Absence of brainstem reflexes with intact Vth and VIIth nerves in Shy Drager syndrome.
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Brain Stem↗
Biomedical subjects
D Mancia
Publications and source records attributed to D Mancia.
Precocious loss of physiological sleep in a case of Creutzfeldt Jakob disease: a serial polygraphic study.
Creutzfeldt-Jakob disease (CJD) is a prion-related subacute encephalopathy producing widespread neuronal degeneration and spongiform pathological changes, especially in the neocortex. Progressive dementia, motor signs and electroencephalographic (EEG) alterations characterize the full stage of the disease. A series of eight 24-hour polygraphic recordings were carried out in the last 3 months of life of a 68-year-old female patient affected by CJD that was confirmed neuropathologically. Genetic classification demonstrated this patient to have a sporadic form of the disease. The polygraphic recordings demonstrated three types of EEG findings, as follows: 1) sustained pseudoperiodic discharges (SPD), characterized by long-lasting diffuse sequences of slow sharp waves or di- or triphasic slow waves recurring at 0.5- to 1.5-second intervals; 2) discontinuous pseudoperiodic discharges (DPD), consisting of runs of pseudoperiodic discharges (PD)(phase A) cyclically replaced at about 1-minute intervals with semi-rhythmic theta-delta activities (phase B); 3) non-rapid eye movement (NREM) sleep-like pattern, with dominant 0.5- to 4-Hz activities, less rhythmic than the EEG of phase B. Only these three EEG patterns occurred spontaneously during the repeated polygraphic sessions. The NREM sleep-like pattern was found only in the first recording, whereas the following polygraphic sessions were occupied exclusively by SPD or by a DPD pattern. SPD was associated with either a relatively high level of vigilance (along the first three recordings) or a state of alert-appearing silent immobility (following the fourth recording). During DPD, the patient was unable to accomplish any voluntary movement and fluctuated between levels of greater arousal (phase A) and lesser arousal (phase B). Just as in stage 2 coma, the fluctuations between phases A and B of DPD were synchronous with phasic modifications of muscle activity and neurovegetative functions. In particular, reinforcement of muscle tone and myoclonic spasms coincided with phase A, whereas heart rate deceleration and respiratory pauses or decrease in flow were synchronous with phase B. As EEG evolved toward the disappearance of DPD and finally to flatness, the phase-locked coordination among arousal, somatic and vegetative activities was progressively impaired and was replaced with an uncontrolled exaggeration of cardiorespiratory activity. The genetic, neuropathological and polysomnographic differences between CJD and another prion disease, fatal familial insomnia, are discussed.
Aged↗
Migrant sensory neuritis associated with AIDS: case report.
We studied an AIDS patient who suffered from numbness, paresthesias and pain in the territories of different non-contiguous cutaneous nerves at different times. A transitory partial loss of touch and pinprick sensibility was also present in the same cutaneous areas. Sensory conduction velocities and SAP amplitudes were normal. The clinical picture was consistent with the Migrant Sensory Neuritis of Wartenberg. This rare neuropathy has never previously been described in patients affected by AIDS.
Acquired Immunodeficiency Syndrome↗
Reliability of EEG in the diagnosis of Creutzfeldt-Jakob disease.
Although EEG is generally considered a useful tool for the diagnosis of Creutzfeldt-Jakob disease (CJD), some cases have been reported where the EEG was non-specific. We reviewed a series of 15 CJD patients, observed in our institute in the period 1975-91. In 12 cases the diagnosis was confirmed on post-mortem examination. The prominent aspect of the present series was the homogeneity of clinical, neurophysiological and neuropathological data. All patients showed the presence of periodic sharp wave complexes (PSWC) and EEG reactivity to external stimuli or drugs was uniform. The EEG can give essential information for the diagnosis of CJD if 2 basic conditions are satisfied: (1) serial recordings are performed in relation to the different stages of the disease, and (2) not only the presence of PSWC is considered, but also the reactivity of EEG to dynamic events such as the response to external stimuli and drugs, and the level of consciousness.
Adult↗
Steadiness of amyotrophic lateral sclerosis in the province of Parma, Italy, 1960-1990.
A clinical and epidemiological study on amyotrophic lateral sclerosis (ALS) was conducted in the province of Parma, Italy, from 1960-1990. A total of 121 cases were collected from hospital records. The average annual incidence was 0.98 per 100,000 inhabitants, with a male/female ratio of 1.1. Age-specific incidence was maximal in the age group 60-69 years. No difference between rural and urban areas was found. Prevalence on October 26th, 1981 was 2.5 per 100,000. Mean age at onset was 60 years, with no significant sex difference. Mean duration of the disease was 30 (sd 21.4) months. Bulbar forms were significantly (p < 0.05) shorter than conventional forms, with a mean duration of 23.4 (sd 21.4) months. Age at onset did not influence prognosis. A comparison of three decades was made, to verify whether possible variations of the disease had occurred with time. From our data a definite stability was found in such epidemiological parameters as incidence, prevalence, mean duration and mortality of ALS in the period.
Adult↗
Peripheral neuropathy associated with primary Sjögren's syndrome.
Clinical and electrophysiological signs of peripheral neuropathy were found in 10 of 46 patients (21.7%) with primary Sjögren's syndrome, symmetric polyneuropathy in seven (mainly sensory in five, mainly autonomic in two), sensory neuronopathy in two patients, and mononeuropathy multiplex in one patient. Peripheral neuropathy was the presenting manifestation in five patients (10.9%). Onset of the disease after 50 years was significantly more common in the polyneuropathy group (six of seven) than in non-neuropathic patients with primary Sjögren's syndrome (14 of 36; p = 0.034). No other difference in clinical or laboratory variables between neuropathic and non-neuropathic patients with primary Sjogren's syndrome was found. Neurophysiological study showed variable findings predominantly suggesting an axonopathy. Nerve biopsy showed moderate remyelination and regeneration in four patients, and fibre loss, mainly of large size, in three. Necrotising vasculitis was not seen but alterations of the endoneurial microvessels were prominent.
Adult↗
Post-anoxic theta and alpha pattern coma.
Theta pattern coma is a rare EEG finding, described in the course of post-anoxic or post-traumatic coma and interpreted as a "malignant" variant of alpha pattern coma. A case of isolated theta pattern coma, followed by alpha pattern coma is reported. Short temporal succession of such patterns would seem to confirm the hypothesis of a close relationship between them. Possible physiological mechanisms involved in the generation of the patterns are discussed.
Alpha Rhythm↗
On the cause of tendon areflexia in the Holmes-Adie syndrome.
A case of Holmes-Adie syndrome was studied clinically and electrophysiologically. A total loss of tendon reflexes was observed in upper and lower limbs. The main electrophysiological finding was a marked increase of the central conduction time after stimulation of peroneal and tibial nerves. Motor evoked potentials in soleus muscle after transcranial magnetic stimulation showed normal threshold and latencies. We suggest that tendon areflexia could be a consequence of a dysfunction of somatosensory large diameter afferent fibres at the spinal level.
Adie Syndrome↗
Acute sensory and autonomic neuropathy: possible association with coxsackie B virus infection.
This report describes a 26 year old woman with a Coxsackie B virus infection complicated by an acute pandysautonomic and sensory neuropathy. Electrophysiological studies suggested an axonal neuropathy. A sural nerve biopsy performed early in the disease showed axonal degeneration with a virtual absence of unmyelinated fibres and moderate loss of myelinated fibres, mainly affecting the small fibres; this differs from previous reports. An immune-mediated or direct virus action might explain the pathogenesis of this unusual evolution of a viral infection.
Adult↗
Presurgical electrophysiological findings in acoustic nerve tumours.
The clinical involvement of the facial nerve is a rare finding among the initial symptoms of acoustic neurinomas. However, compression of the facial nerve is a common intraoperative finding. Blink reflex was recorded in 20 patients affected by cerebellar-pontine angle tumor confirmed at surgery. Recordings were also made of the M-response of the facial nerve from the naso-labial folds. In 6 cases jaw reflex was also recorded. In summary, these electrophysiological studies revealed a facial nerve damage in 13 and a trigeminal nerve dysfunction in 2 out of 18 clinically unaffected patients. The combined study of the 3 tests proved to be useful when the blink reflex showed an isolated R1 delay, that is, in cases in which the level of damage along the trigemino-facial reflex arc cannot be defined by the recording of the blink reflex alone.
Adolescent↗
Magnetic motor evoked potentials (MEPs) in masseter muscles.
Electromyographic responses of the masseter muscles and orbicularis oris muscles following transcranial magnetic stimulations were recorded with surface and needle electrodes. MEPs in masseter muscles (latency 6.9 +/- 0.71 ms, mean +/- SD) due to activation of controlateral cortico-nuclear connections were evoked by magnetic stimulations at 4 cm laterally to the vertex on the biauricular line. These MEPs were followed bilaterally by a silent period lasting about 20 ms and, less constantly, by a later silent period lasting up to 80 ms. The ipsilateral responses to the same stimuli presented shorter latencies and higher amplitudes and they were ascribed to direct stimulation of the trigeminal nerve, probably its intracisternal portion. Ipsilateral masseter "cortical" MEPs could be elicited only by a lower output setting (70% of the maximum output) of the stimulator. Orbicularis oris MEPs were polyphasic and dispersed with latencies ranging from 7 to 11 ms. In patients with hemispheric or capsular ischemic lesions "cortical" MEPs were absent when stimulating the affected hemisphere and present when stimulating the unaffected one. We suggest that the direct corticomotoneuronal projections for the masseter are mainly crossed.
Adult↗
Motor-evoked potentials in masseter muscle by electrical and magnetic stimulation in intact alert man.
The electromyographic responses of the masseter after different types of transcranial stimulation were recorded with surface and needle electrodes. Magnetic stimulation at 4 cm lateral to the vertex on the biauricular line elicited MEPs in the contralateral masseter (latency 6.9 ms) due to activation of motor cortex or adjacent elements along the cortico-nuclear pathway. The ipsilateral responses to the same stimuli and to more lateral ones had shorter latencies and were ascribed to direct stimulation of the trigeminal nerve, probably its intracisternal portion. This was also the probable origin of the ipsilateral MEPs after both anodic and cathodic bipolar electrical stimulation at 7 and 11 cm lateral to the vertex on the biauricular line.
Adult↗
[Masseteric responses following electrical stimulation of the scalp].
Electromyographic responses of the masseter muscles following electric transcranial stimulations by a conventional constant current stimulator were recorded with surface and needle electrodes. Ipsilateral motor evoked responses following both anodic and cathodic bipolar electrical stimulations performed at 7 and 11 cm laterally to the vertex on the biauricular line were recorded, with latencies ranging from 2 to 3.6 ms. Contralateral responses were not elicited. The ipsilateral responses to stimuli were ascribed to direct stimulation of the trigeminal nerve, probably its intracisternal portion.
Adult↗
[Magnetic stimulation of the scalp: the responses of the masseter muscles. I].
Magnetic stimulation of nervous elements is a new neurophysiological technique. It easily succeeds in stimulating the brain motor cortex. We applied this technique to the trigeminal motor system, recording responses in masseter muscles. The magnetic stimulations at 4 cm laterally to the vertex on the biauricular line elicited responses in the contralateral masseter due to activation of motor cortex or adjacent elements along the cortico-nuclear pathway. The ipsilateral responses to the same stimuli and to more lateral ones presented shorter latencies and they were ascribed to direct stimulation of the trigeminal nerve, probably its intracisternal portion. The latency values were 6.9 +/- 0.71 ms and 3.6 +/- 0.3 ms respectively.
Adult↗
[Magnetic stimulation of the scalp: the responses of the masseter muscles. II].
Magnetic stimulation of nervous elements is a new technique of investigation in intact alert man. Electromyographic responses of the masseter muscles were recorded in normal volunteers and in two patients with hemispheric or capsular lesions, both by surface and needle electrodes. In the patients "cortical" responses were absent when stimulating the affected hemisphere and present when stimulating the unaffected one. These findings suggest that the direct facilitating cortico-nuclear projections for the masseter muscle are mainly crossed. Nevertheless the presence of clear ipsilateral responses after stimulation of the unaffected hemisphere demonstrates the existence of uncrossed projections; in fact only the ipsilateral motor cortex can be the site of origin of the responses in these patients.
Adult↗
Anterior interosseous nerve syndrome secondary to forearm thrombo-phlebitis.
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Female↗
Carpal tunnel syndrome presenting with prominent skin symptoms.
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Carpal Tunnel Syndrome↗
Polyneuropathy and systemic vasculitis. An electrophysiological study.
A review of cases of systemic vasculitis followed for one year in a rheumatology department resulted in the detection of 18 patients who on the clinical and electrophysiological evidence had symmetrical distal polyneuropathy. A moderate impairment of the motor conduction velocity was present in 7 patients, with electromyographic neurogenic changes in the distal lower limbs of all but one patient. The sensory action potential of the sural nerve was bilaterally absent in one case, and its amplitude was reduced in 14 out of 16 patients, with a decreased sensory conduction velocity in 9 cases. The sural nerve biopsy, performed in 6 patients, was prevalently suggestive of previous axonal degeneration. This investigation illustrates the spectrum of diffuse peripheral nerve involvement associated with systemic vasculitis. A variable impairment of the sensory action potential, ranging from slight decrease of amplitude to no response, is the most common finding. Conduction velocities appear to be relatively spared.
Adult↗