[Intracranial hypotension syndrome: a review of the magnetic resonance findings]. / Sindrome de hipotension intracraneal: revision de hallazgos en resonancia magnetica.

INTRODUCTION: Intracranial hypotension syndrome (IHS) is a syndrome with a variable aetiology and clinical presentation that is, in most cases, caused by leakage of cerebrospinal fluid (CSF) through the thecal sac. Orthostatic headache associated to the typical magnetic resonance imaging (MRI) findings, secondary to depletion of CSF, is the key to a correct diagnosis. AIMS: To show the imaging findings that, within a suitable clinical context, allow this condition to be identified and diagnosed. DEVELOPMENT: Decreased CSF volume plays an important role in IHS, which leads to an increase in the compensatory volume of blood, essentially dependent on the venous system. MRI is a sensitive technique in the diagnosis of IHS. Yet, separate findings are unspecific. The MRI findings include diffuse and homogeneous dural enhancement, the presence of small bilateral subdural collections, caudal displacement of the encephalic structures (pseudo-Chiari), dilatation of the cortical and medullar veins, and the recent sign of venous distension. This last sign is a highly sensitive finding of IHS, which tends to disappear following the patient's clinical improvement even before the disappearance of the pachy-meningeal enhancement, and could be used as a marker for response to treatment. CONCLUSIONS: IHS is a condition that is difficult to diagnose clinically for which several typical MRI findings have been reported; both neurologists and radiologists must be familiar with these findings.

Lumbalgia como síntoma inicial de una adrenomieloneuropatía / Low-pain back as initial symptom of adrenomyeloneuropathy

Introducción. La adrenoleucodistrofia es una enfermedad peroxisomal, recesiva ligada al X, caracterizada bioquímicamente por acúmulo de ácidos grasos de cadena extralarga (AGCE).Se distinguen seis fenotipos: presintomático, forma cerebral infantil, del adolescente y del adulto, adrenomieloneuropatía y enfermedad de Addison aislada. Presentamos un paciente con adrenomieloneuropatía cuya manifestación clínica inicial fue una lumbalgia.Caso clínico. Varón de 23 años, sin antecedentes personales de interés, que acudió por lumbalgia de dos años de evolución. En la exploración del paciente había piramidalismo y disminución de las sensibilidades epicríticas en miembros inferiores. La analítica mostró una insuficiencia suprarrenal y aumento de las concentraciones de AGCE en suero, mononucleares y fibroblastos. Una RM mostró una lesión hiperintensa en la rodilla del cuerpo calloso. Los PESS fueron patológicos, mientras que el electroneurograma mostró un aumento bilateral de la latencia de la onda F. El paciente fue diagnosticado de adrenomieloneuropatía, por lo que se inició tratamiento con hidrocortisona, dieta pobre en AGCE y `aceite de Lorenzo'. La lumbalgia fue desapareciendo progresivamente, coincidiendo con la disminución de las concentraciones plasmáticas de AGCE. El aceite fue retirado por plaquetopenia moderada. Se inició, entonces, tratamiento con lovastatina 40 mg/día. Dos años después, el paciente se encuentra sin lumbalgia y estable desde el punto de vista neurológico. Conclusiones. La lumbalgia asociada a la adrenomieloneuropatía es probablemente debida a desmielinización en los tractos espinales. Aunque la lumbalgia es un cuadro habitualmente `benigno', se debe realizar una buena anamnesis a todo paciente con dicho síntoma (AU)

[Backache as the initial symptom of adrenomyeloneuropathy]. / Lumbalgia como síntoma inicial de una adrenomieloneuropatía.

INTRODUCTION: Adrenoleukodystrophy is a paroxysmal disorder, with recessive linking to the X chromosome, characterized biochemically by the accumulation of extra-long-chain fatty acids. Six phenotypes are distinguished: pre-symptomatic, infantile, adolescent and adult cerebral forms, adrenomyeloneuropathy and isolated Addison's disease. We describe a patient with adrenomyeloneuropathy in whom the presenting symptom was lumbago. CLINICAL CASE: A 23 year old man with no significant previous clinical history complained of having lumbago for over two years. On examination he had pyramidal signs and reduced epicritic sensitivity of the legs. Laboratory investigations showed adrenal failure, increased plasma extra-long-chain fatty acids concentration, mononuclear cells and fibroblasts. On MR there was a hyperintense lesion of the genu of the corpus callosum. SSEP were pathological while the electroneurogram showed bilateral increase in latency of the F wave. The patient was diagnosed as having adrenomyeloneuropathy and treatment started with hydrocortisone, a diet low in extra-long-chain fatty acids and 'Lorenzo's oil'. The lumbago gradually disappeared while the plasma extra-long-chain fatty acids concentration dropped. The oil was stopped because of moderate thrombocytopenia, and treatment was started with lovastatin 40 mg/day. Two years later the patient has no lumbago and is neurologically stable. CONCLUSIONS: The lumbago associated with adrenomyeloneuropathy is probably due to demyelination of the spinal tracts. Although lumbago is usually a benign condition, a careful history and examination is necessary in all such cases.