EAN guideline on palliative care of people with severe, progressive multiple sclerosis.

BACKGROUND AND PURPOSE: Patients with severe, progressive multiple sclerosis (MS) have complex physical and psychosocial needs, typically over several years. Few treatment options are available to prevent or delay further clinical worsening in this population. The objective was to develop an evidence-based clinical practice guideline for the palliative care of patients with severe, progressive MS. METHODS: This guideline was developed using the Grading of Recommendations Assessment, Development and Evaluation methodology. Formulation of the clinical questions was performed in the Patients-Intervention-Comparator-Outcome format, involving patients, carers and healthcare professionals (HPs). No uniform definition of severe MS exists: in this guideline, constant bilateral support required to walk 20 m without resting (Expanded Disability Status Scale score > 6.0) or higher disability is referred to. When evidence was lacking for this population, recommendations were formulated using indirect evidence or good practice statements were devised. RESULTS: Ten clinical questions were formulated. They encompassed general and specialist palliative care, advance care planning, discussing with HPs the patient's wish to hasten death, symptom management, multidisciplinary rehabilitation, interventions for caregivers and interventions for HPs. A total of 34 recommendations (33 weak, 1 strong) and seven good practice statements were devised. CONCLUSIONS: The provision of home-based palliative care (either general or specialist) is recommended with weak strength for patients with severe, progressive MS. Further research on the integration of palliative care and MS care is needed. Areas that currently lack evidence of efficacy in this population include advance care planning, the management of symptoms such as fatigue and mood problems, and interventions for caregivers and HPs.

Pitfals in recognition and management of trigeminal neuralgia.

BACKGROUND: Trigeminal neuralgia (TN) is a severe, disabling form of painful cranial neuropathy. Even though TN has a typical clinical picture, diagnosis it is often missed or delayed in clinical practice. In order to investigate the occurrence of diagnostic and therapeutic errors in TN, we studied 102 patients suffering from TN recruited through a multicentric survey. METHODS: We performed a Pubmed database search on errors and pittfalls in TN diagnosis and management. Then, patients with TN were consecutively enrolled in the period from February 2017 to October 2019, by several European Headache Centers participating in the study, following a call of the Headache and Pain Scientific Panels of the European Academy of Neurology (EAN). Diagnosis of Classical Trigeminal Neuralgia (CTN) was made according to the International Headache Society (IHS) criteria (Tölle et al., Pain Pract 6:153-160, 2006). All the patients were evaluated using telephone/frontal interviews conducted by headache/pain specialists using an ad hoc questionnaire. RESULTS: A number of 102 patients were recruited, mostly females (F:M ratio 2.64:1). Eighty-six percent of the patients consulted a physician at the time they experienced the first pain attacks. Specialists consulted before TN diagnosis were: primary care physicians (PCP) (43.1%), dentists (in 30.4%), otorhinolaryngologists (3.9%), neurosurgeons (3.9%), neurologists or headache specialists (14.7%), others (8%). The final diagnosis was made mainly by a neurologist or headache specialist (85.3%), and the mean interval between the disease onset and the diagnosis made by a specialist was 10.8 ± 21.2 months. The "diagnostic delay" was 7.2 ± 12.5 months, and misdiagnoses at first consultation were found in 42.1% of cases. Instrumental and laboratory investigations were carried out in 93.1% of the patients before the final diagnosis of TN. CONCLUSION: While TN has typical features and it is well defined by the available international diagnostic criteria, it is still frequently misdiagnosed and mistreated. There is a need to improve the neurological knowledge in order to promptly recognize the clinical picture of TN and properly adhere to the specific guidelines. This may result in a favorable outcome for patients, whose quality of life is usually severely impaired.

Patient and caregiver involvement in the formulation of guideline questions: findings from the European Academy of Neurology guideline on palliative care of people with severe multiple sclerosis.

BACKGROUND AND PURPOSE: Patient and public involvement in clinical practice guideline development is recommended to increase guideline trustworthiness and relevance. The aim was to engage multiple sclerosis (MS) patients and caregivers in the definition of the key questions to be answered in the European Academy of Neurology guideline on palliative care of people with severe MS. METHODS: A mixed methods approach was used: an international online survey launched by the national MS societies of eight countries, after pilot testing/debriefing on 20 MS patients and 18 caregivers, focus group meetings of Italian and German MS patients and caregivers. RESULTS: Of 1199 participants, 951 (79%) completed the whole online survey and 934 from seven countries were analysed: 751 (80%) were MS patients (74% women, mean age 46.1) and 183 (20%) were caregivers (36% spouses/partners, 72% women, mean age 47.4). Participants agreed/strongly agreed on inclusion of the nine pre-specified topics (from 89% for 'advance care planning' to 98% for 'multidisciplinary rehabilitation'), and <5% replied 'I prefer not to answer' to any topic. There were 569 free comments: 182 (32%) on the pre-specified topics, 227 (40%) on additional topics (16 guideline-pertinent) and 160 (28%) on outcomes. Five focus group meetings (three of MS patients, two of caregivers, and overall 35 participants) corroborated the survey findings. In addition, they allowed an explanation of the guideline production process and the exploration of patient-important outcomes and of taxing issues. CONCLUSIONS: Multiple sclerosis patient and caregiver involvement was resource and time intensive, but rewarding. It was the key for the formulation of the 10 guideline questions and for the identification of patient-important outcomes.

Management of dystonia in Europe: a survey of the European network for the study of the dystonia syndromes.

BACKGROUND AND PURPOSE: Dystonia is difficult to recognize due to its large phenomenological complexity. Thus, the use of experts in dystonia is essential for better recognition and management of dystonia syndromes (DS). Our aim was to document managing strategies, facilities and expertise available in various European countries in order to identify which measures should be implemented to improve the management of DS. METHODS: A survey was conducted, funded by the Cooperation in Science and Technology, via the management committee of the European network for the study of DS, which is formed from representatives of the 24 countries involved. RESULTS: Lack of specific training in dystonia by general neurologists, general practitioners as well as other allied health professionals was universal in all countries surveyed. Genetic testing for rare dystonia mutations is not readily available in a significant number of countries and neurophysiological studies are difficult to perform due to a lack of experts in this field of movement disorders. Tetrabenazine is only readily available for treatment of dystonia in half of the surveyed countries. Deep brain stimulation is available in three-quarters of the countries, but other surgical procedures are only available in one-quarter of countries. CONCLUSIONS: Internationally, collaboration in training, advanced diagnosis, treatment and research of DS and, locally, in each country the creation of multidisciplinary teams for the management of dystonia patients could provide the basis for improving all aspects of dystonia management across Europe.

Real-world effectiveness of fingolimod in patients with multiple sclerosis in Bulgaria. / Eficacia en la vida real del fingolimod en pacientes con esclerosis múltiple en Bulgaria.

INTRODUCTION: Fingolimod, a sphingosine-1-phosphate receptor agonist used for the treatment of multiple sclerosis (MS). Our goal was to assess the impact of fingolimod on quality of life in patients with relapsing-remitting multiple sclerosis (RRMS) after 2 years of treatment in this real-world study. PATIENTS AND METHODS: This was a 2-year, prospective, observational study conducted in Bulgaria in RRMS patients treated with fingolimod. Quality of life was assessed using the Bulgarian-language version of the MSQoL-54 scale. The primary endpoint was the change from baseline in the MSQoL-54 score after 2 years of treatment. Secondary endpoints included the change from baseline in the MSQoL-54 score after one year of treatment, furthermore the assessment of depression level using the Hamilton D-17 score. RESULTS: A total of 87 eligible patients were included in the study with a mean age of 38.7 ± 8.45 years. The median Expanded Disability Status Scale (EDSS) score was 3.5 points. We found statistically significant improvement in 10 subscales at month 12 and in seven subscales at month 24. The mental health composite score increased from 64.0 ± 16.69 points to 67.5 ± 15.94 points at month 24 (p = 0.012). The physical health composite score increased from 61.7 ± 17.61 to 66.3 ± 16.70 (p = 0.001). Depression level measured by the HAM-D17 decreased significantly by month 12 and month 24. The EDSS score decreased or remained stable in more than half of the patients (61.6%). We detected better quality of life in patients with a lower EDSS score. CONCLUSIONS: Quality of life scores and the depression level are improved in RRMS patients treated with fingolimod over 2 years in real-life setting.

TITLE: Eficacia en la vida real del fingolimod en pacientes con esclerosis múltiple en Bulgaria.Introducción. El fingolimod es un agonista del receptor de esfingosina-1-fosfato utilizado para el tratamiento de la esclerosis múltiple (EM). Nuestro objetivo era evaluar los resultados del fingolimod en la calidad de vida de los pacientes con EM recurrente-remitente tras dos años de tratamiento en este estudio de la vida real. Pacientes y métodos. Se trata de un estudio observacional prospectivo de dos años de duración realizado en Bulgaria en pacientes con EM recurrente-remitente tratados con fingolimod. Se evaluó la calidad de vida mediante la versión en búlgaro de la escala Multiple Sclerosis Quality of Life-54 (MSQoL-54). El criterio de valoración principal fue el cambio respecto al valor inicial en la puntuación en la MSQoL-54 tras dos años de tratamiento. Los criterios de valoración secundarios fueron el cambio respecto al valor inicial en la puntuación en la MSQoL-54 tras un año de tratamiento, además de la evaluación del nivel de depresión mediante la puntuación de la escala de puntuación de la depresión de Hamilton (HAM-D17). Resultados. En el estudio se incluyó a 87 pacientes elegibles con una edad media de 38,7 ± 8,45 años. La mediana de la puntuación en la Expanded Disability Status Scale (EDSS) fue de 3,5 puntos. Se halló una mejora estadísticamente significativa en 10 subescalas en el mes 12 y en siete subescalas en el mes 24. La puntuación combinada de salud mental aumentó de 64 ± 16,69 puntos a 67,5 ± 15,94 puntos en el mes 24 (p = 0,012). La puntuación combinada de salud física aumentó de 61,7 ± 17,61 a 66,3 ± 16,7 (p = 0,001). El nivel de depresión medido por la HAM-D17 disminuyó considerablemente en el mes 12 y en el mes 24. La puntuación de la EDSS disminuyó o se mantuvo estable en más de la mitad de los pacientes (61,6%). Detectamos una mejor calidad de vida en los pacientes con una puntuación más baja en la EDSS. Conclusiones. Las puntuaciones de calidad de vida y el nivel de depresión mejoraron en los pacientes con EM recurrente-remitente tratados con fingolimod durante dos años en un entorno real.

Evaluation of diagnostic and prognostic value of clinical characteristics of migraine and tension type headache included in the diagnostic criteria for children and adolescents in International Classification of Headache Disorders--second edition.

UNLABELLED: Data about the sensitivity and the specificity of the items included in the diagnostic criteria for migraine and tension type headache (TTH) in children is limited and sometimes controversial. AIM: To evaluate the diagnostic value of characteristics of migraine and TTH included in the diagnostic criteria of ICHD-II and according to results to suggest additional criteria for diagnostic differentiation of primary paediatric headache. PATIENTS AND METHODS: The investigation consisted of an epidemiological school-based study (1029 pupils completed the study and 412 had chronic or recurrent headache) and a clinical study conducted in Paediatric Neurology Ward and outpatient clinic (203 patients with chronic or recurrent headache). Inclusion criterion was at least two episodes of headache during the last year. Exclusion criteria were: headache occurring only during acute infections; withdrawal of informed consent. ICHD - II was used to classify headache. The diagnostic value of characteristics of migraine and TTH was measured using sensitivity, specificity, odds ratio and area under receiver operating characteristic curve (AUC). RESULTS: Regarding the AUC, the best diagnostic items for migraine are: moderate or severe intensity or only severe intensity, pain aggravation by physical activity, pulsating quality, respectively, for TTH - no photophobia, no nausea, no aggravation by physical activity, mild or moderate intensity and non-pulsating quality. The most significant symptom for increasing the migraine risk was pulsating pain and the most significant items for TTH risk were no photophobia, bilateral location and no nausea. Family history of migraine also increased migraine risk and could be either included in the diagnostic criteria for migraine or recommended as additional item in differentiating migraine and TTH with overlapping diagnostic criteria. According to AUC, we could recommend changing the content of the item of intensity for migraine as only severe intensity.

Clinical and electromyographic characteristics of tremor in patients with generalized anxiety disorder.

The tremor in patients with anxiety was supposed to be an enhanced physiological tremor. However no detailed clinical and electromyographic examination of tremor in patients with anxiety has been published. It has been also supposed that propranolol or benzodiazepines may influence the tremor The aim of this investigation was to establish the clinical and electromyographic pattern of tremor in patients with anxiety and to compare the effects of propranolol and alprazolam treatment. One hundred and twenty patients with tremor and generalized anxiety disorder were investigated. Tremor was scored clinically by the Webster Tremor Scale. Electromyographic examination of tremor activity from antagonistic hand muscles was performed. Sixty patients were treated with alprazolam and propranolol, in an open, crossover design, while the other sixty patients received no particular treatment for the tremor The patients were randomly assigned to treatment with propranolol, alprazolam, or received no tremor treatment. Our results revealed a postural and kinetic tremor with characteristics of an enhanced physiological tremor. Tremor involved only upper limbs and no other body parts. Treatment with propranolol or alprazolam had similar favorable effect on tremor. In conclusion tremor in generalized anxiety disorder is an enhanced physiological tremor The kinetic tremor disturbs patients and particular tremor treatment with propranolol or alprazolam should be applied.

The epidemiology of multiple sclerosis in Europe.

Multiple sclerosis (MS) is a chronic and potentially highly disabling disorder with considerable social impact and economic consequences. It is the major cause of non-traumatic disability in young adults. The social costs associated with MS are high because of its long duration, the early loss of productivity, the need for assistance in activities of daily living and the use of immunomodulatory treatments and multidisciplinary health care. Available MS epidemiological estimates are aimed at providing a measure of the disease burden in Europe. The total estimated prevalence rate of MS for the past three decades is 83 per 100,000 with higher rates in northern countries and a female:male ratio around 2.0. Prevalence rates are higher for women for all countries considered. The highest prevalence rates have been estimated for the age group 35-64 years for both sexes and for all countries. The estimated European mean annual MS incidence rate is 4.3 cases per 100,000. The mean distribution by disease course and by disability is also reported. Despite the wealth of epidemiological data on MS, comparing epidemiological indices among European countries is a hard task and often leads only to approximate estimates. This represents a major methodological concern when evaluating the MS burden in Europe and when implementing specific cost-of-illness studies.

Effects of sleep on spinal nociceptive reflexes in humans.

Controversy continues to surround the monosynaptic and polysynaptic spinal reflexes during the different stages of sleep. In animal studies both of these reflexes were found to be depressed during desynchronized sleep. In humans, the H reflex was unchanged whereas the second component of the nociceptive flexion reflex was increased. However, abolition of the H reflex and F waves during REM sleep has also been reported. The aim of this investigation was to examine the effects of different sleep stages on the polysynaptic nociceptive flexion reflex. Six healthy volunteers were studied. The RIII reflex was studied according to Willer's method (1977) during the different stages of NREM and REM sleep. The RIII reflex threshold was found to increase during stage 2 of NREM sleep. It remained higher during stages 3 and 4. During REM sleep a further increase in the reflex threshold was observed. The reflex latency was prolonged during stage 4 of NREM sleep. There was evidence of further latency prolongation during REM sleep. It was also during REM sleep that the maximum increase in the amplitude and duration of the reflex were recorded.

A comparative study of methods for estimation of presynaptic inhibition.

The influence of vibration on the H-reflex and on the tendon reflex amplitudes was compared and the efficacy of both methods for the assessment of the presynaptic inhibition was studied. One hundred and twenty patients with post-stroke spastic hemiparesis were investigated. Muscle tone, muscle force and tendon reflexes were assessed. The H-reflex and the Achilles tendon reflex (TA) were recorded under identical experimental conditions. Vibration at a frequency of 100 Hz and an amplitude of 2 mm was applied to the TA. Just after vibration the maximal amplitudes of both reflexes were measured. The ratios of reflex amplitudes after vibration to normal maximal reflex amplitudes (Hvibr/Hmax and TAvibr/TAmax) were evaluated. In all patients with hemiparesis the healthy side was used as a control. Our results revealed significantly increased amplitude ratios on the spastic side. Hence it is concluded that presynaptic inhibition is decreased in spasticity. The amplitude ratios on the healthy and the spastic side were consistent. There was good positive correlation between Hvibr/Hmax and TAvibr/TAmax ratios, suggesting that they provide similar and reliable estimates of presynaptic inhibition.

Electromyographic differentiation of tremors.

OBJECTIVE: The aim of this study was to explore the opportunity for differentiation of tremors on the basis of the electromyographic examination. METHODS: We report data from 525 patients with different types of tremor included in this study. Two hundred and twenty patients with essential tremor, 110 patients with Parkinson's disease, 24 patients with midbrain tremor, 22 patients with cerebellar tremor, 120 patients with enhanced physiological tremor due to anxiety, and 29 patients with psychogenic tremor participated in the study. A detailed neurological and electromyographic examination was performed. The tremor was studied in all limbs positions. Surface electromyographic recordings of the most involved body part were performed to all patients. RESULTS: According to the electromyographic examination all patients were divided to two groups: Patients with synchronous tremor and patients with alternating tremor in the antagonist muscles. The comparisons were performed only between the patients with tremor caused by different etiology within the group of synchronous or alternating tremor. The group of tremors with synchronous pattern included the patients with essential tremor type A (with synchronous activity), cerebellar, and enhanced physiological tremor. The group of tremors with alternating pattern included patients with Parkinsonian tremor, essential tremor type B (with alternating activity), rubral, and psychogenic tremors. Our results revealed that electromyographic examination of tremor pattern, frequency, amplitude, and burst duration is useful for tremor differentiation. CONCLUSION: The electromyographic examination may serve as a tool for differentiation of tremors.

Vestibular and auditory influences on segmental motoneuron excitability--a comparative study.

This study aimed to investigate the vestibular and acoustic influences on segmental motoneuron excitability in the same normals in order to compare the effects of both sensory systems. The results show that both systems exert similar influences on H-reflex in the same subjects. These indicate similarities in the function of motor control and suggest common final pathways of the vestibular and auditory activity to the segmental motoneuron. The alternative effect, facilitation or inhibition, of acoustic and vestibular stimuli on H-reflex is devoted to processing of the labyrinthine activation in the brainstem reticular formation, or to the state of the spinal interneurons.

Different effect of high doses of naloxone on spinal reflexes in normal subjects and chronic paraplegic patients.

There is still controversy over the effects of naloxone on spinal reflexes in view of the fact that both facilitatory and inhibitory activities have been observed. Dosage, supraspinal influences and interactions with different opiate receptors may account for the different findings. We investigated the effect of placebo (saline) and high doses of naloxone (1.66 mg/kg) on the monosynaptic (H reflex) and nociceptive polysynaptic reflex (RIII reflex) in five normal subjects and three chronic paraplegic subjects. Following the administration of naloxone, there were no changes in the RIII reflex threshold in either group. By contrast, there was a marked facilitation of the H reflex amplitude in the normal subjects, but not in the spinal cord-injured subjects after treatment with naloxone. Saline induced no changes in the RIII reflex threshold or the H reflex amplitude in either of the two groups. Our data suggest that under normal conditions the opiatergic modulation of the nociceptive reflex is not functionally active whereas the tonic inhibitory modulation of the monosynaptic reflex is mediated by descending pathways.

Effects of hypnosis on diffuse noxious inhibitory controls.

The neurophysiological mechanisms of hypnotic analgesia are still under debate. It is known that pain occurring in one part of the body (counterstimulation) decreases pain in the rest of the body by activating the diffuse noxious inhibitory controls (DNICs). The aim of this study was to explore the effects of hypnosis on both pain perception and heterotopic nociceptive stimulation. The A forms of both the Harward Group Scale of Hypnotic Susceptibility and the Stanford Hypnotic Susceptibility Scale were administered to 50 healthy students. Twenty subjects were selected and assigned to two groups: group A, consisting of 10 subjects with high hypnotic susceptibility; and group B, consisting of 10 subjects with low hypnotic susceptibility. The subjects were then randomly assigned first to either a control session or a session of hypnotic analgesia. The nociceptive flexion reflex (RIII) was recorded from the biceps femoris muscle in response to stimulation of the sural nerve. The subjective pain threshold, the RIII reflex threshold, and the mean area with suprathreshold stimulation were determined. Heterotopic nociceptive stimulation was investigated by the cold-pressor test (CPT). During and immediately after the CPT, the subjective pain threshold, pain tolerance, and mean RIII area were determined again. The same examinations were repeated during hypnosis. Hypnosis significantly reduced the subjective pain perception and the nociceptive flexion reflex. It also increased pain tolerance and reduced pain perception and the nociceptive reflex during the CPT. These effects were found only in highly susceptible subjects. However, the DNIC's activity was less evident during hypnosis than during the CPT effects without hypnosis. Both hypnosis and DNICs were able to modify the perception of pain. It seems likely that DNICs and hypnosis use the same descending inhibitory pathways for the control of pain. The susceptibility of the subject is a critical factor in hypnotically induced analgesia.

Clinical and electromyographic assessment of essential tremor treatment.

It is a matter of debate whether beta-blockers or primidone have a better influence on essential tremor. It is also controversial whether essential tremor with synchronous electromyographic activity is influenced by the administration beta-blockers, while tremor with alternating electromyographic activity is influenced by primidone. The aim of this study was to reevaluate the effects of beta-blockers and primidone on essential tremor. We also aimed to evaluate the differential effects of beta-blockers and primidone on tremor with synchronous or alternating electromyographic activity and on tremor in different limb positions. One hundred patients (57 female, 43 male) with essential tremor were included in this study. According to the electromyographic pattern recorded from the antagonistic muscles, the patients were subdivided into group with synchronous (SYN) and group with alternating (ALT) activity. After the initial clinical and electromyographic examinations the patients were randomly assigned first to treatment with propranolol (180 mg daily), or to treatment with primidone (500 mg daily) for 30 days. After a washout period of 20 days the patients were assigned to the other treatment. Our results revealed that the effects of propranolol and primidone on essential tremor and on SYN or ALT tremor were similar. Differences between both drugs existed as regards to better primidone influence on the kinetic and intention tremors and to tremor localized outside the limbs. In conclusion it is better to treat patients with kinetic and intention essential tremor and tremor localized outside the limbs with primidone.

A cross-over clinical and electromyographic assessment of treatment for parkinsonian tremor.

BACKGROUND: Whether dopaminergic and anticholinergic drugs exert influence on parkinsonian rest and postural tremor is a subject of debate. Different types of tremor may be influenced differently by the drugs. The aim of this study was to reevaluate the differential effects of levodopa and anticholinergic drugs on parkinsonian tremor in different limb positions and on different types of postural tremor. METHODS: Thirty-eight patients with parkinsonian resting tremor and postural tremor were included in this study. Patients were divided into two groups according to the electromyographic pattern of the postural tremor. We found fast synchronous postural tremor (>7 Hz) in 16 patients, and slow alternating postural tremor in 22 patients. The tremor was scored clinically in each limb position using the Webster Tremor Scale. Surface electromyographic recordings from the most involved limb in all positions were also performed. The patients were randomly assigned to levodopa (one 250/50-mg tablet), or to biperiden (one 3-mg tablet). Tremor was assessed by clinical and electromyographic examinations at base line 1h following ingestion of the drug. The subjective tremor improvement was also assessed. RESULTS: We found that levodopa had a good effect on the amplitude of the resting tremor, while the effect of biperiden was weaker. Both levodopa and biperiden has less effect on postural tremor. However, levodopa's effect was better than that of biperiden. Levodopa and biperiden had better effect on slow alternating postural tremor than on fast synchronous postural tremor. They had no effect on kinetic and intention tremors. CONCLUSIONS: Levodopa and anticholinergic drugs have differing effects on both resting and postural tremor Also, the different categories of postural tremor respond differently to treatment. The mechanisms underlying resting parkinsonian tremor may be different from those underlying postural, kinetic and intention tremor. Moreover, the mechanisms underlying different types of postural tremor may be different.

Contralateral hemi-parkinsonism and atypical tremor after stroke (a case report).

Extrapyraramidal parkinsonian-like syndromes have been observed as manifestations of different central nervous system lesions. A 45-year-old-man gradually developed tremor and slowing of movements 2 years after a stroke. Neurological examination revealed slight hemiparesis, muscle rigidity and bradykinesia in the left limbs. A tendency for hypomimia and impaired postural reflexes were found. There were resting, more pronounced postural and kinetic and most pronounced intentional tremors involving the left limbs. On electromyographic examination the static tremor was of 4Hz frequency, while the postural, kinetic and intention tremors were of 7Hz frequency. All tremors were with alternating activity in antagonist muscles. In conclusion our findings confirm the common anatomical basis of parkinsonism and essential like tremor. Both can appear after disruption of the pathways within and adjacent to the basal ganglia.

Clinical and electromyographic examinations of patients with essential tremor.

BACKGROUND: It is believed that no clinical differences exist among essential, familial and senile tremor, or between the tremor with synchronous or alternating electromyographic activity. The aim of this study was to evaluate the clinical and electromyographic findings in a large group of patients with different types of essential tremor. METHODS: Two hundred and twenty patients with sporadic, familial or senile variants of essential tremor were examined. According to the electromyographic activity recorded from the antagonistic muscles, the patients were subdivided into a group with synchronous (SYN) and a group with alternating (ALT) activity. The historical aspects of the disease were noted, and a detailed neurological examination was performed. RESULTS: A widespread tremor involving upper and lower limbs and 3-4 different anatomical regions was typical for familial tremor. It also had higher amplitude than the sporadic and senile tremor. ALT tremor had a higher amplitude and longer burst duration than SYN and more often involved lower limbs. Rest tremor was common in the ALT group. Overall, ALT tremor was more common than previously supposed. CONCLUSION: The familial and ALT tremors are more disabling than other types of essential tremor. Since electromyographic ALT activity is common in essential tremor, its presence does not reliably distinguish essential and Parkinsonian tremor.

Writer's cramp and tremor due to brain tumor.

Writer's cramp is frequently associated with hand tremor and with other focal dystonias. Sometimes brain infarction, haemorrhage, arterio-venous malformations or tumors are found to cause symptomatic focal dystonias. A 44-year-old man came to our attention due to writer's cramp of his right hand and postural 6-8 Hz tremor. About three months after the onset of the disease the tremor and dystonia disappeared, while a right hemiparesis gradually appeared. Cranial computed tomography showed a hypodense nonhomogeneous lesion in the left cerebral hemisphere parietally, that was assumed to represent a cystic tumor. The patient underwent craniotomy and the histological diagnosis revealed an astrocytoma (Grade II-III). In conclusion our findings confirm the common anatomical basis of symptomatic focal dystonia and tremor. Both can appear after disruption of the pathways within and adjacent to the basal ganglia.

A comparison of methods to assess the excitability of lower motoneurones.

The purpose of this investigation was to compare three methods of assessing the excitability of lower motoneurones--TA-reflex, H-reflex and F-wave--in 120 patients with spastic hemiparesis following a stroke. The H-reflex was recorded from the soleus muscle after submaximal electrostimulation of the tibial nerve. The T-Achilles (TA) reflex was recorded from the soleus muscle after percussion of the Achilles tendon. The F-wave was recorded in the distal limb muscles after supramaximal electrostimulation of the median, ulnar, fibular and tibial nerves. The patient's healthy side was used as a control. The TA-reflex, H-reflex and F-wave showed increased amplitudes on the spastic side. All amplitude ratios: TA/M, H/M, Fmax/M and Fmean/M were increased. The H-reflex thresholds were decreased. The F-wave duration, persistence and number of phases were also increased on the spastic side. Despite clinically decreased muscle tone, there were no changes in TA or H-reflex parameters after treatment. On the other hand, F-wave parameters tended to normalize after treatment in all groups. In conclusion, the F-wave is a more sensitive method than the TA and H-reflexes in assessing the excitability of the lower motoneurone.