Partial epilepsy in neurologically normal children: clinical syndromes and prognosis

A clinical and electroencephalographic study of 107 neurologically normal children with partial seizures was undertaken to verify the existence and determine the frequency of epileptic syndromes reported in selected populations. Sixty-three children had simple partial seizures, 39 had complex partial seizures, and 5 children were unclassifiable. The syndrome of benign partial epilepsy of children with rolandic spikes (BPEC, 38 cases) was clearly identified and its uniformly benign final prognosis was confirmed even if some of these children had at times severe or poorly controlled seizures. Among the children with simple partial seizures outside the BPEC (25 cases) and complex partial seizures (39 cases), no homogeneous clinical or electroclinical subgroup could be found. Two children with benign partial epilepsy and myoclonic-astatic seizures ("atypical benign partial epilepsy of childhood") and one child with "benign epilepsy with occipital spike-waves" were identified. 74% of children with epilepsy with complex partial seizures (ECP) had a 1-year seizure-free interval, and many children with epilepsy with simple partial seizures outside the BPEC group (ESP) had no more than two seizures. A benign course is thus not limited to the BPEC but is difficult to predict. Prospective studies are necessary to confirm the existence of well-defined benign syndromes among the idiopathic partial epilepsies of childhood, which appear quite rare outside the BPEC.     

Nocturnal Paroxysmal Dystonia with Short-Lasting Attacks: Three Cases with Evidence for an Epileptic Frontal Lobe Origin of Seizures

The epileptic or nonepileptic origin of nocturnal paroxysmal dystonia (NPD) has been debated. We studied three patients with frequent attacks during non-REM sleep. During prolonged video-EEG monitoring, two patients had a convulsive seizure after a typical NPD episode and on these occasions EEG showed epileptiform discharge. In the three patients, attacks occurred repeatedly with different intensity, representing "fragments" of the same seizure. These fragments of the attack could occur periodically every 20-40 s. We postulate that short NPD attacks are actually epileptic seizures originating from the frontal lobes. The rhythmicity of the episodes may be due to rhythmic oscillation of cortical function during non-REM sleep.     

Paroxysmal dystonia and paroxysmal tremor in a young patient with multiple sclerosis

A 16-year-old patient with multiple sclerosis (MS) showed paroxysmal movement disorders during a recurrence of the disease. The paroxysms took the form ot brief unilateral dystonic posturings of the right body suggestive of paroxysmal dystonia (PD); they completely receded with acetazolamide. A single episode of a high amplitude, rythmic slow and coarse generalized tremor, present at rest and increasing with movement, particularly involving the head in a no-no movement, occurred soon after recovery from PD and lasted three hours. The present report provides evidence that MS has to be considered in the diagnostic approach to symptomatic childhood PD and underlines the efficacy of acetazolamide in the treatment of PD attacks. It also describes a rare paroxysmal movement disorder, defined as paroxysmal dystonic tremor, that can be considered as falling within the spectrum of PD.This study was partially supported by the Paolo Zorzi Association for Neurosciences, and the Harry De Jur Foundation.     

The Epley maneuver for the treatment of benign paroxysmal positional vertigo

In recent years, different forms of physical therapy have been proposed for the treatment of benign paroxysmal positional vertigo (BPPV). These mainly consist of maneuvers aiming to reposition and disperse free-floating endolymph particles in the posterior semicircular canal. We report our experience with one of these procedures, the Epley maneuver (EM), in treating 30 cases of BPPV. Twenty-six patients (87%) were cured, while four (13%) did not respond to treatment. These results are similar to those reported in the literature for the EM and the Sémont maneuver.     

Benign intracranial hypertension vs. intracranial arteriovenous malformation: A possible CT dilemma

Summary A nine-year-old boy, presenting only with signs of increased ICP, underwent computerized tomography. This examination demonstrated no abnormalities in the precontrast scan. Following C. E., it showed scattered areas of blood-like density in both hemispheres, as well as a presumedly abnormal vessel in the left occipital region and dilated vein of Galen, sinus rectus, and tentorial veins. The presumptive diagnosis of left occipital AVM was not confirmed by angiography, which also ruled out obstructions of the intracranial sinuses.The possible mechanism responsible for this atypical CT picture is briefly discussed in the light of pertinent literature. It is suggested that careful consideration should be given to the indications for angiography in similar cases, in the presence of a hypervascular aspect of the postcontrast CT scan, particularly if a considerable amount of contrast medium has been used.     

Cytologic diagnosis of cavernous hemangioma of the liver with fine-needle biopsy.

We present the cytopathologic findings in seven cases of cavernous hemangiomas of the liver diagnosed by direct "squash" smears made on tissue obtained through image-guided fine-needle biopsy. The diagnosis in each case was confirmed histologically. Utilizing this simple cytologic technique, the morphologic findings in these common hepatic lesions are as accurate and diagnostic as histologic examination.     

Creatine kinase isoenzyme (CK-BB) in combination to prostatic acid phosphatase measured by RIA in the diagnosis of prostatic cancer

Summary Creatine kinase isoenzyme (CK-BB) measured by mass was used to determine its value in the early diagnosis of prostatic cancer. Sera of patients with prostatic carcinoma of various stages (treated and untreated) were compared to normal male sera and sera of patients with benign hyperplasia of the prostate (BPH) with respect to CK-BB. The sera were simultaneously tested for PAP content. The sensitivity of the CK-BB-RIA was 1.63+/-0.08 g/l and reproducibility in the higher and lower concentration range 7.6% and 10.5%, respectively. CK-BB alone or in combination with PAP is no marker for early detection of prostatic cancer. In individual cases changes occurred similar to those found with a malignant growth of the prostate.     

Becker's X-linked muscular dystrophy histological,enzyme-histochemical,and ultrastructural studies of two cases,originally reported by Becker

Summary Muscle biopsies of two patients originally reported in the Göttingen family by Becker (1962) that formed the basis of separating a benign X-linked muscular dystrophy from the rapidly progressive Duchenne-type X-linked muscular dystrophy, revealed mild pathological changes in the younger patient and more advanced in the older one, consisting of increased spectra of fiber diameters, endomysial fibrosis, angulated fibers, pyknotic nuclear clumps and small groups of atrophic fibers. Essentially, both biopsies showed the same changes, but of different severity, possibly due to the differences in age and muscle biopsy sites. These changes were regarded myopathic, but a neurogenic component was suggested. Our observations accord well with those of a larger series (Bradley et al., 1978) where both electromyography and histopathology revealed a mixed myopathic-neurogenic pattern in patients with Becker-type dystrophy. Differential diagnostic aspects encompass Duchenne's muscular dystrophy, the other hereditary dystrophies and X-linked proximal spinal muscular atrophies. The precise nature of Becker-type muscular dystrophy requires morphological data on peripheral nerves, spinal roots and spinal cord anterior horn cells as well as sequential biopsy analysis to substantiate the primary site of pathology. However, on the basis of available data, it seems reasonable to suggest that the early changes of degeneration/regeneration which are accompanied by a markedly elevated CPK eventuate in the histopathologic and electromyographic patterns illustrated in these two patients with Beckertype dystrophy.These studies have in part been supported by a grant from The Volkswagen-Stiftung (HHG). Parts of this paper have been presented at the Neurochemical Group Symposium, Bath University, March 30, 1977, and at the Annual Meeting of the German Society of Neuropathology, Tübingen University, October 17–19, 1977     

An unusual case of rhythmic movement disorder

Rhythmic movement disorder is one of the sleep-wake transition disorders listed in the International Classification of Sleep Disorders. According to this classification, the condition commonly occurs in infants and toddlers, and persistence beyond 4 years of age is unusual. Recently, we encountered a case in which rhythmic movement disorder persisted up until the age of 12 years with spikes registering on the sleep electroencephalogram. Epileptic seizure was ruled out because of the characteristic rolling movement, absence of any other epileptic symptoms (e.g. vocalization and tonic-clonic seizure) and cessation as a result of removal of the blanket.     

Phonomicrosurgery for benign vocal Fold lesions - our experience

Benign lesions of glottis creates stiffness of the vocal fold and inefficient glottal valve, aerodynamically effecting the vocal quality by preventing smooth vocal edge closure. Introduction of phonomicrosurgical techniques based on Hirano ’s principle of vocal fold epithelium have revolutionized results of voice surgery. Our experience in the management of benign vocal fold lesions by phonomicrosurgical techniques with pre and post operative stroboscopic, perceptual and computerized acoustic voice analysis is described.