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A case is presented of a 33-year-old male with ophthalmoplegia, ataxia and areflexia followed by complete recovery. There was no clinical evidence of motor weakness or sensory disturbances in the limbs. Electromyography and nerve conduction studies were normal. Sural nerve biopsy during the sixth week of illness revealed no abnormalities.
Cardiovascular responses on active standing in children with unexplained syncope were investigated with continuous non-invasive finger artery pressure monitoring (Finapres). We examined 34 symptomatic patients (13 boys and 21 girls), aged 8-16 years, and 24 age-matched controls. Finger blood pressure and heart rate were monitored continuously for 5 min in the supine position and for 10 min while standing. Ten of 34 patients developed fainting symptoms with hypotension during upright posture (fainters). In the initial standing phase (0-30 s), two prominent abnormal blood pressure responses were found in patients: a marked decrease 45 +/- 18/23 +/- 8 mmHg and a prolonged recovery time (16.5 +/- 2.9 versus 27.7 +/- 13.6 s), which appeared to be based on impaired vasoconstriction. Either or both abnormalities were observed in 21 (62%) of the 34 patients and in 2 (8%) of the controls. In addition, fainters also had a more marked increase in heart rate during standing compared with non-fainters. Our findings suggested that more than half of children with syncope had abnormal cardiovascular reflexes in the initial phase which appeared to be associated with vasodepressor syncope. The active standing test with a continuous beat-to-beat blood pressure recording has a high sensitivity in detecting abnormalities of autonomic function in patients with unexplained syncope.
We obtained micturitional histories and performed urodynamic studies in six patients with pure autonomic failure. All patients had urinary symptoms. Urodynamic studies showed postmicturition residuals in two, small bladder capacities in two, detrusor hyperreflexia in four, low bladder compliance in two, detrusor-external sphincter dyssynergia in one, neurogenic sphincter electromyography in three, and denervation supersensitivity of the bladder in two. Micturitional disturbance is a common feature in pure autonomic failure because of peripheral and central types of abnormalities.
BACKGROUND: Vogt-Koyanagi-Harada (VKH) syndrome is a systemic condition characterized by ocular inflammatory disease as well as skin, ear, and meningeal manifestations. Patients with VKH often report tinnitus and hearing loss, but these symptoms tend to be given secondary consideration because most undergo treatment with steroids to prevent blindness resulting from granulomatous uveitis, exudative retinal detachment, and optic nerve inflammation. METHODS/STUDY DESIGN: In the current retrospective review, 24 patients with this syndrome were screened for auditory system abnormalities. All patients denied history of noise exposure or ototoxic agent exposure. The age range of the patients was 13 to 42 years. RESULTS: Three patients reported tinnitus and two patients reported sudden hearing loss. One patient experienced vertigo and aural fullness. Eight of 24 patients had pure-tone thresholds greater than 25 dB hearing loss at two or more frequencies. Five of 24 of these patients experienced hearing loss outside of the 95% confidence interval for published age-matched control populations. There was sloping sensorineural hearing loss at 4 kHz and above in five of 24 patients. All eight patients with hearing loss experienced some degree of hearing loss at 4 kHz or above. Three patients had mild to moderate low-frequency sensorineural hearing loss. There were no tympanometric abnormalities suggestive of conductive involvement. Abnormal acoustic reflex decay was observed in one patient. CONCLUSIONS: We conclude that a significant number of patients with VKH experience sensorineural hearing loss and that every patient with VKH should undergo a review of systems for auditory abnormalities and referral for audiologic testing if symptomatic. It is possible that untreated patients may experience worse symptoms.
The growth of ultrasound applications in diagnostic medicine has helped stimulate related biological effects investigations. Current data related to effects associated with diagnostic ultrasound indicate the need for additional research on cell surface structures, motility and developmental effects. Research on biological effects, especially for evaluating in vivo end points analogous to those employed by investigators using in vitro systems and simple organisms, are needed. Particular emphasis relative to potential effects on fetal and embroyonic development is indicated. Limited data also suggest the need to investigate possible effects on the immune response. There is a growing realization of the potential importance of nonthermal effects and increasing evidence that the temporal peak intensity is potentially related to the production of some effects. A number of recent comprehensive reviews have helped identify, analyze and evaluate some of the relevant data.
The neurophysiological findings in eight patients with the stiff man syndrome (SMS), including four of six tested with autoantibodies against glutamic acid decarboxylase, are presented. Neurophysiological findings did not make it possible to discriminate between patients with and those without autoimmunity against GABAergic neurons. Investigation of mono- and polysynaptic reflexes revealed abnormal results in a variable number of SMS patients, the abnormalities largely corresponding to those seen in spastic paresis. A stereotyped motor response to electrical stimulation of peripheral nerves was recorded from the trunk muscles of all patients investigated. This response was termed spasmodic reflex myoclonus and consisted of a sequence of 1-3 synchronous myoclonic bursts, 60-70 ms after median nerve stimulation, followed by a tonic decrescendo activity over a number of seconds. The recruitment order of muscles along the neuraxis in spasmodic reflex myoclonus suggested that the latter was generated in the spinal cord and conveyed via propriospinal tracts. It is thought that spasmodic reflex myoclonus may serve not only as a diagnostic tool, but also as a key to understanding some aspects of the pathophysiology of both spasms and stiffness in SMS. It is speculated that stiffness is a fragment of spasms, both being generated by common neuronal mechanisms tentatively ascribed to interneurons in the spinal grey matter.
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The purpose of this study was the identification of possible sequelae of the infection of human individuals with Virus of Venezuelan Equine Encephalitis (VEE). Special emphasis was laid on exploring neurological, psychological and behavioural aspects and particularly on the search for a possible association of the disease with epileptic phenomena, brain damage and/or mental deficiency. A four-year period of observation was conducted on a sample of children from El Carmelo (Colombia) where an epidemic of VEE took place in 1967. A group of seven children who presented the encephalitic type of the illness and were hospitalized with symptoms of CNS involvement and a confirmed diagnosis of VEE constituted the index group. This group was compared with four control groups of children with matched demographic characteristics but separated according to whether they were ill at the time of the epidemic and whether they presented serological evidence of having been infected with VEE Virus. Evaluations were conducted at three points in time during the follow-up period and they included the following procedures: (1) Survey on personal background and behaviour (through a standard questionnaire dealing with all areas of functioning); (2) Complete paediatric examination, including a careful neurological check-up; (3) Psychological examination using intelligence tests; (4) Electro-encephalographic examination according to standard techniques, with the patient awake, asleep and under photic stimulation; (5) Bi-weekly home visits to keep a record on intercurrent illness throughout the observation period. Statistical analysis of results shows significant differences between the groups in regard to the distribution of abnormalities.
1. A postulated role for the bronchial circulation in the development of pulmonary congestion may be based on recent studies of bronchovascular control. 2. The bronchial circulation is the nutrient blood supply of the conducting airways and, therefore, plays an important role in the function of the bronchial mucosa. Mucosal swelling secondary to elevation of mucosal capillary hydrostatic pressure may decrease airway calibre, increase resistance to airflow and precipitate symptoms of pulmonary congestion. 3. Resting mucosal capillary hydrostatic pressure is relatively constant due to autoregulation of bronchial blood flow and is maintained low by nett bronchovascular constriction due to the dominance of autonomic vasoconstriction over nitric oxidedependent vasodilatation. 4. Bronchial blood flow is also regulated by cardiac afferent reflexes. Stimulation of cardiac vagal and spinal afferents produces vasodilatation and vasoconstriction, respectively. Tonic activity of cardiac spinal afferents probably contributes to the resting autonomic vasoconstriction. 5. Therefore, mild heart failure, which is associated with abnormal cardiovascular reflex function, may decrease cardiac spinal afferent-mediated bronchial vasoconstriction and produce active dilatation due to stimulation of cardiac vagal afferents by excessive myocardial stretch, leading to bronchial mucosal swelling and pulmonary congestion.
Six patients with alternating hemiplegia of childhood (AHC) underwent motor evoked potentials (MEPs), somatosensory evoked potentials (SEPs) and blink reflex recording. No SEP abnormality was found. As for MEP recording, central conduction time did not differ between patients studied during either interictal or ictal phase, and normal subjects, suggesting that the pyramidal system function is spared. In blink reflex recording, the latencies of both the ipsilateral (iR2) and contralateral (cR2) R2 components were significantly longer (P<0.01) in patients during the interictal phase than in normal subjects. Moreover, the iR2 and cR2 areas were significantly reduced (P<0.01) in patients during the interictal phase, compared with normal subjects. During the ictal phase, the ipsilateral R2 latency was significantly decreased after stimulation of the hemiplegic side, compared with the interictal phase (P<0.05). The blink reflex abnormalities suggest a brainstem dysfunction, which may be linked to the pathophysiological mechanisms of the disease.
Analysis of the temporal sequence of neurologic events, neurophysiologic abnormalities, and longevity in 36 Niemann-Pick type C patients revealed two clinical subgroups with five stages of severity within each group. Patients with a preschool onset (group I; n = 18) had a higher mortality than did patients with a school-age onset (group II; n = 18). An asymptomatic phase (stage 0) was defined by biochemical and histopathologic evidence of disease. The initial manifestations of stage 1 were a movement disorder (group I) and cognitive difficulties (group II) accompanied by impaired vertical saccadic eye movements and abnormal acoustic reflexes. Stage 2 was characterized by the sequential occurrence of vertical supranuclear gaze palsy (VSGP), cognitive difficulties, and dysarthria in group I and a movement disorder, VSGP, and dysarthria in group II. Pyramidal tract signs and abnormal brainstem auditory evoked responses defined stage 3 in both groups. Stage 4 culminated in a nonambulant, vegetative state.
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Perineal sensory and motor function was investigated in 28 women with genuine stress incontinence of urine and compared with a matched control group. Electrosensitivity of the dorsal nerve of the clitoris and of the urethral mucosa was significantly diminished in these patients (eight measurements 'insensitive'). Three different reflex latency measurements (dorsal nerve to external anal sphincter, dorsal nerve to urethral sphincter, urethral mucosa to external anal sphincter) were prolonged in incontinence (14 absent reflexes). Mean motor unit potential duration of the external anal sphincter was also prolonged, reflecting an early neuropathy. Anorectal manometry detected significantly weaker squeeze pressures in stress incontinence although other variables were unaffected.
Duane's syndrome is a congenital eye movement disorder characterized by marked limitation or absence of abduction, variable limitation of adduction, and narrowing of the palpebral fissure with retraction of the globe on attempted adduction. We treated a patient with bilateral Duane's syndrome associated with crocodile tear and external, middle, and inner ear anomalies. Exploratory tympanotomy showed an absence of the oval window and a rudimentary superior structure of the stapes that was connected to the abbreviated incudal lenticular process with connective tissue. A small hole was drilled into the vestibulum and the ossicular chain was reconstructed by inserting a Teflon wire piston to minimize the air-bone gap. Pertinent literature is reviewed, and the pathogenesis of Duane's syndrome and the aspect of concomitant anomalies are discussed.
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A 60-year-old right-handed man showed dysprosody and agnosia for environmental sounds. His mother tongue was Japanese, and he could not speak foreign languages. He gradually developed difficulty in speaking from the age of 57 years, speaking non-native Japanese. In addition, he often complained of difficulty in hearing sounds, but audiometry showed no abnormalities. At the age of 60 years, the standard language test of aphasia showed no abnormalities in repetition, verbal comprehension, or reading, suggesting the absence of aphasia. However, in speaking, marked abnormality in rhythm, and occasional lack of postpositional particles and syllable-stumblings were observed. Writing was almost accurate, but a few grammatical errors were observed in speaking were observed. There were no cerebellar symptoms, pyramidal signs, pathologic reflexes, or abnormalities in phonation-related organs. Though the recognition of verbal sounds was maintained, impairment in the recognition of non-verbal sounds was observed. An environmental sound perception test showed correct answers only in 8 of 21 non-verbal sound sources (such as a car starting, glass breaking and so on), suggesting agnosia for environmental sounds. He insisted that the difficulty in perception was due to hearing impairment. However, re-examination with an increase in the sound volume showed similar results. He had no inconvenience in daily life and was not aware of agnosia for environmental sounds. He could recognize and differentiate sounds he heard once. His intelligence was normal, and neither apraxia nor frontal lobe symptoms were observed. MRI of the brain revealed slight atrophy of the right temporal lobe. Cerebral blood flow SPECT showed decreased blood flow from the superior temporal gyrus to the area around the arcuate fasciculi in the right temporal lobe. We considered that the lesion responsible for environmental auditory sound agnosia was present in the area around the secondary auditory area of the right temporal lobe and this patient differed from slowly progressive aphasia characterized by decreased blood flow in the left temporal lobe. Although the pathological process occurring in the area of hypoperfusion remained unclear, early stage of some degenerative disorders was more likely than cerebrovascular disease.
In this paper, one case of syndrome of caudal suppression associated with a syndrome of Klippel Feil is discussed. It concerned a 14 years old boy, born form a diabetic mother with congenital abnormalities such as: urinary incontinence, facial dysmorphia, a short neck, an arachnodactylia, a lumbar kyphosis, a permanent flexion of the knee, a kalipe cavus, the spine, a partial agenesis of the third and fourth lumbar vertebrae a total agenesis of the fifth and sacrum; This syndrome is infrequent and the pathophysiological mechanisms are still under discussion and no therapeutic strategy is proposed. This case offers an opportunity to the authors to make review of the medical literature on this subject. They emphasized on the interest to look for a vertebro-medullary malformation every time a child comes to us with walking problem or enuresia, this fact should be enforced when mother is diabetic.