Creutzfeldt-Jakob disease cluster in the health area of Meixoeiro Hospital.

OBJECTIVE: Galicia is the Spanish region in which most bovine spongiform encephalopathy cases have been registered. Meixoeiro Hospital is included in the Galician Health Service (SERGAS). The aim of the study was to analyze the clinical and epidemiological characteristics of Creutzfeldt-Jakob disease (CJD) in the health area of Meixoeiro Hospital and to identify possible specific risk factors to the general public. METHODS: All incident cases of CJD were identified in the health area of Meixoeiro Hospital (187,877 inhabitants) over a 14-year period, 1997-2010, and classified according to WHO diagnostic criteria. We obtained clinical detail and epidemiological information on all cases. Crude and age-specific incidence rates were calculated. A review of surgical or invasive medical procedures was undertaken. RESULTS: We diagnosed 12 patients with CJD, 10 sporadic CJD (sCJD), and two genetic CJD (gCJD). No iatrogenic or variant CJD was detected. According to Poisson distribution, 3.9 CJD cases would be expected for our area over the 14 years researched. The average yearly mortality rate from CJD was 4.6 cases per million (3.8 from sCJD and 0.8 from gCJD). Eight patients (67%) underwent at least one surgical or invasive medical procedure. Sixteen of twenty-seven (59%) of these procedures were undertaken in Meixoeiro Hospital. CONCLUSIONS: The incidence of CJD in the health area of Meixoeiro Hospital is three times higher than expected. The hypothesis that at least some cases of sCJD are apparently because of covert transmission or zoonosis events should not be formally refuted and might explain the high rate found.

[Muscular biopsy in ceroid lipofuscinosis: 3 case reports of juvenile form]. / Biopsia muscular en la ceroidolipofuscinosis: estudio de tres casos de la forma juvenil.

Ceroid lipofuscinosis (CLF) is a progressive hereditary neurodegenerative disease characterised by deposits in the central nervous system and other tissues of ceroid lipopigment. Symptomatology and clinical course of the disease are heterogeneous and up to ten types have been distinguished, although the most frequent and best known are the Santavuori-Haltia infantile form, the Jansky-Bielschowsky late infantile form, the Spielmeyer-Vogt juvenile form and the Kufs adult form. We present here three cases diagnosed as having juvenile ceroid lipofuscinosis by means of muscular biopsy. Although morphological and ultrastructural findings in CLF have already been described in literature, the use of muscular biopsy as a means of diagnosis is not usual and its usefulness is not very well known. For this reason, we especially recommend this method for its simplicity and diagnostic specificity.

[Normotensive hydrocephalus as a manifestation of meningovascular syphilis]. / Hidrocefalia normotensiva como manifestación de sífilis meningovascular.

Progressive general paralysis, tabes dorsalis and meningovascular syphilis are different manifestations of tertiary syphilis. The clinical picture of meningovascular syphilis is of a subacute or chronic meningeal syndrome. It may be associated with focal neurological signs of cerebral arteritis. Inflammation of the leptomeninges may impede circulation of the cerebrovascular fluid at different levels, giving rise to noncommunicating, or exceptionally to communicating hydrocephalus. Diagnosis of this depends on the clinical signs and neuro-imaging changes. We present the case of a man with meningovascular syphilis who developed clinical signs of normotensive hydrocephalus.

[Cephalic fibromuscular dysplasia and stroke in infancy: a case report] and review of literature]. / Displasia fibromuscular cefálica e ictus en la infancia: caso clínico y revisión de la literatura.

Fibromuscular dysplasia (DFM) is an arterial lesion of unknown aetiology which affects medium sized arteries and is multifocal. In the cephalic form extracranial arteries may also be affected; the usual clinical finding is an ischaemic stroke related to arterial stenosis or obstruction, or to arterio-arterial thrombo-embolism. Diagnosis of DFM in infancy is exceptional, and unlike the adult forms, affectation of the intracranial arteries is usual. Angiography of the cranial vessels is the most important diagnostic investigation, showing focal lesions with circular stenosis alternating with dilatations of the arterial wall and described as ¿a pile of coins'; or there may be stenosis and obstruction of extracranial and intracranial arteries. We describe the case of an eleven-year-old girl who presented with a motor deficit of sudden onset in relation to a parietal infarct, and in whom angiography of the supra-aortic and intra-cranial trunks showed evidence compatible with DFM of the extracranial internal carotid artery and carotid syphon. We also review the literature of all cases of infantile DFM published to date.

[Cervical pain as a clinical sign of spontaneous intracraneal hypotension]. / Cervicalgia como manifestación clínica de un síndrome de hipotensión intracraneal espontánea.

We report a patient with spontaneous intracranial hypotension who developed severe neck pain after hard exercise. The pain was worse when the patient was standing and was relieved when he lay flat. Radionuclide cisternography demonstrated a central spinal fluid leak in the thoracic region of the spine. The syndrome resolved with conservative treatment. Although the most typical feature of spontaneous intracranial hypotension is postural headache, unnecessary testing can be avoided if we suspect this entity in the presence of cervical pain that worsens when the patient is upright and disappears or improves when he or she is lying down.