Polysomnographic characteristics of severe obstructive sleep apnea vary significantly between hypertensive and normotensive patients of both genders.

PURPOSE: Hypertension is a common finding in patients with obstructive sleep apnea (OSA), but it has remained unclear whether or not the amount of disturbed breathing and characteristics of individual respiratory events differ between hypertensive and normotensive patients with severe OSA. METHODS: Full polysomnographic recordings of 323 men and 89 women with severe OSA were analyzed. Differences in the duration of individual respiratory events, total apnea and hypopnea times, and the percentage of disturbed breathing from total sleep time (AHT%) were compared between normotensive and hypertensive patients separately by genders. Furthermore, differences in the respiratory event characteristics were assessed between three AHT% groups (AHT% ≤ 30%, 30% < AHT% ≤ 45%, and AHT% > 45%). RESULTS: Hypertensive women had lower percentage apnea time (15.2% vs. 18.2%, p = 0.003) and AHT% (33.5% vs. 36.5%, p = 0.021) when compared with normotensive women. However, these differences were not observed between hypertensive and normotensive men. Percentage hypopnea time was higher in hypertensive men (13.5% vs. 11.2%, p = 0.043) but not in women (15.2% vs. 12.2%, p = 0.130) compared with their normotensive counterparts. The variation in AHI explained 60.5% (ρ = 0.778) and 65.0% (ρ = 0.806) of the variation in AHT% in normotensive and hypertensive patients, respectively. However, when AHT% increased, the capability of AHI to explain the variation in AHT% declined. CONCLUSIONS: There is a major inter- and intra-gender variation in percentage apnea and hypopnea times between hypertensive and normotensive patients with severe OSA. OSA is an important risk factor for hypertension and thus, early detection and phenotyping of OSA would allow timely treatment of patients with the highest risk of hypertension.

Long-term glutamate supplementation failed to protect against peripheral neurotoxicity of paclitaxel.

Toxic peripheral neuropathy is still a significant limiting factor for chemotherapy with paclitaxel (PAC), although glutamate and its closely related amino acid glutamine were claimed to ameliorate PAC neurotoxicity. This pilot trial aimed to evaluate the role of glutamate supplementation for preventing PAC-induced peripheral neuropathy in a randomized, placebo-controlled, double-blinded clinical and electro-diagnostic study. Forty-three ovarian cancer patients were available for analysis following six cycles of the same PAC-containing regimen: 23 had been supplemented by glutamate all along the treatment period, at a daily dose of three times 500 mg (group G), and 20 had received a placebo (group P). Patients were evaluated by neurological examinations, questionnaires and sensory-motor nerve conduction studies. There was no significant difference in the frequency of signs or symptoms between the two groups although neurotoxicity symptoms presented mostly with lower scores of severity in group G. However, this difference reached statistical significance only with regard to reported pain sensation (P = 0.011). Also the frequency of abnormal electro-diagnostic findings showed similarity between the two groups (G: 7/23 = 30.4%; P: 6/20 = 30%). This pilot study leads to the conclusion that glutamate supplementation at the chosen regimen fails to protect against peripheral neurotoxicity of PAC.

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.

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).

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.

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.

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.

Can salivary composition and high flow rate explain the low caries rate in children with familial dysautonomia?

PURPOSE: Extremely low caries rate and increased major salivary gland flow rare have been previously reported in children with familial dysautonomia (FD). The purpose of this study was to explore the possibility that, in addition to increased salivary flow, children with FD have alterations in their salivary components, which may suggest an explanauon to their low caries rate. METHODS: Whole unstimulated and stimulated saliva samples were collected from 13 children with FD who were found to be caries free, and from 28 age- and ethnic-matched healthy children, 15 caries-free children and 13 caries-affected children. The electrolyte and protein content of the unstimulated saliva and the microbial count and buffering capacity of the stimulated saliva were determined. RESULTS: Children with FD had the highest salivary flow rate and the lowest levels of mutans streptococci and lactobacilli, as well as the lowest concentration of chloride, magnesium, total protein and IgA. Healthy caries-affected children displayed the highest mutans streptococci and lactobacilli levels and lysozyme concentration, concomitantly with the lowest potassium and calcium concentrations. CONCLUSIONS: The results of this investigation suggest that the caries-free state in FD may be associated with high salivary flow rate, while in healthy children, low caries rate may be associated with high salivary calcium concentration.

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.

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.

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.

[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.

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.

Paraneoplastic cerebellar degeneration heralding fallopian tube adenocarcinoma.

The objective of this paper is to describe an 81-year-old woman with subacute cerebellar degeneration due to fallopian tube adenocarcinoma. Serum anti-Yo antibodies were used to screen for pelvic malignancy. Their presence led to a meticulous search, which included bilateral salpingoophorectomy. Subsequently an occult fallopian tube adenocarcinoma was discovered. This case report highlights the diagnostic value of antineuroneal antibodies in females with subacute neurologic impairment in the form of paraneoplastic syndrome.

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.

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.

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.