists Society was founded. A few split-offs would follow. The number of members of the NSPN increased from 205 in 1920 to 585 in 1960. In the early 1960s, the Society was reorganised and would consist of two sections, one for psychiatry and one for neurology. However, this would not last, as a full separation was established in 1974. For several reasons, the name of the journal was changed four times until it assumed its present name in 1974. The 100th volume of CNN was not published, as expected. in 1996, but in 1998, because of two skipped publication years, one during WWII and another in the 1970s. During the last decades of the nineteenth century, teaching of neurology was mostly given within the frame of psychiatry, following the German tradition of 'brainpsychiatry' (organic or biologic psychiatry). The first official chair of psychiatry was founded at Utrecht, 1893 (Winkler). In Amsterdam, private teachers such as Delprat taught 'electro-therapy and nervous diseases' since the 1880s. The first extraordinary chair of neurology and electrotherapy was founded for his successor, Wertheim Salomonson in 1899. The first university clinic for psychiatry and neurology started at the Amsterdam Municipal University, when Winkler became professor of psychiatry and neurology in Amsterdam in 1896. Around the turn of the century, chairs of psychiatry and neurology were also founded in Groningen and Leiden. Separate chairs for neurology and psychiatry appeared in Amsterdam in 1923 and in Utrecht in 1936. Following an initiative of Brouwer, the first neurological university clinic opened its doors in Amsterdam in 1929. In the 20th century, a number specialised peripheral neurological clinics and epilepsy institutes were founded. In 1909, the the Central Institute for Brain Research was established in Amsterdam.

Acute transverse myelitis. A localized form of postinfectious encephalomyelitis.

We analysed the clinical, imaging, electrophysiological, laboratory findings, course and prognostic factors in 31 patients with acute transverse myelitis (20 men and 11 women; mean age, 30 years; range, 18-51 years). All patients were assessed for maximal clinical deficit 'deficit score'; pattern-shift visual, auditory and somatosensory evoked potentials were measured, CSF was examined, and neuroimaging of the spinal cord and brain (MRI or CT myelography) was carried out. The myelitis was preceded by febrile illness in 25 (81%) of the patients. The site of the lesion was cervical in 11 (36%), upper thoracic in two (6%), lower thoracic in 16 (52%). MRI of the spinal cord was abnormal in 10 out of the 20 patients examined (50%); in the remaining 11 patients, only CT was carried out and it was normal in all of them. Somatosensory evoked potentials were abnormal in 19 (61%), while pattern-shift visual and brainstem auditory evoked potentials were normal in all patients. CSF was abnormal in 94% of patients with pleocytosis, increased protein or both. Eighteen patients (58%) had good outcome. All patients had monophasic illness. Three variables have emerged as being associated with significant worsening of the outcome: (i) abnormal somatosensory evoked potentials; (ii) abnormal imaging and (iii) high 'deficit score' at onset. Acute transverse myelitis affects a complete segment of the spinal cord, is monophasic and represents a localized form of postinfectious acute encephalomyelitis.

The use of and familiarity with neurological eponyms at the close of the 20th century by Dutch neurologists. An inventory.

In order to get an impression on the opinion of the use of neurological eponyms we sent questionnaires on 205 eponyms to 850 members of the Netherlands Association for Neurology. The responses by 256 (30%) were analyzed. A positive correlation was found between age and the knowledge of eponyms. The best known eponyms belong to category II (tests and manoeuvres). Surprisingly, many of the responding colleagues did not prefer descriptive terms to eponyms: 57% of the eponyms were preferred by more than 50% of the respondents.

Genetic and biochemical impairment of mitochondrial complex I activity in a family with Leber hereditary optic neuropathy and hereditary spastic dystonia.

A rare form of Leber hereditary optic neuropathy (LHON) that is associated with hereditary spastic dystonia has been studied in a large Dutch family. Neuropathy and ophthalmological lesions were present together in some family members, whereas only one type of abnormality was found in others. mtDNA mutations previously reported in LHON were not present. Sequence analysis of the protein-coding mitochondrial genes revealed two previously unreported mtDNA mutations. A heteroplasmic A-->G transition at nucleotide position 11696 in the ND4 gene resulted in the substitution of an isoleucine for valine at amino acid position 312. A second mutation, a homoplasmic T-->A transition at nucleotide position 14596 in the ND6 gene, resulted in the substitution of a methionine for the isoleucine at amino acid residue 26. Biochemical analysis of a muscle biopsy revealed a severe complex I deficiency, providing a link between these unique mtDNA mutations and this rare, complex phenotype including Leber optic neuropathy.

Neuroprotective effect of selenium on iminodipropionitrile-induced toxicity.

The present investigation was undertaken to study the effect of selenium on experimental dyskinesia in rats. The movement disorders were produced in rats by intraperitoneal administration of iminodipropionitrile (IDPN) in the dose of 100 mg/kg per day for 12 days. Selenious acid was administered daily 30 minutes before IDPN in the doses of 5 mumol/kg, 10 mumol/kg and 20 mumol/kg bodyweight in three different groups of rats. Animals were observed daily for any neurobehavioral changes including circling, backwalking, head weaving and twitching. Immediately after behavioral studies, blood and brain specimens were collected for analysis of thiobarbituric acid reactive substances (TBARS) to measure the extent of free radical production. Our results showed that concurrent use of selenium significantly inhibited IDPN-induced neurobehavioral changes in a dose-dependent manner. Treatment of rats with selenium also reduced the TBARS production in blood and different regions of brain. These findings suggest that selenium attenuates the IDPN-induced neurotoxicity by inhibiting lipid peroxidation.

Non-progressive familial congenital cerebellar hypoplasia.

A syndrome is reported of congenital non-progressive, gradually slightly improving, ataxia in 3 out of 5 male sibs, issues of a first-order consanguineous mating. Additional characteristic features included: moderate microcephaly, generalised muscle weakness and hypotonia, nystagmus, and moderate mental retardation. A pyramidal syndrome of hyperreflexia and Babinski signs, without any spasticity, became manifest in the 2nd or 3rd year of life. In all three, the caudal part of the vermis was absent, the enlarged IVth ventricle opening up via Magendie's foramen into the cisterna magna. The middle and rostral vermian parts as well as the sagittal paravermian parts of the cerebellar hemispheres were hypoplastic. The differential diagnosis of this syndrome is analysed and the developmental pathogenetic mechanisms likely to produce the typifying distribution of aplasia are indicated.

Dipyridamole attenuates the development of iminodipropionitrile-induced dyskinetic abnormalities in rats.

The present investigation was undertaken to study the effect of dipyridamole on experimental dyskinesia in rats. The movement disorders were produced by intraperitoneal administration of iminodipropionitrile (IDPN) in the dose of 100 mg/kg per day for 12 days. Dipyridamole was administered orally, daily 30 min before IDPN in the doses of 0.5 g/kg, 1 g/kg, and 1.5 g/kg bodyweight in three different groups of rats. Twenty-four hours after the last dose of IDPN, animals were observed for neurobehavioral changes including vertical and horizontal head weaving, circling, backwalking, grip strength, and righting reflex. Immediately after behavioral studies brain specimens were collected for analysis of vitamin E, conjugated dienes, and lipid hydroperoxides as indices of oxygen-derived free radical (OFR) production. Our results showed that concurrent use of dipyridamole significantly protected rats against IDPN-induced neurobehavioral changes in a dose-dependent manner. Treatment of rats with dipyridamole inhibited IDPN-induced decrease of vitamin E and increase in conjugated dienes and lipid hydroperoxides in brain. These findings suggest the involvement of OFR in dipyridamole induced protection against the development of IDPN dyskinesia. Further studies are warranted to determine the role of dipyridamole as a prophylactic agent against the drug induced dyskinetic abnormalities.

Effect of selenium and vitamin E on iminodipropionitrile induced dyskinesia in rats.

The present study was undertaken to determine the effect of combination of selenium and vitamin E on experimentally induced dyskinesia in rats. The dyskinetic syndrome was produced in 4 groups of 6 male rats each weighing 250-300g by intraperitoneal (ip) administration of iminodipropionitrile (IDPN) in doses of 100 mg/kg body weight daily for 12 days. A group of 6 rats (group 1) served as control and received normal saline only. The rats in group 2 (IDPN only) received normal saline (ip) 30 minutes before the administration of IDPN. The animals in groups 3, 4 and 5 received selenous acid (5 mumol/kg), vitamin E (500 mg/kg p.o.) and a combination of selenous acid and vitamin E respectively, daily, 30 minutes before IDPN for 12 days. Twenty four hours after the last dose of IDPN, the dyskinetic behavior including vertical head movements (retrocollis), horizontal head movements (laterocollis), circling and backwalking of each rat was studied for a period of 10 minutes. Immediately after behavioral studies, the animals were sacrificed and brains were dissected out for the analysis of conjugated dienes, lipid hydroperoxides and vitamin E. The results of this study showed that treatment of rats with IDPN only for 12 days produced dyskinetic syndrome in all the rats characterized by vertical and horizontal head movements, circling and backwalking. Concomitant treatment of rats with vitamin E and selenium individually reduced IDPN induced dyskinesia, and the symptoms were almost completely absent when the combination of these two agents was used.(ABSTRACT TRUNCATED AT 250 WORDS)

Duration of illness in Huntington's disease is not related to age at onset.

The age at onset and duration of illness were studied in patients with Huntington's disease in the Leiden Roster which at 1 July 1990 contained 2787 patients. Of 1106 patients, 800 deceased and 306 alive, the age at onset was known. The median duration was 16.2 (range 2-45) years. In contrast to the current opinion, the median duration was independent of the age of onset. The median duration in juvenile Huntington's disease was 17.1 years, which is much longer than reported in the literature, and comparable with the categories for the age of onset of 20-34 and 35-49 years. Only in the group where onset was over 50 years of age was the median duration somewhat shorter (15.6 years), which can be ascribed to unrelated causes of death. As age of onset and duration of illness are not related, at least two mechanisms to determine the clinical course have to be postulated: one for age of onset and another for duration of illness. Duration was shorter for males, especially for those with an affected father.

Altered postural reflexes in Parkinson's disease: a reverse hypothesis.

In subjects standing on a movable platform, sudden dorsiflexion of the ankle joint elicits a set of reflexes in leg muscles. These responses include a short latency (SL) and medium latency (ML) stretch reflex in the gastrocnemius muscle and a distal to proximal innervation sequence of long latency (LL) reflexes in the shortened tibialis anterior and vastus lateralis muscles. Because of their role in maintaining upright stance these responses have been termed postural reflexes. In patients with Parkinson's disease (PD), the following abnormalities have been described: 1) enhanced ML-amplitudes; 2) a reversed LL innervation sequence; and 3) delayed onset latencies. These abnormalities are thought to be due to defective motor programming and disturbed control of spinal and supraspinal reflex centers by basal ganglia circuits. The altered reflexes have been held responsible for some of the clinical features of PD, including balance impairment and rigidity. In this paper, we argue the reverse hypothesis that postural reflexes are essentially normal in PD, and that the observed alterations are at least in part consequence rather than cause of balance impairment, the stooped parkinsonian posture and rigidity of PD patients.

Hereditary spastic dystonia with Leber's hereditary optic neuropathy: neuropathological findings.

Neuropathological findings in a 59-year-old male case of hereditary spastic dystonia with Leber's hereditary optic atrophy included: marked depletion of myelinated nerve fibres in the posterior funiculi, corticopontine tracts and striatum; practically complete neuronal depletion in the putamen and lateral part of the caudate, and mild cell loss in the substantia nigra. The putamina had changed into a spongy fibrillary scar, the pallidal fibres and laminae were practically all degenerated. Moreover, there was generalised mild fibre degeneration of the white matter. The optic nerve showed marked, predominantly central, loss of nerve fibres with demyelination.

The hypothalamic lateral tuberal nucleus and the characteristics of neuronal loss in Huntington's disease.

Neurons in the hypothalamic lateral tuberal nucleus (NTL) were counted in 16 Huntington's disease (HD) patients and 12 controls. The control range was 47,500-71,700. In the HD cases the number ranged from 2,800 to 40,600. The log-transformed counts of the HD patients correlated closely with age-at-death (r = 0.66, P less than 0.01) and age-at-onset (r = 0.78, P less than 0.001), but not with duration of disease, nor with the severity of the neostriatal changes. Because of its vulnerability to the effects of the HD gene and its simplicity, the NTL seems fit to study the characteristics of neuronal death in HD.