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Biomedical subjects

A L Ritaccio

Publications and source records attributed to A L Ritaccio.

5 recordsLinked to original sources

Reflex seizures.

Reflex seizures are best defined as a collective of different seizure types distinguished by their ability to be precipitated by a specific stimulus. This article reviews established reflex syndromes, including data gleaned from the application of intensive monitoring. Animal models are also discussed. The majority of stimulus-sensitive seizures occur in the context of well established, generalized, or localization-related epilepsy syndromes. The study of these phenomena offers insight into sensory and cognitive processing.

Brain

Characterization of intractable juvenile myoclonic epilepsy: new perspectives on primarily generalized seizures.

Twelve patients were identified at an epilepsy center who had medically intractable juvenile myoclonic epilepsy. Significant characterization of this group included the long duration of their epilepsy (averaging 21 years) during which the diagnosis and appropriate treatment was delayed. A high percentage of these patients had asymmetries or focal discharges on scalp EEG (6 of 9 patients). A review of the literature and the findings in these 12 patients lead to the conclusion that juvenile myoclonic epilepsy is not necessarily a benign epilepsy. Alternative therapies, such as epilepsy surgery, may be indicated in such extreme cases.

Adult

The role of dominant premotor cortex and grapheme to phoneme transformation in reading epilepsy. A neuroanatomic, neurophysiologic, and neuropsychological study.

We studied a 24-year-old man who had reading epilepsy after removal of a left frontal arteriovenous malformation. Lesion analysis by means of a neuroanatomic template placed a 2-cm region of encephalomalacia anterior to the left central sulcus in premotor cortex (Brodmann's area 6). Lexical and nonlexical reading activation tests demonstrated seizures during reading and increased discharge rates when the patient was reading aloud or silently articulating. Seizures (perceived or actual jaw clicking) were electrographically characterized by brief left frontocentral epileptiform transients. Grapheme to phoneme transformation, not linguistic complexity, appears to be the critical stimulus in some reading epilepsies. The case adds anatomic relevance to the phonologic component of reading and supports the putative role of dominant premotor cortex in activation of precise sequences of motor linguistic output in reading and writing. Reading epilepsy may be a reflex or action myoclonus syndrome localized to Brodmann's area 6 (Exner's area).

Adult

Myasthenia gravis with thymoma and pure red blood cell aplasia.

A case of myasthenia gravis with histopathologic confirmation of spindle cell thymoma and pure red blood cell aplasia is reported. This is the twelfth case in the literature in which a simultaneous occurrence of all three disorders, with documented thymic pathology, is noted. Immunologic observations in this patient include an elevated acetylcholine receptor antibody and antinuclear antibody titer, agglutination of mouse red blood cells when combined with the patient's serum, and lack of inhibition of binding of radioactive erythropoietin to mouse red cell receptors when combined with the patient's serum. Although both myasthenia with thymoma and pure red blood cell aplasia may have a common immunologic denominator, our findings in this case indicate that inhibition of erythropoiesis is unrelated to erythropoietin receptor blockade. An alternative hypothesis is offered based on defective T-cell function.

Aged

Benign monoclonal IgAK gammopathy associated with polyneuropathy and dysautonomia.

The first case of benign IgAK monoclonal gammopathy associated with peripheral neuropathy is described. Dysautonomia is an unusual, yet prominent, manifestation of neuropathy in this patient. Electrodiagnostic testing and nerve biopsy were compatible with demyelination and axonal loss. Myelin sheath, perineural, and endoneural interstitial tissue fixation of anti-IgA and anti-kappa light chains was demonstrated by direct immunofluorescence microscopy. Absorption studies utilizing human peripheral nerve myelin resulted in complete removal of the paraprotein band. Analytic procedures with myelin-associated glycoprotein and gangliosides, however, were negative. Based on these findings, an alternative etiology for this neuropathy is hypothesized.

Aged