Clinical Neuroscience

[Common variable immunodeficiency with coexisting central nervous system sarcoidosis. Case report and literature review with implications for diagnosis and pathogenesis]

MAGDALENA Dziadzio, HORTOBÁGYI Tibor, DESMOND Kidd, RONNIE Chee

NOVEMBER 30, 2011

Clinical Neuroscience - 2011;64(11-12)

[We describe a patient with a history of longstanding primary generalised epilepsy, on anticonvulsant therapy, who presented with fever, headache, worsening seizures and hallucinations. Among various investigations, the patient had high CSF protein and ACE levels, leptomeningeal nodular enhancement on MRI brain and non-caseating granulomas in the brain and meninges on the biopsy. The patient was diagnosed with neurosarcoidosis. Subsequently, he was found to be panhypogammaglobulinaemic and was diagnosed with probable common variable immunodeficiency (CVID). The coexistence of common variable immunodeficiency and neurosarcoidosis is rare. Typically, non-caseating granulomas in CVID patients are localised in the lymphatic tissue and solid organs. To our knowledge, there are only five reports of the granulomas of the central nervous system (CNS) in CVID. We discuss the diagnostic difficulties in this case and review the literature.]

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[Based on our previous survey on the capacities of neurological services and on the predictable changes in the neurologist workforce in Hungary, we present a proposal for the organization of the structure of neurological services in the future. We discuss the diagnostic groups treated by neurologists, the neurological services and their progressive organization. Using the current capacities as baseline, we propose patient groups to be treated by neurologists in the future, and the levels of services. Based on the tendencies seen in the last years we suggest to consider to allocate acute stroke services exclusively to stroke units in neurological departments, and we identify a few other diagnostic groups where neurology should have a larger share in patient care. We define three levels for inpatient care: university departments, regional/county hospitals, city hospitals. Instead of minimum criteria we assign outpatient and inpatient standards that are functional from the economic point of view as well. University departments cover all areas of neurological services, have a function in graduate and postgraduate training, and on a regional basis they participate in professional quality assurance activities at the county and city hospital levels, and would have a more independent role in residency training. As far as patient care is concerned, the task of the regional/county hospitals would be similar to that of university departments - without the exclusively university functions. A general neurological service would be offered at the city hospital level - the representation of all subspecialties of neurology is not required. Neurorehabilitation would be organized at special units of neurological wards at the city hospital level, at independent neurorehabilitation wards in regional/county hospitals, and also as an outpatient service offered at the patients’ home. The most significant organizational change would affect the outpatient neurological services. In addition to the special outpatient units associated with university departments and regional/county hospitals, the general neurological outpatient services would be organized as private practices, similarly to the current system of general practitioners, where the individual practices contract independently with the health insurance fund. Their task would be a general neurological service offered 30 hours per week, and also basic, screening neurophysiological and neurosonological examinations, with proper equipment and trained assistance. A transformation in residency training and a change in financing is needed for this plan to fulfill.]

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[Background and purpose - We analyzed the statistical characteristics of subarachnoid hemorrhage (SAH) in Hungary in 2009. Methods - Using data supplied by the hospitals about their inpatient services to the National Health Insurance Fund with ICD-10 code I60. Results - 1403 SAH hospital cases were recorded in 1028 patients. That is much more than we expected from previous data. 63.6% were women, hospital case fatality was 12.2%. The average hospital stay was 6.47 days. 763 CT examinations were done (74.2% of the patients). Hypertension was recorded in 61.3% of the patients. The incidence was increasing with age till the age group of 51- 60 years, and decreased beyond that. In 531 patients the source of bleeding could be verified. Aneurysm of the anterior communicating artery was more frequent in men, aneurysm of the middle cerebral artery (MCA) and internal carotid artery in women. In total MCA aneurysm was the most frequent. Arteriovenous malformation was present in 7.6% of the patients. SAH was most frequent in January and February, rarest in April and August. Conclusion - SAH is more frequent in Hungary than previously thought.]

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