Frontal intermittent rhythmic delta activity (FIRDA) in patients with dementia with Lewy bodies: a diagnostic tool?

The accuracy of the clinical diagnosis of dementia with Lewy bodies (DLB) remains poor, especially in early phases of the disease, in spite of applying current consensus diagnostic criteria. The need for supportive diagnostic tools is therefore warranted. In this study EEG recordings showed a main pattern of bilateral frontal intermittent rhythmic delta activity (FIRDA) in 7 of 10 patients, aged 58-83 years, 8 of whom were diagnosed as affected by "probable" and 2 by "possible" DLB. Conversely, the same EEG abnormality was found only in 2 of 9 age-matched patients, 8 of whom had "probable" and 1 "possible" Alzheimer's disease, according to NINCDS-ADRDA criteria, taken as controls. The degree of cognitive impairment was comparable among the two groups of patients. If these findings will be confirmed in a larger series, FIRDA, even though an aspecific EEG pattern, could be of value in improving the diagnostic accuracy of DLB.

Triphasic waves associated with acute naproxen overdose: a case report.

Triphasic waves (TWs) can be recorded on EEG in the course of several metabolic disorders, mainly hepatic encephalopathy. A case of acute encephalopathy due to naproxen intoxication is reported, in the course of which diffuse, bilateral and symmetrical TWs were recorded. Biochemical mechanisms that might determine both a complex encephalopathy and TWs are discussed.

The masseteric inhibitory reflex in the diagnosis of multiple sclerosis.

In order to evaluate the reliability of the masseteric inhibitory reflex (MIR) as a screening method in the diagnosis of multiple sclerosis (MS), a series of 41 consecutive patients affected by clinically defined, long-duration forms (mean duration 10.9 years) of the disease was examined. In all cases magnetic resonance imaging and CSF isoelectrofocusing confirmed the diagnosis. Sensitivity of MIR, Blink Reflex and BAEPs were compared. Statistical analysis of data suggested the following considerations: 1) a significant concordance was found between MIR and the other neurophysiological tests performed (MIR vs. BAEPs in 78.4% of cases, p < 0.001; MIR vs. blink reflex in 68.3%, p < 0.02). 2) The S1 early component of MIR is a more reliable indicator than S2 late component. 3) In detecting brainstem lesions the sensitivity of MIR equaled that of the other neurophysiological tests. 4) Poor localizing concordance between neurophysiological tests and neuroimaging was found in our series. A possible utilization of MIR, as a part of a multimodal neurophysiological approach, even in patients affected by possible or probable MS is suggested.

Spindle coma and alternating pattern in the course of measles encephalitis.

Acute disseminated encephalomyelitis (ADEM) is a well known complication of measles infection. Electroencephalographic abnormalities may occur frequently, in the form of moderate to severe diffuse high voltage theta-delta activity. We report a case of measles encephalitis with rapid benign outcome, that showed peculiar EEG features both of a "spindle coma" pattern and of an "alternating pattern." Possible physiological explanations of the EEG findings are discussed.

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.

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.

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.

Growth of heifers fed 100 or 115% of National Research Council requirements to 1 year of age and then changed to another treatment.

Eighty-nine Holstein heifers were used from 3 mo of age until 21 d before their estimated calving date to determine the effect of two feeding programs on growth and onset of puberty. From 3 to 12 mo of age, heifers were fed either 100 or 115% of 1989 NRC nutrient requirements for heifers gaining .7 kg/d. At 12 mo, the treatments were switched (100 to 115% and 115 to 100%) until 21 d before calving. When the 115% diet was fed from 3 to 12 mo of age, rate of BW gain, BW at 12 mo, body condition score, and heart girth increased. Heifers fed the 115% diet from 3 to 12 mo of age were 22 d younger at onset of puberty than those fed the 100% diet, but BW at onset of puberty was similar (281 vs. 278 kg, respectively). When diets were switched at 12 mo of age, the rates of BW gain and growth for the heifers switched from 115 to 100% decreased, but rate of BW gain and growth increased for the other group. At 24 mo of age, BW and body size did not differ between treatments. Neither feeding program resulted in a clear advantage for any trait measured, but the results provide further evidence that extra nutrients fed beyond the NRC requirement for .7 kg/d of BW gain enhance various growth traits in Holstein heifers.

Unilateral trismus caused by vertebrobasilar dolichoectasia.

Vertebrobasilar dolichoectasia is a relatively uncommon pathological entity and generally asymptomatic. We report a quite unusual case of unilateral motor trigeminal involvement with trismus, due to VD. Apart from tetanus or local morbid conditions of the mouth, trismus is often attributed to disturbed programming and co-ordination of the masticatory muscles within the mesencephalic nucleus. The possibility of truncal compression of the trigeminal motor root by vertebrobasilar dolichoectasia being responsible for masseter muscle spasm, in analogy with the pathogenetic mechanisms proposed in hemifacial spasm, is proposed as an alternative explanation in the present case.

Adult postanoxic "erratic" status epilepticus.

A 66-year-old woman with posttraumatic anoxic coma after diffuse cerebral fat embolism had continuous alternating-side myoclonic jerks. Usually, this kind of myoclonic status epilepticus (SE) occurs in newborn infants. We postulate the unusual combination of diffuse cerebral anoxia plus commissural fiber damage as a possible explanation.

Transient traumatic reticular myoclonus. Case report.

Reticular myoclonus in man is a rare manifestation, generally due to uremia or post-hypoxic conditions. The case of a patient who showed transient myoclonus of sternocleidomastoid and facial muscles, immediately after direct cervical trauma, is described. Absence of paroxysmal EEG activity, topographic distribution of jerks and sequence of muscle activation suggest a reticular myoclonus. Possible physiopathological mechanisms are discussed.

MR findings in Meige syndrome.

A 65-year-old woman was diagnosed clinically to have Meige syndrome in the oromandibular dystonic clinical variant of Marsden. On magnetic resonance imaging a reduced signal intensity in the head of the caudate nuclei, more prominent on the right, and in both putamina was shown.

Myokymia in the course of Bell's palsy. An electromyographic study.

Facial myokymia has been described in association with many morbid conditions, most frequently multiple sclerosis (MS) and brainstem tumors (BST). Very few reports deal with myokymia in the course of Bell's palsy, despite high frequency of the disease. A series of 88 consecutive patients with Bell's palsy is presented, in which serial EMG controls were performed in the initial phases of facial palsy. Twenty three showed myokymic activity detected by EMG, while clinically evident myokymia could be observed only in 8. EMG features did not reveal any peculiarity as compared with myokymic discharges reported in other pathological situations. Considering the high incidence of myokymic activity reported in the course of Bell's palsy, it is the authors' opinion that an occasional finding of myokymia, both clinically evident or detected by EMG, should not necessarily lead one to suspect serious aetiologies.

[Prevalence and incidence of multiple sclerosis in the province of Parma, 1965-1980. Preliminary data]. / Prevalenza e incidenza della sclerosi multipla nella provincia di Parma nel periodo 1965-80. Dati preliminari.

Recent epidemiological studies in small communities report very high prevalence rates of multiple sclerosis (MS), suggesting that Italy should be included among high risk areas for MS. Such data are not confirmed when populations exceeding 300.000 are considered. The total prevalence rate of probable MS in the province of Parma was 23.07 per 100.000 on the prevalence day Dec. 31, 1980. Up to date, very few studies deal with incidence of MS, due to the fact that in many cases it is difficult to establish the exact outburst of the disease. Yet, incidence will indicate the real risk for the disease for a given period in a more accurate manner. Mean annual incidence of MS in the province of Parma in the period 1965-80 was 1.14 per 100.000. A previous survey in the period 1950-59 reported, for the same area, an incidence rate of 1.01 per 100.000, versus a prevalence rate of 12.33 per 100.000. In our opinion, when comparison of data between two different areas, in different periods, is to be made, incidence rates for MS should be preferred to prevalence studies.

The sunflower syndrome.

Some photosensitive epileptic patients show absence attacks preceded by a "seeking of a source of light" and stereotyped movements of various kinds. The common explanation is an attempt to self-induce seizures. The case reported is of a young girl who had not shown evidence of photosensitivity for many years. Alternative interpretations of this phenomenon are discussed.

Hypercalcemic encephalopathy in the course of hyperthyroidism.

A hypercalcemic condition can be observed in association with hyperthyroidism. The case of a patient suffering from hypercalcemia and hyperthyroidism is reported. A confusional state and EEG alterations, among which diffuse monomorphic delta rhythms were remarkable, are shown. As soon as normalization of calcemia was achieved, a rapid clinical and EEG improvement took place. A hypothetical interpretation is proposed, according to which a prolonged, though inconstant, and mild hypercalcemia in the course of hyperthyroidism could determine an encephalopathy, concealing in some way thyrotoxic symptoms.

Isolated benign cerebral vasculitis. Case report and review.

A case of isolated cerebral vasculitis showing benign outcome is presented. Review of pertinent literature suggests that isolated benign arteritis of the CNS should be considered as a distinct entity, in a heterogeneous group of vasculitides. The common features of the syndrome are as follows: 1(0) benign evolution; 2(0) sensitiveness to cortisone; 3(0) absence of CSF alterations; 4(0) constant angiographic pattern of arteritis. Early cortisone treatment is suggested in any case of cerebral arteritis.

Amyotrophic lateral sclerosis in the province of Parma, Italy: a clinical and epidemiological study in the period 1960-1980.

A clinical and epidemiological study on ALS was conducted in the province of Parma, Italy, in the period 1960-1980. A total of 78 cases was collected from hospital records. The average annual incidence was 0.93 per 100,000 with no difference between rural and urban areas. On prevalence day (October 24, 1971) a prevalence rate of 2.27 was found. Mean age at onset was 59 years, with no sex difference. In 70% of cases the onset was between 50 and 70 years, this datum being statistically significant (p less than 0.001). Mean duration of the disease was 30.4 +/- 20 months, with no difference for sex and age at onset. A comparison was then made between two decades (1961-70 vs 1971-80), in order to verify the clinical impression of a worsening of some ALS parameters in recent years. Although statistical evaluation did not show any significant data, a tendency to a worsening of some parameters of the disease may be postulated.

[Autoscopic occipital seizures and occipital poroencephalic lesion: considerations on a case]. / Crisi occipitali autoscopiche e lesione poroencefalica occipitale: considerazioni a proposito di un caso.

Autoscopy is an hallucinatory phenomenon during which the subject see his own image. It may be caused by organic processes like migraine, vascular diseases, tumoral lesions, and exceptionally by epileptic seizure. The case of 15 years old boy is reported, affected by hemianopia, surgically treated squint, who presented occipital epileptic seizures consisting of autoscopic hallucinations, leftward conjugate eye deviation, followed by a typical major seizures. A right parieto-occipital epileptic focus was a constant finding on EEG. On CT a poroencephalic cyst in the corresponding cerebral region could be demonstrated.