[Vertigo as an atypical symptom of intraspinal cord tumor]. / Vértigo como síntoma atípico de un tumor intramedular.

Vertigo is an unpleasant sensation of movement of the subject or of his surroundings. There are many causes of vertigo. Traditionally these are divided into 'central' and 'peripheral'. It is unusual to find it as a symptom of myelopathy. We describe the case of a 67 year-old woman with no significant previous clinical history who complained of the sudden onset of vertigo a few hours previously, which worsened on turning her neck towards the left. On examination, the vertigo recurred on turning her neck to the left after a latent period of a few seconds together with the appearance of ipsilateral conjugated nystagmus, which gradually disappeared. On neurological examination, there was amyotrophy of both arms with weakness, considerably reduced reflexes, except for clonus of the left wrist. There was slight paraparesia with bilateral cutaneoplantar flexion and a level of sensitivity at C2-C3. The sensitivity disorder was more marked in the arms with a bilateral distribution approximately at C5-C7 affecting predominantly thermoanalgesia. Cervical MRI showed a mass consistent with ependymoma. This was removed surgically 2 weeks after admission. The findings of the intraoperative biopsy confirmed the radiological diagnosis. The vertigo improved one week after admission, although the patient died of pneumonia one month after operation. Despite the vertigo was an atypical symptom, it was the clue that led to the diagnosis. Hypotheses about the cause of vertigo are discussed.

[HTLV-I myelopathy: presentation of a new case]. / Mielopatía por HTLV-I: presentación de un nuevo caso.

INTRODUCTION: HTLV-I is a human retrovirus which has been implicated in the genesis of tropical spastic paraparesis (HTLV-I-associated myelopathy). So far five cases of this illness have been detected in Spain, five of them in immigrants. We present a new case in Spain, with a characteristic chronic clinical picture. CASE REPORT: A 36-year-old black woman native of Ecuatorial Guinea, developed along 10 years a progressive paraparesis of asymmetric onset with important back pain, that arrives to paraplegic spastic phase at the present time. She presents distal amyotrophies, ulcers of decubitus and loss of control of sphincters, with normal mental status. Laboratory tests: blood, biochemistry and microbiologic studies: normal, or negative. She presented positive Western Blot serology for HTLV-I, confirmed by means of PCR technique. Cranial MRI: small and hyperintense subcortical lesions on T2 weighted images; spinal MRI: local atrophy at high thoracic level. A lumbar puncture was performed, with no cells, and with presence of oligoclonal bands, and a high IgG index. Urodynamic study: neurogenic spastic bladder. EMG: mild axonal polyneuropathy with prevalence in legs. CONCLUSIONS: In the differential diagnosis of progressive paraperesis, and mainly with epidemic antecedents, it is necessary to include a determination of HTLV-I between the diagnostic tests.

[MELAS syndrome masquerading as herpes encephalitis: genetic diagnosis]. / Síndrome MELAS que asemeja una encefalitis por virus herpes: diagnóstico genético.

INTRODUCTION: MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke like episodes) is a mitochondrial disease related to the mitochondrial DNA mutation. The tRNALeu(UUR) mutation at the base pair 3234 is associated to 80% of cases of MELAS syndrome. The onset with the stroke like episodes is often before the age 40. Herpes simplex encephalitis (HSE) presents as acute episodes of focal neurologic deficit that are often related to the temporal lobe. Occasionally MELAS syndrome and HSE may have a similar clinical presentation. CLINICAL CASE: A 33-year-old woman presented with seizures and a right parietotemporal syndrome of acute onset. The differential diagnosis between HSE and MELAS syndrome was established. MELAS syndrome was suspected based on her phenotype. The diagnosis of MELAS syndrome was confirmed by the presence of the mitochondrial tRNALeu(UUR) mutation at the base pair 3243. CONCLUSION: The clinical presentation of the MELAS syndrome may mimic that of the HSE, and antiviral treatment should be given until the diagnosis of MELAS syndrome is definitive. The study of the mitochondrial DNA helps diagnosing in a non invasive way many patients with MELAS syndrome.

[Spinal epidural abscess in a patient with septicaemia]. / Absceso epidural vertebral en un paciente con septicemia.

INTRODUCTION: Spinal epidural abscess is a rare entity requiring early diagnosis and treatment. Sepsis is a factor with an unfavourable prognosis. CASE REPORT: We report the case of a 57 year old female with acute low back pain who was admitted to hospital suffering from a state of septic shock and multiple organ failure secondary to an infection disseminated by Staphylococcus aureus, which was treated early on with vancomycin. The probable source of infection was assumed to be necrotizing fasciitis of the left arm. Once the acute phase had been overcome, serious paraparesis became apparent and this led to magnetic resonance imaging of the spine being carried out, the results of which showed the existence of a lumbar spondylodiscitis with associated epidural abscess. CONCLUSION: In patients with sepsis and some previous symptom that arouses suspicion, it is important to consider this possible diagnosis, since treatment with antibiotics alone does not manage to prevent neurological complications in all cases.

[Metachromatic leukodystrophy: an exceptional cause of dementia in the adult]. / Leucodistrofia metacromática: una causa excepcional de demencia en el adulto.

Hereditary metabolic diseases are an exceptional cause of neurological disorders in adults. Metachromatic leukodystrophy is a hereditary alteration of the metabolism of myelin which may be manifested in adults as intellectual deterioration. A case of metachromatic leukodystrophy presented in adulthood is presented with cognitive deterioration and behavioral alterations as the only clinical manifestation. The patient was a 28 year old male studied for dementia of one year of evolution. Computerized tomography and cranial magnetic resonance demonstrated diffuse and symmetric involvement of the periventricular white matter. The visual evoked potentials were involved while the brain stem auditory potentials were normal. Study of the speed of nerve conductions was compatible with demyelinating neuropathy. The diagnosis of metachromatic leukodystrophy was confirmed by enzyme study revealing very diminished levels of aryl-sulfatase A. Although it is exceptional the adult form of metachromatic leukodystrophy should be included in the differential diagnosis of dementia. Computerized tomography and cranial magnetic resonance together with neurophysiologic studies are the principle procedures orienting diagnosis to this disease.

Absceso epidural vertebral en un paciente con septicemia / Spinal epiural abscess in a patient with septicaemia

Introducción. El absceso epidural espinal es una entidad rara que requiere un diagnóstico y tratamiento precoz. La sepsis es un factor de mal pronóstico. Caso clínico. Mujer de 57 años con una lumbalgia aguda, que ingresó en estado de choque séptico y fracaso multiorgánico secundarios a una infección diseminada por Staphylococcus aureus, que se trató con vancomicina precozmente. Se asumió como probable fuente de infección una fascitis necrotizante del brazo izquierdo. Tras superar la fase aguda, se hizo evidente una paraparesia grave que llevó a la realización de una resonancia magnética dorsolumbar, y se demostró una espondilodiscitis lumbar con absceso epidural asociado. Conclusión. En pacientes con sepsis y algún síntoma de sospecha previo, es importante contemplar esta posibilidad diagnóstica, dado que el tratamiento antibiótico aislado no consigue evitar las complicaciones neurológicas en todos los casos (AU)

Síndrome MELAS que asemeja una encefalitis por virus herpes: diagnóstico genético / Melas syndrome masquerading as herpes encephalitis: genetic diagnosis

Introducción. El síndrome MELAS asocia miopatía mitocondrial, encefalopatía, acidosis láctica y episodios tipo ictus, que ocurren habitualmente antes de los 40 años. Es una enfermedad mitocondrial producida por una mutación puntual en el ADN mitocondrial (ADNmt), que afecta de forma más frecuente al par de bases 3243 del gen del ARN de transferencia de la leucina (ARNtLeu(UUR)). La encefalitis herpética cursa como cuadros agudos de déficit focal, habitualmente de localización temporal. En ocasiones, la presentación clínica de ambos cuadros puede asemejarse. Caso clínico. Mujer de 33 años que presentó un cuadro de instauración aguda de crisis y déficit parietotemporal derecho y que suscitó un diagnóstico diferencial entre episodio tipo ictus y encefalitis herpética. Se llegó al diagnóstico de síndrome MELAS, sospechado por el fenotipo de la paciente, mediante la detección en linfocitos de una mutación puntual en la posición 3243 del gen del ARNtLeu(UUR). Conclusiones. La presentación clínica del síndrome MELAS y de la encefalitis herpética puede tener datos similares, y debe realizarse tratamiento antivírico hasta que se confirme el diagnóstico. La determinación de la mutación puntual en la posición 3243 del ADNmt puede ser de gran ayuda para llegar al diagnóstico de síndrome MELAS de una manera no invasiva (AU)