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Biomedical subjects

N Gadoth

Publications and source records attributed to N Gadoth.

At least 19 recordsLinked to original sources

Electrogastrography in patients with Parkinson's disease.

BACKGROUND: Impaired gastrointestinal motility in Parkinson's disease may affect absorption of levodopa and contribute to the disabling response fluctuations (RF). In this study gastric myoelectric activity was recorded with electrogastrography in patients with PD and correlated with the duration, severity and the presence of RF. METHOD: Electrogastrography (EGG) was performed in 36 patients with PD of which 22 were men. The mean age was 67 years (48-81), mean duration of disease was 7.07 years (1-20), and mean duration of treatment with levodopa was 5.07 years (1-20). Gastric dysrhythmia was diagnosed when either preprandial or postprandial dysrhythmia for more than 30% of the recording period was detected. RESULTS: The EGG was abnormal in 24 of 36 patients. Significant association was found between preprandial dysrhythmia and duration of disease (P=0.002); duration of levodopa treatment (P=0.003), severity of 86RF (P=0.001), but not with age (P=0.076). Out of 18 patients with RF, 17 had at least one pattern of dysrhythmia. In 11 out of the 18 patients without RF, the EGG was normal while the remaining seven had at least one pattern of dysrhythmia. CONCLUSION: Abnormal EGG was quite common in this group of patients with PD, particularly in those with RF. The most common pattern of abnormality was preprandial dysrhythmia, which was positively associated with disease duration and length of levodopa treatment. Although frequently asymptomatic, preprandial dysrhythmia leading to impaired gastric emptying may contribute to irregular absorption of levodopa from the small intestine and contribute to disabling response fluctuations.

Aged↗

Case report: multiple endocrine neoplasia type 2B misdiagnosed as familial dysautonomia.

BACKGROUND: Familial dysautonomia (FD) is a rare autosomal recessive disorder of the peripheral nervous system, affecting exclusively Jewish children of Ashkenazi extraction. The typical clinical features consist of somatic abnormalities: failure to thrive, characteristic facies, excessive sweating, labile blood pressure, recurrent aspiration pneumonias, lack of tears, and diminished and later absent deep tendon reflexes with generalized reduction of pain sensation. Oro-dental features include a lack of tongue fungiform papillae, impairment of taste, oro-dental self-mutilation, dental crowding, excessive plaque and calculus accumulation, salivary over production and low caries experience. CASE REPORT: A child with multiple endocrine neoplasia type 2B (MEN 2B) received, at the age of 11 months, an incorrect diagnosis of familial dysautonomia (FD). At the age of 6 years, a paediatric dentist experienced with FD noticed a normal number and shape of tongue fungiform papillae, while expecting to find a smooth tongue lacking those structures. The presence of numerous submucosal neuromata initiated a meticulous neurological and endocrine work-up, which established the diagnosis of MEN 2B. This led to an early detection and appropriate treatment of asymptomatic medullary thyroid carcinoma (MTC).

Adolescent↗

Repeated vs single physical maneuver in benign paroxysmal positional vertigo.

OBJECTIVES: To evaluate the effectiveness and possible side effects of a single session of repeated particle repositioning maneuver (PRM) to treat posterior canal benign paroxysmal positional vertigo (BPPV) and the usefulness of post-treatment restrictions. MATERIALS AND METHODS: A total of 125 consecutive patients with idiopathic BPPV participated in the study. Fifty patients received a single session of repeated PRM only (group I). Results were compared with those of 50 patients with BPPV who received a single PRM (group IIb), and 25 patients who received a single PRM followed by the use of a neck collar and keeping the head upright for 48 h (group IIa). RESULTS: Forty-six patients (92%) of group I, 40 patients (80%) of group IIb, and 21 patients (84%) of group IIa were completely free of signs and symptoms when re-examined 1 week after treatment. Transient nausea and disequilibrium following treatment were reported equally in all subgroups and well tolerated. Nearly all patients of group IIa considered the post-treatment restrictions very inconvenient. CONCLUSIONS: A single session of repeated physical procedure seems to be clinically superior to one single maneuver and well tolerated. Additional post-treatment measurements are inconvenient and should be abandoned.

Adult↗

Caffeine-induced headache in children and adolescents.

Caffeine is the most widely used behaviourally active substance. Excessive caffeine consumption, mostly in the form of coffee and tea, is a well-recognized cause of headache or migraine, and withdrawal can cause headache. Nevertheless, caffeine abuse headache is not listed as a separate category in the International Headache Society classification, 1988. We report our experience with children and adolescents with daily or near-daily headache and excessive consumption of caffeine in the form of cola drinks. Over a period of 5 years we have encountered, in a tertiary headache clinic in a general hospital, 36 children and adolescents (17 girls and 19 boys) with daily or near-daily headache related to excessive caffeine intake in the form of cola drinks. The mean age of the subjects was 9.2 years (range 6-18) and mean headache duration was 1.8 years (range 0.6-5). All were heavy cola drinks consumers; at least 1.5 L of cola drinks per day (192.88 mg of caffeine daily), and an average of 11 (range 10.5-21) L of cola drinks a week, which amounts to 1414.5 mg of caffeine (range 1350.1-2700.3). Patients were encouraged to achieve gradual withdrawal from cola drinks, which led to complete cessation of all headaches in 33 subjects, whereas one boy and two adolescent girls continued to suffer from migraine without aura not frequent enough to justify prophylactic medication. Children and adolescents with high daily caffeine consumption in the form of cola drinks may suffer from caffeine-induced daily headache. Gradual withdrawal can be achieved without withdrawal headache and with complete disappearance of the induced chronic daily headache.

Adolescent↗

Vestibulo-ocular arreflexia in families with spinocerebellar ataxia type 3 (Machado-Joseph disease).

OBJECTIVE: To identify the presence of vestibulo-ocular arreflexia in patients with Machado-Joseph disease (MJD), which can easily be diagnosed at the bedside. METHODS: Seven patients with MJD from five unrelated families and 11 patients with sporadic or hereditary cerebellar ataxia other than MJD underwent a detailed neuro-otological and oculomotor examination. Six MJD and five non-MJD patients also underwent electro-oculographic recordings and caloric tests. RESULTS: Gaze evoked nystagmus, smooth pursuit, and saccade abnormalities were found in both MJD and non-MJD patients. However, in all seven MJD patients but in none of the non-MJD patients, sudden passively induced head thrust to both sides elicited pathological corrective catch-up saccades, indicating bilateral loss of the horizontal vestibulo-ocular reflex. This was further confirmed in six MJD patients who had absent vestibular response to both a standard caloric test and ice water ear irrigation. Nystagmus was induced by standard caloric irrigation in all non-MJD patients examined. There was no correlation between the loss of vestibular function and the severity of cerebellar impairment. CONCLUSIONS: The presence of vestibulo-ocular arreflexia, as measured by the head thrust test in a patient with dominant cerebellar ataxia, strongly suggests the diagnosis of MJD.

Adult↗

The use of baclofen in cluster headache.

Cluster headache is a rare, clinically well-characterized disabling disorder that occurs in both episodic and chronic forms. The very painful short-lived unilateral headache attacks are associated with autonomic dysfunction. A large number of drugs such as ergotamines, steroids, methysergide, lithium carbonate, verapamil, valproate, capsaicin, leuprolide, clonidine, methylergovine maleate, methylphenidate, and melatonin are considered beneficial for prophylaxis. Nevertheless, this extremely painful condition is occasionally refractory to conventional treatment. The antispastic agent baclofen has been shown to possess an antinociceptive activity. Its efficacy in neuralgias, central pain following spinal lesions, painful strokes, migraine, and medication misuse chronic daily headache suggests that it may be useful for prevention of cluster headache attacks. Therefore, we treated 16 symptomatic patients with cluster headache with daily baclofen, 15 to 30 mg, in three divided doses for the cluster period and 2 weeks after. Within a week, 12 patients reported the cessation of attacks. One was substantially better and became attack free by the end of the following week. In the remaining three patients, the attacks worsened and corticosteroids were prescribed. One of these was also given verapamil. Three of the 16 patients had an additional cluster period, which cleared with a second course of baclofen. In this pilot study, baclofen seemed to be effective, safe, and well tolerated for cluster headache, and seemed to retain its efficacy on repeated clusters.

Adrenal Cortex Hormones↗

Seizure frequency and characteristics in children with Down syndrome.

Seizures have not historically been considered a major component of Down syndrome. We examined the prevalence of epileptic seizures in 350 children and adolescents with Down syndrome evaluated at a regional center between 1985 and 1997. Results showed that 28 patients (8%) had epileptic seizures: 13 (47%) partial seizures; 9 (32%) infantile spasms, and 6 (21%) generalized tonic-clonic seizures. In the infantile spasm group, there was no relationship between the initial electroencephalogram (EEG) pattern and response to treatment or long-term seizure control, or between type of pharmacologic treatment (valproic acid, adrenocorticotropic hormone or both) and clinical remission, EEG normalization or long-term seizure control. Neurodevelopmental outcome was poor despite good seizure control in the infantile spasm group. This regional study reinforces the relative association of seizures and Down syndrome. A prospective study including a national/international registry with emphasis on developmental assessment and long-term follow up is warranted.

Adolescent↗

Abnormal skin wrinkling in the less affected side in hemiparkinsonism-a possible test for sympathetic dysfunction in Parkinson's disease.

Some patients with Parkinson's disease (PD) suffer from autonomic dysfunction, even in the early stage of the disease. We examined the skin wrinkling response following immersion of the hands in warm water in 18 patients with hemiparkinsonism. This test evaluates the function of the sympathetic autonomic system. Mean age of the patients was 61 +/- 10 and mean disease duration 5.5 +/- 3.5 years. Both hands of each patient were immersed in warm water for 30 minutes and the number of skin ridges of the fingertip of each finger was counted. The results of each hand were compared to those of nine healthy controls. The mean number of the ridges of the less affected hand was significantly decreased as compared to the affected hand and controls (6.1 +/- 6.8 vs 13.1 +/- 6.8 and 15.3 +/- 8.5, respectively; P < 0.01). These results suggest that autonomic dysfunction is prevalent in the less affected side of patients with PD and can be simply tested by the skin response test.

Adult↗

Clinical and polysomnographic characteristics of 34 patients with Kleine-Levin syndrome.

There is only scant information on sleep characteristics and long-term follow-up in patients with Kleine-Levin syndrome (KLS). This study describes the clinical course, results of polysomnography and long-term follow-up in a relatively large group of patients with KLS. During the years 1982-97, we encountered 34 patients (26 males and eight females) with KLS. We were able to obtain the original polysomnographs from 28 males and four females. In 25 patients, data regarding their present state of health were obtained. Fourteen agreed to be present at a detailed interview and examination while 11 gave the information by phone. The mean age at onset was 15.8 +/- 2.8 years and the mean diagnostic delay, 3.8 +/- 4.2 years. The mean duration of a single hypersomnolent attack was 11.5 +/- 6.6 days. The main abnormal findings extracted out of 35 polysomnographs obtained from 32 patients during and/or in-between attacks included: decreased sleep efficiency, and frequent awakenings from sleep stage 2. All 25 patients reported present perfect health, with no evidence of behavioral or endocrine dysfunction. In adolescents with periodic hypersomnia, the diagnosis of KLS should be explored. Sleep recordings during a hypersomnolent period will often show frequent awakenings from sleep stage 2. The long-term prognosis is excellent.

Adult↗

Is blood homocysteine elevated in migraine?

OBJECTIVE: To determine total serum homocysteine levels in a large group of patients with migraine with and without aura. BACKGROUND: Hypercoagulable state is a known risk factor for stroke in the young. The existence of a hypercoagulable state has been postulated in migraine and homocysteinemia with young-onset stroke. To the best of our knowledge, blood homocysteine has not been studied in a significant number of patients with various forms of migraine. METHODS: Total serum homocysteine was measured with high-performance liquid chromatography in 78 patients with migraine and in 126 age- and sex-matched healthy volunteers. RESULTS: Seventy-eight patients aged 18 to 65 years were studied: 22 with migraine with aura and 56 with migraine without aura. Only 1 man had significantly elevated blood homocysteine (38.6 micromol/L), while another had a borderline elevation (15.8 micromol/L) (reference value for both sexes in our laboratory is 4 to 14 micromol/L). Both patients suffered from migraine without aura. CONCLUSIONS: Blood homocysteine is not elevated in migraine.

Adult↗

Epidemiology of idiopathic intracranial hypertension in Israel.

OBJECTIVES: To determine the incidence, demographic, and clinical features of Pseudo Tumor Cerebri (PTC)/Idiopathic Intracranial Hypertension (IIH) in Israel. MATERIALS AND METHODS: The chairpersons of all neurology and ophthalmology departments in Israel were asked to complete questionnaires regarding patients diagnosed with PTC/IIH from 1998 through 1999. Each questionnaire contained details regarding patient's age, sex, country of birth, age at diagnosis, weight, height, presence of obesity, and the results of lumbar puncture, brain computed tomography, magnetic resonance imaging, and/or magnetic resonance venography. RESULTS: Ninety-one patients with PTC/IIH were diagnosed during the years 1998 to 1999. Eighty-five (93.4%) patients were females and six (6.6%) patients were males. The calculated incidence of PTC/IIH in the Israeli general population was 0.57 to 0.94 per 100,000 persons, with incidences of 1.82 per 100,000 for women and 0.034 per 100,000 for men. The incidence for women during the childbirth years was 4.02 per 100,000. The female to male ratio was higher than previously reported for Western countries. CONCLUSIONS: Although the population of Israel is a mixture of people originating from Eastern and Western countries, the incidence of PTC/IIH was found to be similar to that of Western countries. This finding is an additional support to the notion that PTC/IIH is more common in obese populations.

Adult↗

Do men with pseudomotor cerebri share the same characteristics as women? A retrospective review of 141 cases.

OBJECTIVE: To determine whether males with pseudotumor cerebri (PTC) differ from females by clinical presentation, risk factors, and outcome. METHODS: The medical records of patients diagnosed with PTC or idiopathic intracranial hypertension (IIH) in two major university hospitals were obtained. Diagnostic criteria, clinical features, presence of obesity, mode of treatment, and outcome were tabulated. RESULTS: A total of 134 patients (18 males and 116 females) fulfilled the Dandy diagnostic criteria for PTC. Females and males shared similar clinical features and outcome. There was a substantial difference between the groups regarding body weight. The majority of females (77.8%) were considered significantly overweight, compared to 25% of the males. CONCLUSION: Pseudotumor cerebri in males is relatively rare. The clinical features are identical to those found in females. The fact that the majority of the male patients had a normal body weight may indicate that increased body weight does not play a major role in causing PTC in men, whereas it is an established major risk factor in women.

Adolescent↗

Vestibular findings in professional divers.

The purpose of the present study was to investigate possible inner ear changes related to professional diving, by the documentation of auditory and vestibular function in 13 asymptomatic professional divers and 12 nondiver controls. A higher average pure tone hearing threshold, although of no clinical significance, was found in the study group (8.53 +/- 4.85 versus 6.67 +/- 3.54 dB hearing level, p = .04). In the vestibular evaluation, the smooth harmonic acceleration test phase leads for 0.01, 0.02, and 0.04 Hz were significantly lower in the divers (0.01 Hz, 38.46 degrees +/- 7.15 degrees versus 45.83 degrees +/- 9.02 degrees, p = .02; 0.02 Hz, 21.08 degrees +/- 5.19 +/- versus 25.17 degrees +/- 5.78 degrees, p = .05: 0.04 Hz, 12.38 degrees +/- 3.69 degrees versus 14.25 degrees +/- 3.14 degrees, p = .05). We suggest that the lower smooth harmonic acceleration phase values found in the professional divers, reflecting longer vestibulo-ocular reflex primary time constants and enhancement of the velocity storage mechanism, are the result of a habituation process that augments the low-frequency response of the canal-ocular system.

Adult↗

Successful withdrawal from analgesic abuse in a group of youngsters with chronic daily headache.

Abuse of ergotamine and analgesics is common in adults. It coexists with headache and can also induce headaches. Ten to 15% of patients attending headache clinics and 1% of the general population suffer from chronic daily headache due to medication misuse. Indeed, this phenomenon was recently regarded as an epidemic. Nonetheless, analgesic-induced headache in children and adolescents was first reported in 1998. We report on our experience with children and adolescents with daily or almost-daily headache concomitant with daily or almost-daily analgesic intake. Over a period of 3 years, we evaluated 26 children (19 girls and 7 boys) with chronic daily or near-daily headache related to daily analgesic intake. The mean age of the group was 14.2 years (range, 12-18), and the mean headache history duration was 1.6 years (range, 3 months to 4 1/2 years). The mean number of headache days per month was 28.1 (range, 19-31). All patients had no history of migraine prior to the chronic headache phase according to the International Headache Society criteria. They were using at least one dose of analgesic drug for each headache, whereas 16 were using analgesic drugs daily. The weekly analgesic intake averaged 28.1 tablets (range, 19-41). The majority abused simple analgesics. Twenty-one took acetaminophen alone. Five took a combination; four took a compound containing acetaminophen, caffeine, and codeine; and the fifth patient took a compound containing aspirin, caffeine, and codeine. All patients were informed about the phenomenon of medication-induced headache and were encouraged to achieve drug withdrawal. Withdrawal led to complete cessation of all headaches in 20 patients. In 5 patients, the daily headache resolved; however, they suffered from intermittent episodic migraine attacks, which were frequent enough in 3 to initiate prophylactic medication. One adolescent continued to have daily headache. Analgesic-induced headache does occur in adolescents. Successful withdrawal from the offending analgesics was achieved without hospitalization or significant interference with daily life and with complete disappearance of the induced chronic daily headache in 25 of 26 patients.

Adolescent↗

[The many faces of syringomyelia].

During July-August 1998, we encountered 4 patients who suffered from syringomyelia. The diagnosis in each case could not be reached on clinical grounds only, since the characteristic thermoanalgesia dissociation was absent in all patients. In each case the spinal MRI showed the typical intraspinal cystic lesion, which finally established the diagnosis and led to appropriate treatment.

Aged↗

Kleine Levin syndrome (KLS) in young females.

During the years 1982-1998, we encountered 7 adolescents and one young woman suffering from KLS. In 4 patients, hypersomnolence was accompanied by hyperphagia and hypersexuality, while in the remaining 4, recurrent hypersomnia was the only symptom. Mean age at onset of hypersomnolent attacks was 15.1+/-3.5 yrs. The mean duration of a hypersomnolent attack was 9.9+/-5.4 days, and the number of attacks per patient was 6.2+/-3.4. Polysomnographic recordings from 3 patients inbetween attacks, and from one patient during an attack, showed relatively normal sleep structure with decreased sleep efficiency due to numerous awakenings from sleep stage 2. Besides the recurrent hypersomnia, all patients enjoyed good health, with no evidence of behavioral or endocrine dysfunction. Similarly aged males with KLS from our clinic and previously reported females, had similar clinical features.

Adolescent↗

[Pitfalls in the diagnosis of benign paroxysmal positional vertigo].

Benign paroxysmal positional vertigo (BPPV) is a common but often unrecognized cause of treatable vertigo. Possible causes of misdiagnosis of BPPV were studied by review of the records of 191 patients referred to our neurology clinic; 36 were identified as having BPPV not previously diagnosed. On referral the patients carried the following diagnoses: unspecified dizziness/vertigo (33%), transient ischemic attacks (28%), cervicogenic vertigo (19%), psychogenic dizziness/vertigo (11%), and others (8%). The paroxysmal nature of the vertigo and position-precipitating factors were not spontaneously reported by 31%. Atypical, even bizarre, symptoms including dizzy sensations were reported by 19%, and neck pain and headache were cardinal symptoms that accompanied vertigo. The Dix-Hallpike maneuver, which is essential for the diagnosis of BPPV, was not performed in any of the patients prior to referral. 30 (83%) experienced complete resolution of signs and symptoms after the first physical treatment session. We conclude that non-paroxysmal, non-positional vertigo does not rule out BPPV. Atypical and even bizarre complaints of dizziness, as well as neck pain and headache could be cardinal symptoms of BPPV. The Dix-Hallpike maneuver is mandatory in those complaining of dizziness and vertigo.

Adult↗

Bipolar affective disorder heralding cerebral demyelination in adreno-myelo-leukodystrophy.

A young male with adrenomyeloleukodystrophy (AMLD), diagnosed at the age of 25 years, presented with signs of bipolar affective disorder (BPD) concomitant with radiological findings of central demyelination. There was a marked deterioration of the earlier relatively benign neurological dysfunction, leading to fatal bulbar syndrome. The association of BPD with central demyelination in AMLD is in agreement with previously reported cases of this and other types of multifocal central demyelination diseases.

Adrenoleukodystrophy↗