Abstract: A 38-year-old HIV-1 infected woman affected with bilateral tonic pupils. Ophthalmologic examination confirmed Holmes-Adie syndrome (HAS), and peripheral distal polyneuropathy, orthostatic hypotension and leg hyperhidrosis were detected on further workup. The HAS can be either idiopathic or associated with neuropathy of various etiology (autoimmune, paraneoplastic and infectious). In our patient, the pupillotonia was the first and early symptom of hitherto unrecognized HIV neuropathy. HAS has been previously observed in association with syphilis, Lyme borreliosis, herpes simplex and parvovirus B19 infection. Our case is the first report of HAS in a case of HIV infection.
Abstract: Selective posterior rhizotomy (SPR) represents a standard neurosurgical approach in the treatment of spasticity in children with cerebral palsy (CP). Beside the reduction of spasticity in lower limbs, SPR may have suprasegmental effects, considerably above the surgery site. In this communication, we report on the improvement of smooth pursuit eye movements (SPEM) in two children after SPR.
Abstract: Paraneoplastic syndromes related to soft tissue tumors are very uncommon and an association of paraneoplastic diseases with a malignant fibrous histiocytoma (MFH) has not been reported so far. Opsoclonus-myoclonus is a rare paraneoplastic nervous system syndrome, that was well documented in adult patients with neoplasms particularly of the lung and breast. A 77-year-old woman developed typical opsoclonus-myoclonus syndrome and rapidly progressed to coma. The opsoclonus and generalized myoclonus continued until the patient's death seven months later. An autopsy examination identified a pleomorphic MFH in the retroperitoneum without a metastatic spread. Microscopic examination of the cerebellum revealed an atrophy of the granular layer along with a marked patchy loss of Purkinje cells, and a loss of neurons in the cerebellar nuclei accompanied by distinct peridental demyelination and astrogliosis. A moderate loss of neurons and neuronal chromatolysis were observed also in the inferior olivary nuclei. However, the omnipause neurons of the nucleus raphe interpositus, that are supposed to be responsible for opsoclonus generation under pathological conditions, as well as ocular brain stem nuclei were all intact. We failed to prove the presence of known antineuronal anti-Yo, anti-Hu or anti-Ri autoantibodies in both serum and cerebrospinal fluid (CSF) of the patient. However, the confirmation of the intrathecal IgG synthesis by oligoclonal bands in CSF and the immunohistochemical detection of IgG deposits on membranes of Purkinje cells, neurons of cerebellar granular layer and in cerebellar nuclei in our case support the presumed autoimmune nature of the disease.
Abstract: The occurrence of spasticity is most commonly attributed to the lack of presynaptic inhibition. Perinatal damage to the central nervous system, as it happens in cerebral palsy, leads to pathological reflex response both on segmental and polysegmental levels. It results not only in clinical signs typical for spasticity but also in alterations of brainstem function, such as dysarthria or congenital nystagmus. Selective posterior rhizotomy is a neurosurgical method, routinely used in the treatment of spasticity. The lumbosacral posterior roots are partially cut under perioperative neurophysiological control. The aim of the treatment is the reduction of afferentation for posterior horns resulting in a decrease of pathological reflex responses. Selective posterior rhizotomy consequently decreases lower limbs spasticity. The improvement of upper extremities fine skills, the improvement of speech and cognitive functions has been also observed after selective posterior rhizotomy. The possible pathophysiological explanations of these so-called suprasegmental effects are discussed in the article.
Abstract: At present 19 antiretroviral drugs are available, which offer together 969 possible triple-combinations. Only a few combinations, mainly of nucleoside inhibitors of reverse transcriptase, are contraindicated due to undesirable interactions, which may negatively influence the treatment efficacy or increase toxicity. With raising number of antiretroviral drugs there must also be paid more attention to possible interactions of these drugs.
Abstract: Acute HIV infection occurs in 85% of people recently infected with HIV-1 after an incubation period of 2-6 weeks. Diagnosing the acute HIV infection is difficult because the symptoms are non-characteristic and include fever, pharyngitis, and skin rash. Early recognition of HIV infection is, of course, maximally desirable in order to prevent further transmission of the infection and because the early treatment can slow down later progression of the disease.
Abstract: Baclofen affects in experiments the reactivity of the vestibuloocular reflex by influencing the time constant of the vestibular nystagmus. The authors compare in three healthy volunteers the reactivity of the vestibular apparatus after administration of Baclofen and a new muscle-relaxant drug tizanidine. The authors evaluate basic parameters of the vestibular and optokinetic nystagmus (gain and time constant), discuss the problem of GABAergic (Baclofen) and monoaminergic (tizanide) receptors kin the area of the vestibular apparatus. As regards the effect on the reactivity of the vestibular system, tizanidine does not affect immediately the response of the vestibuloocular reflex but interferes with processes ensured by the central neuronal integrator.
Abstract: Vestibulological examination of boxers revealed that 80% had vestibular abnormalities. These were obvious, but well compensated, so that they remained hidden in everyday life and appeared only on laboratory examination or under stress. Regarding the origin of vestibulo-ocular reflex, abnormalities in boxing contribute three etiopathogenetic factors: the first is the direct traumatization of the vestibular apparatus by a direct blow. The second is the diffuse damage of the brain-stem white matter and of vestibular nuclei. The third factor is the traumatization of the cervical spine via the traumatization of the proprioceptive systems, cervical sympathetic and the adjacent vascular mechanisms. One-third of the abnormalities are caused by direct labyrinthine traumatization. The findings of simultaneous EEG, OKN and ETT abnormalities in one-half of our subjects suggests that there are probably also supratentorial lesions caused by the above-mentioned mechanisms.
