[Horner syndrome as a manifestation of carotid artery dissection]. / Síndrome de Horner como manifestación de disección carotídea.

CLINICAL CASE: A 42-year-old man presented with ptosis and miosis in his left eye and a history of headache over the last 20 days. An angioresonance showed dissection of internal carotid artery. DISCUSSION: "Painful Horner's Syndrome" is considered to be a medical emergency due possible onset of an internal carotid artery dissection. We consider that awareness of neuro-ophthalmologic emergencies is very important in the clinical praxis of an ophthalmologist. Multidisciplinary treatment and follow-up of these patients is required.

[Compromise of the cognitive functions in progressive secondary multiple sclerosis]. / Afectación de las funciones cognitivas en la esclerosis múltiple secundaria progresiva.

AIM: To define the patterns of cognitive impairment in a homogeneous group of secondary progressive multiple sclerosis (SPMS) patients. PATIENTS AND METHODS: Forty-two SPMS patients were included with a similar degree of disability; all had been treated with interferon beta-1b for a minimum of 3 months. They voluntarily complimented a battery of 10 neuropsychological tests selected for this study, distributed in two sessions of one hour. In addition, the emotional state was evaluated with the Beck Depression Inventory and the Hamilton Anxiety Scale. We considered cognitive impairment as more than two tests altered, according with previously reported studies. RESULTS: 73.8% of patients were women; mean age was 45 years (range: 25-62); mean EDSS was 5.4 (range: 3.0-7.5); mean evolution time was 34.5 months (range: 24-80); mean treatment duration was 13.5 months (range: 3-38). Cognitive impairment was present in 78.5% of patients. The most frequently impaired functions were: attentional capacity, visuospatial perception, verbal fluency, short-term and long-term logic memory and abstract reasoning. The presence of cognitive impairment was related to the time of evolution of the disease (r = 0.31; p < 0.05) but not with the age, the degree of disability or the treatment duration. CONCLUSION: Cognitive impairment in the SPMS patients is a frequent finding, being the alteration in the speed for the acquisition and processing of new information, and the abstract reasoning the most frequent and severe altered functions. The also frequent impairment of visuospatial information was a differential finding in our study that could contribute to diagnosis of clinical progression.

[Total brain T2-hyperintense lesion-volume and the axonal damage in the normal-appearing white matter of brainstem in early lapsing-remitting multiple sclerosis]. / Relación entre el volumen lesional cerebral total en resonancia magnética secuencia T2 y el daño neuroaxonal en la sustancia blanca aparentemente normal del tronco del encéfalo en la esclerosis múltiple remitente-recurrente.

AIM: To evaluate the relationship between the total brain T2-hyperintense lesion volume (TBT2LV) and the axonal damage in the normal-appearing white matter of brainstem measured by 1H-MRS in a group of early relapsing-remitting multiple sclerosis patients. SUBJECTS AND METHODS: 40 relapsing-remitting multiple sclerosis patients and ten sex- and age-matched healthy subjects were prospectively studied for two years. T2-weighted MR and 1H-MRS imaging were acquired at time of recruitment and at year two. The TBT2LV was calculated with a semiautomatic program; N-acetylaspartate (NAA), creatine (Cr) and choline (Cho) resonances areas were integrated with jMRUI program and the ratios were calculated for four volume elements that represented the brainstem. RESULTS: At basal study we obtained an axonal loss (as a decrement of NAA/ Cho ratio) in the group of patients compared with controls (p = 0.017); this axonal loss increased at the second year of the follow-up for patients (NAA/Cho decrease, p = 0.004, and NAA/Cr decrease, p = 0.002) meanwhile control subjects had no significant metabolic changes. Higher lesion load was correlated with a poor clinical outcome, being the correlation between the basal TBT2LV and the Expanded Disability Status Scale at second year (r = 0.299; p = 0.05). Besides, axonal loss was not homogeneous for all multiple sclerosis patients, being stronger in the subgroup of patients with high basal TBT2LV (p = 0.043; ANOVA). CONCLUSION: Our data suggest that axonal damage is early in multiple sclerosis and higher in patients high basal TBT2LV, suggesting a possible relationship between these two phenomena.

Evolución clinicorradiológica atípica en un caso de variante panencefálica de la enfermedad de Creutzfeldt-Jakob / Atypical clinicoradiological course in a case panancephalic variant of Creutzfeldt-Jakob

Introducción. La enfermedad de Creutzfeldt-Jakob (ECJ) es la encefalopatía espongiforme transmisible más frecuente; su fisiopatogenia aún no se conoce con exactitud. La forma esporádica es la más habitual caracterizada por la tríada: demencia rápidamente progresiva, mioclonías y ataxia. La ECJ tipo panencefálico, descrita excepcionalmente fuera de Japón, se caracteriza por la afectación difusa de la sustancia gris y blanca. El interés internacional por esta enfermedad surge hace más de una década con el descubrimiento de la nueva variante. Desde entonces los avances en resonancia magnética (RM) han contribuido al diagnóstico pre mortem de la enfermedad con la descripción de signos específicos. Caso clínico. Presentamos un caso de ECJ tipo panencefálico en nuestro medio con una evolución clinicorradiológica atípica. Discusión. Nuestra paciente comenzó con síntomas visuales y psiquiátricos. La RM cerebral mostró lesiones inespecíficas de sustancia blanca en regiones parietal posterior y occipital bilateral que se resolvieron tras tratamiento esteroideo. El hallazgo radiológico más característico en las formas esporádicas es la hiperintensidad bilateral y simétrica de los ganglios de la base, en la nueva variante del núcleo pulvinar y en la variante panencefálica, la presencia de lesiones de sustancia blanca de distribución periventricular con tendencia a la extensión local a medida que la enfermedad progresa. En los sucesivos estudios de RM de nuestra paciente pudimos ver cómo los signos característicos iban apareciendo; sin embargo, la resolución completa de las lesiones iniciales nos permite plantear la posible implicación de mecanismos inflamatorios en algún momento de la evolución de las lesiones de sustancia blanca (AU)

Introduction: Creutzfeldt-Jakob disease (CJD) is the most frequent of the human transmissible spongiform encephalopathies. Pathogenic mechanisms of CJD are still unknown. Sporadic CJD, the most habitual, is clinically characterized by rapidly progressive dementia, myoclonia and ataxia. Panencephalic variant CJD, typically from Japan, is characterized by extensive involvement of the cerebral white and gray matter. International interest has grown from more than one decade ago in relation to the diagnosis of new variant (vCJD). New protocols of MR imaging have contributed to the early diagnosis of CJD with specific signs. Case report: We report a case of panencephalic CJD, with atypical clinical presentation and unusual MR imaging findings. Discussion: Our patient developed visual and psychiatric symptoms. Brain MR imaging showed extensive white matter lesions in posterior parietal lobe and occipital regions, which disappeared after steroid treatment. The most characteristic radiological sign for sporadic CJD is the high signal intensity of the basal ganglia, for vCJD the pulvinar sign and, for panencephalic CJD the presence of periventricular white matter lesions, with tendency to the spread when the disease progress. In serial MR imaging studies of our patient, we could see how typical signs were appearing. However, the complete and unusual resolution of the original white matter lesions makes us to think about a possible inflammatory component, in some time in the evolution of white matter damage (AU)

[Subacute uveomeningoencephalitis as the presenting symptom of acute infection by the human immunodeficiency virus]. / Uveomeningoencefalitis subaguda como forma de presentación de la infección aguda por el virus de la inmunodeficiencia humana.

INTRODUCTION: The uveo-meningitic syndrome, or the combination of chronic or recurrent meningitis and acute uveitis, has a specific differential diagnosis. This syndrome can be the clinical debut of systemic disorders, vasculophathies, connective tissue disorders and inmuno-mediated diseases. In patients with AIDS, the syndrome often appears in relation with an opportunist concomitant infection of the central nervous system (CNS). CASE REPORT: We present one case of subacute uveo-meningitic syndrome as symptomatic presentation of a early infection of HIV. The patient was a man, 37 years-old. He was inmunocompetent and did not know his seropositivity for HIV type 1. We relate the results of the neurologic examination and complementary tests. Only serologic test for HIV type 1 and detection of IgG anti-HIV in cerebrospinal fluid were positives. CONCLUSIONS: In patients HIV-positive the ocular infection, usually a posterior uveitis, appears together with systemic disorders or central nervous infections. In other hand, the cause of meningitic infection depends on grade of immunocompromise. Aseptic meningitis, for early stages of the disease, is usually no symptomatic. After, opportunist infections or neoplasic infiltration of CNS can be cause of meningoencephalitis. In this patient, the early infection of HIV causes an subacute uveomeningoencephalitis. Early infection of HIV increases the possibilities of aetiological diagnosis of uveomeningitic syndrome.

[Posttraumatic headache. Pathophysiology, clinical, diagnostic and therapeutic aspects]. / La cefalea postraumática. Fisiopatología, aspectos clínicos, diagnósticos y terapéuticos.

Posttraumatic headache (PTHA) is, usually, one of several symptoms of the posttraumatic syndrome and therefore may be accompanied by somatic, psychological or cognitive disturbances. The aetiology of these symptoms in individuals with mild traumatic brain injury or whiplash injury has been a subject of some controversy with explanations ranging from neural damage to malingering. PTHA can resemble a tension-type headache, migrainous or cervicogenic headaches. Post-whiplash headache habitually is a pain radiating from the neck to the forehead, with moderate intensity and benign, but prolonged course. The pathogenesis of PTHA is still not well-known but might share some common headache pathways with primary headaches. In this chapter, we review recent investigations in the pathophysiology of PTHA, review recognised risk factors for a poor outcome and give some recommendations of management. We also discuss the new diagnostic criteria of IHS Classification, 2004, for PTHA and Headache attributed to whiplash.

Placebo response in a patient with chronic migraine and ergotic overuse.

Facilitation of nociceptive systems has been implicated in Chronic-Migraine pathogenesis. Daily consumption of medication may be a contributing factor. The patient was male, aged 76 years, with history of migraine without aura. Six years ago, he presented with a mild-moderate daily pulsating headache. He was overusing analgesics and ergotamine. After withdrawing, the patient started a nonpulsating headache, diffuse and constant. During follow up, he was refractive to several symptomatic and prophylactic treatments. When we treated him with placebo capsules, the headache responded very well. At first, pain-relief occurred for 6 h and progressively, extended. Two years later, when our patient started to use transdermal patches of fentanyl for treatment of a complex regional pain syndrome type 1, his headache did not improve. Patient has maintained prolonged response to placebo. Actually, he is pain-free for 2-3 days with 1 placebo capsule. We discuss mechanisms of chronic-migraine, drug-overuse, drug-induced headache and placebo addiction. Powerful psychological mechanisms could determine response to placebo in this patient.

[Pseudopolyneuritic symptoms with a medullary origin as the presenting symptom of multiple sclerosis]. / Patrón pseudopolineurítico de origen medular como forma de presentación de una esclerosis múltiple.

INTRODUCTION: Myelopathy is, frequently, the first manifestation of a multiple sclerosis (MS), being the acute transverse mielitis, partial or incomplete, the habitual clinical syndrome. Other sensitive symptoms with an origin in the spinal cord as 'steroanesthesia', 'pseudo-athetosis' or 'pseudo-radiculopathy' have been described as unusual form of presentation in MS, but not the 'pseudo-polyneuropathy', perhaps in relation with its rapid progression in an acute transverse mielitis or other sensory pattern. CASE REPORTS: We presented two patients with a 'pseudo-polyneuropathy' pattern as the first manifestation of MS. In the two cases magnetic resonance imaging showed a cervical lesion of spinal cord with a probably inflammatory origin and other similar lesions in the brain. Neurophysiological studies were normal. We think that the 'pseudo-polyneuropathy' could be caused by an antero-lateral demyelinating process in the cervical spinal cord, with affectation of the anterior comissure and the ventral portion of lateral spinothalamic pathway. CONCLUSION: The 'pseudo-polyneuropathic' syndrome is uncommon form of presentation of MS in young people. We may be capable of recognize the 'pseudo-polyneuropathy' with spinal origin and early choose the appropriate complementary examinations for a correct diagnosis and therapy.

[Efficacy and safety of endovascular treatment of brain aneurysms: experience in our setting]. / Eficacia y seguridad del tratamiento endovascular de aneurismas cerebrales: experiencia en nuestro medio.

INTRODUCTION: The objective of our study is to know the efficacy and safety of endovascular treatment in our setting. PATIENTS AND METHODS: a) prospective evaluation of patients with aneurysmal subarachnoid hemorrhage (SAH) or incidental aneurysm (Dec 1999-Dec 2002), and b) neurological, angiographic and evolutive evaluation of the endovascularly operated patients. RESULTS: A total of 79 patients were evaluated: 75 with SAH, 4 with incidental aneurysm. Arteriographies were performed in 74 (93.6%), detecting 58 aneurysms in 52 patients, 15 of the anterior communicating artery (25%) and 14 of the posterior communicating artery (24%). Fifty-two aneurysms were treated with Guglielmi Detachable Coiling (GDC) (89.6 %). Total occlusion (100 %) was obtained in 48 (82.7%) and partial occlusion in 4. Technique failure occurred in six cases. The earlier complications, after endovascular procedure (< 24 h), were one rebleeding, four angiographic spasms and ischemia and one coil displacement. During the first month, there were other cases of rebleeding and two cases of spasm and ischemia. Morbidity rate was 11.5 %. Death rate was 5.7 %. Follow-up period ranges between 6 and 42 months. After endovascular treatment 40 patients had a favourable outcome (Ranking 1-2) and 9 suffered moderate-severe incapacity (Ranking 3-5). We prescribed angiographic controls one year after the hemorrhagic event and there was only one case of aneurysmal recurrence. DISCUSSION: In our experience endovascular treatment is useful to treat ruptured and unruptured aneurysms, with similar surgical occlusion, morbidity and death rates.

Cefalea por abuso de triptanes / Triptans overuse headache

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