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PubMed1990

A longitudinal study of somatosensory, brainstem auditory and peripheral sensory-motor conduction during vitamin E deficiency in the rat.

A severe deficiency of vitamin E causes a characteristic neurological syndrome in man and experimental animals. In this study a number of electrophysiological modalities in vitamin E deficient and control rats have been investigated over a period of one year to define the time of onset and severity of the abnormalities associated with vitamin E deficiency in the rat. The mean velocities (n = 10) of the sensory evoked potentials were slower at all time points in the vitamin E deficient rats, with the central conduction velocities being more severely affected than the peripheral. Central conduction velocities, following both tibial and median nerve stimulation, were significantly delayed (P less than 0.005) after 8 months of deficiency. Differences in peripheral conduction following tibial stimulation became significantly delayed (P less than 0.005) after 11 months of deficiency. There were no significant differences in the brainstem auditory evoked potentials or peripheral sensory motor responses between the vitamin E deficient and control rats over the 1 year period. These results in the rat are essentially similar to those previously reported in vitamin E deficient man.

Animals↗
PubMed1985

Loss of auditory function in microvascular decompression for hemifacial spasm. Results in 143 consecutive cases.

Auditory function was studied before and after surgery in 143 consecutive patients who were operated on for hemifacial spasm by microvascular decompression of the intracranial portion of the facial nerve. The acoustic middle ear reflex was abnormal preoperatively in 41% of the patients, indicating that the vascular abnormalities that caused the hemifacial spasm also affected the auditory nerve. Three patients suffered a profound hearing loss in the ear on the operated side, and one lost hearing function totally. In addition, 24 patients had a moderate elevation in the pure-tone threshold at one or more octave frequencies. Of these, 16 patients experienced a hearing loss at only one frequency (8000 Hz), while eight had a threshold evaluation of no more than 20 dB in the speech frequency range (500, 1000, and 2000 Hz). Two patients were deaf on the side of the spasm before the operation. Three patients were not tested postoperatively, and one patient was tested only after surgery. Thus, in this series of 143 patients, only 2.8% suffered a significant hearing loss as a complication of facial nerve decompression to relieve hemifacial spasm.

Adult↗
PubMed1999

Spinal angiolipoma: case report and review of literature.

Spinal extradural angiolipomas are distinct, benign, and rare lesions composed of mature lipocytes admixed with abnormal blood vessels. They account for 0.14% of all spinal axis tumors. The case described here was a 72-year-old patient presenting with a history of paraparesis, hypoesthesia under the T2 level, hyperreflexia, and urinary overflow incontinence that appeared within 7 days after the administration of a coronary vasodilator drug regimen. The spinal magnetic resonance scan showed a lipomatous mass with signal void lesions, suggesting a vascular component of the tumor. The patient improved rapidly after surgical resection of the epidural tumor and decompression of the cord. According to the present literature, the duration of neurological symptoms ranges from 1 to 180 months (mean 28 months). But this patient's neurological deterioration took place 4 days before hospitalization. We believe that this can be explained by the increased tumor blood volume caused by vasodilator drugs, which in turn exerted a pulsatile compressive effect on the cord.

Aged↗
PubMed2005

Distal symmetric polyneuropathy: a definition for clinical research: report of the American Academy of Neurology, the American Association of Electrodiagnostic Medicine, and the American Academy of Physical Medicine and Rehabilitation.

The objective of this report was to develop a case definition of distal symmetric polyneuropathy to standardize and facilitate clinical research and epidemiologic studies. A formalized consensus process was employed to reach agreement after a systematic review and classification of evidence from the literature. The literature indicates that symptoms alone have relatively poor diagnostic accuracy in predicting the presence of polyneuropathy; signs are better predictors of polyneuropathy than symptoms; and single abnormalities on examination are less sensitive than multiple abnormalities in predicting the presence of polyneuropathy. The combination of neuropathic symptoms, signs, and electrodiagnostic findings provides the most accurate diagnosis of distal symmetric polyneuropathy. A set of case definitions was rank ordered by likelihood of disease. The highest likelihood of polyneuropathy (useful for clinical trials) occurs with a combination of multiple symptoms, multiple signs, and abnormal electrodiagnostic studies. A modest likelihood of polyneuropathy (useful for field or epidemiologic studies) occurs with a combination of multiple symptoms and multiple signs when the results of electrodiagnostic studies are not available. A lower likelihood of polyneuropathy occurs when electrodiagnostic studies and signs are discordant. For research purposes, the best approach to defining distal symmetric polyneuropathy is a set of case definitions rank ordered by estimated likelihood of disease. The inclusion of this formalized case definition in clinical and epidemiologic research studies will ensure greater consistency of case selection.

Clinical Protocols↗
PubMed1981

Abnormalities in baroreflex control of heart rate in canine heart failure.

The mechanism of the attenuated arterial baroreceptor control of heart rate in heart failure was studied in 18 unanesthetized dogs after the development of chronic right-heart failure and compared to 12 control animals. The change in heart rate and arterial pressure in response to increases in right carotid sinus pressure during isolated carotid sinus perfusion was markedly reduced in heart failure (P less than 0.05). After vagotomy the difference in heart rate responses persisted, but was less pronounced (P = 0.065). The chronotropic response to perfusion of the sinus node artery with acetylcholine in heart-failure dogs showed a selectively depressed response, when compared to norepinephrine and hypertonic saline. The ratio of the change in heart rate in heart-failure vs. control dogs was 57% for acetylcholine but was not diminished for hypertonic saline (114%). The effect of direct vagal nerve stimulation on changes in heart rate was also markedly reduced in heart-failure dogs. These studies demonstrate that the alterations in baroreceptor control of heart rate in heart failure involve both the parasympathetic and sympathetic baroreflex efferent limbs. There is, in addition, a depressed responsiveness of the sinus node to cholinergic stimuli.

Animals↗
PubMed1998

[Evacuation defecography and defecoflometry in diagnosis of chronic constipation. A prospective comparative study of 49 patients].

Chronic constipation can be divided in two large groups: slow-transit constipation, caused by pathological intestinal transit, and obstructive defecation disorder, caused by pelvic and rectal wall abnormalities. Videodefecography and defecoflowmetry are methods used to study dynamic evacuation of the rectum. Videodefecography also enables visual estimation of rectal evacuation. Defecoflowmetry allows analysis of anal and rectal pressures. Within this prospective study we utilized and compared these methods in 19 patients with slow transit and 30 patients with obstructive disease. In patients with slow transit, both investigations demonstrated a significantly higher defecation rate than in obstructive defecation disorder (slow transit: defecoflowmetry 65%, videodefecography 80%; obstructive defecation disorder: 50% and 58%). The evacuation time was pathologically prolonged in both types of constipation, with a range of 43-55 s. Rectoceles are demonstrated in 94% of cases with slow transit and in 72% with obstructive defecation disorder. Also, we often found obstructive components in slow-transit constipation patients. Normal defecography or defecoflowmetry can rule out obstructive defecation disorder. We conclude that videodefecography and defecoflowmetry are important items in the complex diagnostic regimen needed in evaluation of chronic constipation.

Adult↗
PubMed1987

Detrusor hyperactivity with impaired contractile function. An unrecognized but common cause of incontinence in elderly patients.

Little is known about the causes of urinary incontinence in institutionalized elderly people, despite the fact that $8 billion is annually devoted to diapering those afflicted. We have identified a specific physiological abnormality--detrusor hyperactivity with impaired contractile function (DHIC)--that, although previously unrecognized, is the second most common (33%) cause of incontinence in this setting. Detrusor hyperactivity with impaired contractile function is a distinct physiological subset of detrusor hyperreflexia and presents with a seemingly paradoxical set of findings: the bladder is overactive but empties ineffectively. This imparied emptying is due to diminished detrusor contractile function and is associated with bladder trabeculation, a slow velocity of bladder contraction, little detrusor reserve power, and a significant amount of residual urine. Aside from its high prevalence, the importance of DHIC is that it may present as urinary retention, may closely mimic prostatic outlet obstruction, may explain why past therapeutic trials for detrusor hyperreflexia have failed, and may necessitate a change in the current nosology of bladder dysfunction. Furthermore, DHIC may represent a more advanced stage in the natural history of detrusor hyperreflexia, a stage characterized by deterioration of detrusor contractile efficiency. Thus, this previously unrecognized cause of incontinence in the elderly is common and raises several important issues.

Aged↗
PubMed1980

[Toxicological studies on dibekacin for intravenous injection use. II. Chronic toxicity in rats (author's transl)].

The toxic effects of dibekacin sulfate (DKB) in male and female rats were examined in chronic toxicity test (intraperitoneal injection), and the following results were obtained. 1) No death was noted in both male and female. 2) In general conditions, the excretion of soft or diarrheal stool was noted in groups of more than 20 mg/kg of either sex. The mean body weight was less than the control during a certain period in the male group of 40 mg/kg and in the female group of 20 mg/kg. But, in the food intakes, no particular change was noted in each group of either sex. 3) In the auricle reflex, no abnormality was noted in each group of either sex. 4) In the hematological test, the findings such as an increase of BUN, anemia, etc. were noted in the groups more than 10 mg/kg of male and more than 20 mg/kg of female. 5) In the histopathological study, evident degenerative changes of renal tubular epithelia were noted in the groups which were administered more than 20 mg/kg of DKB in both male and female, but no evident pathological findings due to the renal failure were noted in the groups of less than 10 mg/kg. Several slight changes of the thyroid gland noted in a few rats of DKB administration group of both male and female seemed to be artifact, and inflammatory changes owing to intraperitoneal injection were occasionally noted in the peritoneum of DKB injected animals. 6) Considering the above results, "the maximal non effective dose" was estimated to be 10 mg/kg in both male and female.

Animals↗
PubMed2004

Impaired rectoanal inhibitory response in scleroderma (systemic sclerosis): an association with fecal incontinence.

Gastrointestinal abnormalities in systemic sclerosis (SSc) involve both myogenic and neural mechanisms. The aims of this study were to evaluate the rectoanal inhibitory response (RAIR) in SSc patients and to correlate RAIR with duration and subtype of disease, antibody status, and lower gastrointestinal symptoms. Thirty-five patients with SSc completed a questionnaire and underwent anorectal manometry (ARM). Forty-five patients without SSc served as controls. In the 35 SSc patients, 62.3% reported diarrhea, 57.1% reported constipation, and 37.1% reported fecal incontinence. Twenty-five of the 35 scleroderma patients (71.4%) demonstrated an impaired or absent RAIR, compared with none of the 45 controls (P < 0.001). Eleven of 13 incontinent SSc patients (84%) had an impaired RAIR. No correlation was found between RAIR and duration or subtype of SSc, antibody status, or presence of diarrhea or constipation. Impaired RAIR was closely correlated with fecal incontinence, suggesting a possible neural mechanism for maintenance of continence.

Adult↗
PubMed2000

Abnormal cerebral processing of oesophageal stimuli in patients with noncardiac chest pain (NCCP).

In noncardiac chest pain (NCCP), altered visceral perception may result from abnormal cerebral processing of sensory input rather than abnormalities of afferent pathways. However, the interactions between symptoms, autonomic function and oesophageal stimuli are poorly studied. Oesophageal stimulation elicits reproducible cortical evoked potentials [CEP] and modulates heart rate variability via vagal pathways, as visible on power spectrum analysis of heart rate variability [PS-HRV]. These methods are increasingly used to study the function of visceral afferent neural pathways in human. The aim of this study was to compare EP and PS-HRV during oesophageal stimuli in NCCP and controls. Twelve healthy volunteers (one female, 11 male; aged 24-51 years; mean 32 +/- 8 years), and eight NCCP patients (three female, five male; age range 26-58, mean 40.5 +/- 10 years) were studied. Electrical oesophageal stimulation (EOS; 200 microseconds, 0.2 Hz, 25 stimuli) was applied to the oesophageal wall 5 cm above the lower oesophageal sphincter (LOS), and perception thresholds (measured in mA) determined. EP responses were recorded using 22 standard electroencephalogram scalp electrodes. Autonomic activity was assessed using PS-HRV, before, during, and after oesophageal stimulation. Measured PS-HRV indices included high frequency (HF; 0. 15-0.5 Hz) and low frequency (LF; 0.06-0.15 Hz) power, respectively, assessing vagal and sympathetic activity, and the LF/HF ratio. EOS perception occurred at lower thresholds in NCCP than in controls (3. 6 +/- 1 vs. 7.8 +/- 2 mA, P < 0.05). EP amplitude was greater (13 +/- 2 vs. 6 +/- 1 microV, P < 0.0001), and latency longer in controls vs. NCCP (191 +/- 7 ms vs. 219 +/- 6 ms, P < 0.001). In NCCP, EOS decreased sympathetic outflow (low frequency peak on PS-HRV) and increased cardiovagal activity (high frequency peak, P < 0.02) to a significantly higher degree in comparison with controls. During EOS, heart rate decreased in NCCP from 68 vs. 62 beats min-1 (P < 0.003) but not in controls. In NCCP patients, EOS was perceived at lower intensities and was associated with a greater cardiovagal reflex response. EP responses associated with EOS were smaller in NCCP than in controls, suggesting that an increased perception of oesophageal stimuli results from an enhanced cerebral processing of visceral sensory input in NCCP, rather than from hyperalgesic responses in visceral afferent pathways.

Adult↗
PubMed2005

Study of the rectal electric activity of the uninhibited rectal detrusor (overactive rectum): a new concept of pathogenesis.

BACKGROUND/OBJECTIVE: One of the causes of fecal incontinence is uninhibited rectal detrusor syndrome (URDS). Patients with this condition either perceived the first rectal sensation after the onset of involuntary rectal contraction or not at all. We investigated the hypothesis that the abnormal rectal contractility in URDS may be caused by deranged rectal electric activity. METHODS: Twenty-five patients with URD (14 women and 11 men; age, 44.7 +/- 10.3 years) and 10 healthy volunteers (6 women and 4 men; age, 42.8 +/- 8.7 years) were studied. URDS was diagnosed by rectometry and provocative test. A transcutaneous EMG was performed with one electrode placed lateral to each sacroiliac joint and the third one midway between the greater trochanter and the ischial tuberosity. Two 20-minute recording sessions were performed for each subject. RESULTS: Slow waves (SWs) with regular rhythm and similar parameters (frequency, amplitude, conduction velocity) from the 3 electrodes were recorded from the healthy volunteers. They showed a significant increase in the parameters on saline filling of the rectum. The SWs of patients with URDS exhibited a "dysrhythmic" pattern with irregular parameters, which were different in the 3 electrodes and inconsistent during recording. They showed areas of tachyrhythmia, bradyrhythmia, and arrhythmia. On provoking rectal overactivity, the SWs showed an increased dysrhythmic activity. CONCLUSIONS: The patients with URD exhibited a "dysrhythmic" electric pattern with areas of variable electric activity. The tachyrhythmic areas seem to initiate the urgency and fecal incontinence of URDS. It is suggested that a disordered rectosigmoid pacemaker causes the dysrhythmic waves.

Adult↗
PubMed2006

Evidence for cardiovascular autonomic dysfunction in neonates with coarctation of the aorta.

BACKGROUND: Coarctation of the aorta (CoA) is associated with hypertension and abnormalities of blood pressure control, which persist after late repair. Assumptions that neonatal repair would prevent development of blood pressure abnormalities have not been supported by recent data. We hypothesized that early pathological adjustment of autonomic cardiovascular function may already be established in the neonate with coarctation. METHODS AND RESULTS: We studied 8 otherwise well neonates with simple CoA and compared measures of spontaneous baroreflex sensitivity, heart rate variability, and blood pressure variability with 13 healthy newborn babies. Spontaneous baroreflex sensitivity was calculated with sequence methodology from an ECG, and noninvasive blood pressure was recorded with a Portapres. Heart rate variability was determined with time- and frequency-domain measures. Blood pressure variability was measured in the frequency domain. In comparison with normal controls, neonates with CoA had raised blood pressure (78.9+/-3.8 versus 67.1+/-2.1 mm Hg), depressed baroreflex sensitivity (8.7+/-1.5 versus 13.8+/-1.1 ms/mm Hg), reduced heart rate variability (total power 16.5+/-3.1 versus 31.5+/-2.2 ms2), and an increase in the high-frequency component of blood pressure variability (3.1+/-0.3 versus 2.2+/-0. 2 mm Hg2). This is not the pattern expected if neonates with CoA simply had subclinical cardiac failure. CONCLUSIONS: These data suggest that infants with CoA already show signs of pathological adjustment of autonomic cardiovascular homeostasis. Further longitudinal studies are required to determine whether these alterations play a role in the increased risk of late hypertension in these patients.

Aortic Coarctation↗
PubMed2004

Cerebrospinal fluid analysis in fatal thallium poisoning: evidence for delayed distribution into the central nervous system.

The neurologic manifestations of thallium poisoning include a severely painful ascending peripheral neuropathy, autonomic dysfunction, cranial nerve abnormalities, and a toxic encephalopathy. Although thallium has a short half-life, these neurologic manifestations commonly progress, even as the blood concentration of thallium decreases. This suggests either that thallium persists in neuronal tissues or that it initiates an injury cascade that takes time to fully manifest. As the latter mechanism is consistent with many toxin exposures, the concept of a central nervous system reservoir for thallium is often discounted. A recent case provided a unique opportunity to evaluate this possibility. A 48-year-old man was acutely and chronically thallium poisoned by his common-law wife. During his initial exposures, only gastrointestinal symptoms manifested. Following an acute ingestion, hospitalization was required. Over 3 days, his symptoms rapidly progressed from a severely painful neuropathy to slurred speech, ptosis, confusion, coma, respiratory insufficiency, and death. Because of considerations of alternative diagnoses, 2 lumbar punctures were performed, one on admission and another on the day of his death. Serum thallium concentrations obtained from stored blood samples were paired with spinal fluid concentrations from the same days. On day 1, serum and spinal fluid concentrations were 8700 mu/L and 1200 mu/L, respectively. On day 3, although the serum concentration had fallen to 7200 mu/L, the spinal fluid concentration had increased to 2100 mu/L. This case provides evidence to support the hypothesis that thallium distributes into the central nervous system more slowly than the blood compartment, and this may in part account for the progression of neurologic findings in the setting of decreasing serum concentrations.

Consciousness Disorders↗
PubMed2000

Depressor effect by exercise training is associated with amelioration of hyperinsulinemia and sympathetic overactivity.

OBJECTIVE: In hypertensive subjects, exercise training is a therapeutic modality that not only lowers blood pressure but also corrects metabolic abnormality, such as hyperinsulinemia. Insulin causes sympatho-excitation via the modification of baroreflex, norepinephrine release, or central sympathetic outflow. However, the link between neural and metabolic changes by exercise training in hypertensive patients remains unknown. The aim of this study was to examine whether or not the blood pressure lowering effect of exercise training is associated with the improvement of insulin sensitivity in conjunction with the inhibition of sympathetic tone in hypertensive patients. METHODS: We evaluated plasma insulin levels, arterial baroreflex function and humoral parameters before and after exercise training. Twenty-nine patients with essential hypertension under hospitalization participated in the study. Before and after three weeks of exercise training (75% max VO2, 6 min, q.i.d.), 24-hour blood pressure recordings, arterial baroreflex function testing and 75 g glucose tolerance tests were conducted. Area under the curve of insulin (sigma insulin) to glucose load was calculated as an index of hyperinsulinemia. RESULTS: Three weeks of exercise training decreased the 24-hour mean arterial pressure, heart rate and sigma insulin, and improved barorefiex function. There was a significant correlation between the reduction of arterial pressure and the change in sigma insulin. Furthermore, the reduction of sigma insulin was correlated with the improvement of baroreflex function and with the decrease in heart rate. CONCLUSIONS: Exercise training lowered the arterial pressure, with parallel changes in heart rate, baroreflex function and insulin resistance. The correction of sympathetic overactivity was closely associated with the amelioration of hyperinsulinemia. Our results suggest that the improvement of neuro-metabolic factors may be involved in the depressor effect caused by exercise training.

Area Under Curve↗
PubMed1988

Supranuclear disturbances of ocular motility in Lytico-Bodig.

We found abnormal supranuclear ocular or lid motility in all of 37 patients with Lytico-Bodig (amyotrophic lateral sclerosis/parkinsonism-dementia complex). Twenty-one patients had pursuit paresis, 18 abnormal vestibulo-ocular reflex (VOR) cancellation, 15 abnormal convergence, 13 abnormal optokinetic nystagmus (OKN), 12 conjugate gaze limitation, nine nystagmus, nine saccadic paresis, and six abnormal fixation. Lid abnormalities included glabellar hyperreflexia in 21, involuntary levator inhibition in three, and blepharospasm in two. Earlier reports have indicated infrequent ocular disturbances in Lytico-Bodig, but we now find supranuclear eye and lid deficits are universal and sometimes very prominent.

Aged↗
PubMed1989

Evaluation and characterization of the hypothyroid hyt/hyt mouse. I: Somatic and behavioral studies.

Mice homozygous for the autosomal-recessive gene hypothyroid (hyt) had congenital hypothyroidism of fetal onset after 15 days postconception. Neonatal hyt/hyt mice had reduced serum thyroxine ranging from 1/5 to 1/6 of normal as well as significantly delayed somatic and behavioral development. Delayed somatic development included retarded eye opening and ear raising, and reduced body length and body weight. The hyt/hyt animals compared to their normal littermates demonstrated delayed reflexive behavior and abnormal motor and adaptive behavior. The somatic and behavioral measures clearly distinguished hyt/hyt animals from their normal littermates even without T4 determination. The somatic and reflexive behavioral abnormalities in the hyt/hyt mouse were similar to other rodent models of human congenital hypothyroidism. The hyt/hyt mouse provided an ideal model for exploring the effect of severe primary inherited hypothyroidism related to deficient autonomous fetal thyroid function and was consistent with the hypothesis that thyroid hormone deficit in utero and in the early neonatal period significantly altered functional development.

Animals↗