Diagnosis and treatment of disorders of intracranial pressure: consensus statement of the Spanish Society of Neurology's Headache Study Group.

Primary intracranial pressure disorders include idiopathic intracranial hypertension and spontaneous intracranial hypotension. Remarkable advances have been made in the diagnosis and treatment of these 2entities in recent years. Therefore, the Spanish Society of Neurology's Headache Study Group (GECSEN) deemed it necessary to prepare this consensus statement, including diagnostic and therapeutic algorithms to facilitate and improve the management of these disorders in clinical practice. This document was created by a committee of experts belonging to GECSEN, and is based on a systematic review of the literature, incorporating the experience of the participants, and establishes practical recommendations with levels of evidence and grades of recommendation.

Diagnosis and treatment of trigeminal neuralgia: Consensus statement from the Spanish Society of Neurology's Headache Study Group.

INTRODUCTION: Trigeminal neuralgia (TN) is a chronic neuropathic pain disorder affecting one or more branches of the trigeminal nerve. Despite its relatively low global prevalence, TN is an important healthcare problem both in neurology departments and in emergency departments due to the difficulty of diagnosing and treating the condition and its significant impact on patients' quality of life. For all these reasons, the Spanish Society of Neurology's Headache Study Group has developed a consensus statement on the management of TN. DEVELOPMENT: This document was drafted by a panel of neurologists specialising in headache, who used the terminology of the International Headache Society. We analysed the published scientific evidence on the diagnosis and treatment of TN and establish practical recommendations with levels of evidence. CONCLUSIONS: The diagnosis of TN is based on clinical criteria. Pain attributed to a lesion or disease of the trigeminal nerve is divided into TN and painful trigeminal neuropathy, according to the International Classification of Headache Disorders, third edition. TN is further subclassified into classical, secondary, or idiopathic, according to aetiology. Brain MRI is recommended in patients with clinical diagnosis of TN, in order to rule out secondary causes. In MRI studies to detect neurovascular compression, FIESTA, DRIVE, or CISS sequences are recommended. Pharmacological treatment is the initial choice in all patients. In selected cases with drug-resistant pain or poor tolerance, surgery should be considered.

Spinal arteriovenous fistulas in adults: Management of a series of patients treated at a neurology department. / Fístulas arteriovenosas espinales del adulto. Manejo de una serie de casos desde una planta de Neurología.

OBJECTIVE: Spinal arteriovenous fístulas (SAVF), a rare type of vascular malformation, account for 3% of all spinal cord lesions. Without early treatment, the associated morbidity is high; furthermore, SAVF pose a major diagnostic challenge. Our purpose was to evaluate the clinical characteristics of SAVF and review their progress after treatment to determine whether it may be too late for treatment in some cases. METHODS: We present a retrospective series of 10 patients diagnosed with SAVF and treated at a tertiary hospital during a 3-year period. RESULTS: In our sample, SAVF were found to be significantly more frequent in men (80%). Mean age in our sample was 65.4 years. The most common initial symptom was intermittent claudication/paraparesis (70%). In most patients, symptoms appeared slowly and progressively. At the time of diagnosis, the most common symptoms were motor, sensory, and sphincter disorders. Mean time from symptom onset to diagnosis was 24.3 months. Initial diagnosis was erroneous in 60% of the patients. Spinal MRI was diagnostic in 90% of these cases and arteriography in 100%. The most common location of the fistula was the lower thoracic region and the most frequent type was dural (7 cases). All patients were treated with embolisation, surgery, or both and 70% improved after fistula closure regardless of progression time. CONCLUSIONS: Diagnosis of SAVF is difficult and often delayed, which leads to poorer patient prognosis. We should have a high level of suspicion for SAVF in patients with intermittent claudication or paraparesis exacerbated by exercise. Early treatment should be started in these patients. Treatment should always aim to improve quality of life or stabilise symptoms, regardless of progression time.

[Idiopathic intracranial hypertension in a patient with Chiari I malformation]. / Hipertensión intracraneal idiopática en paciente con malformación de Chiari tipo I.

CASE REPORT: The case involves a 22-year-old woman who presented with headache and decreased vision. She showed asymmetric papilledema, and a 6-mm tonsillar descent was observed in the image tests. She was diagnosed with secondary intracranial hypertension coinciding with the symptoms of a Chiari malformation (MC). DISCUSSION: Chiari malformation type I is the most common in this group of malformations, and is characterized by a greater than 5mm descent of the tonsils, being able to cause increased intracranial pressure and papilledema by blocking the flow of the cerebrospinal fluid. In this case, the MC was not the responsible for triggering the secondary intracranial hypertension, but a mere coincidence of both processes.

[Post-traumatic supraorbital neuralgia: a benign condition]. / Neuralgia supraorbitaria postraumática: una entidad benigna.

INTRODUCTION: Supraorbital neuralgia has only recently been described. Most of the cases reported involve patients suffering from chronic idiopathic neuralgias that are difficult to treat and sometimes require surgery to release the nerve. We present our experience in patients with a variant of this neuralgia which has a known causation, is commonly seen and has a benign prognosis. CASE REPORTS: We studied five patients, four females and one male, with a mean age of 55 years (range: 29-69 years). They had all suffered direct banal traumatic injury to the frontal region due to different causes. Four of them developed continuous, piercing or burning-type pain; three of them had paroxysmal pain and one had itching. There were no autonomic manifestations. All of them were found to be abnormally sensitive in the affected area, with tactile hypaesthesia, hyperalgesia or allodynia and a positive Tinel's sign. Neuroimaging tests were normal. Two patients were treated with gabapentin and amitriptyline. One was treated with an anaesthetic blockade, which afforded temporary relief. Three of them received no treatment at all. After one year of follow-up, all of them had improved and three were no longer in pain, although sensory alterations persisted in all cases. CONCLUSIONS: Post-traumatic supraorbital neuralgia is a frequent condition, although it is probably underdiagnosed. It has its own characteristic clinical and developmental features that distinguish it from idiopathic supraorbital neuralgia. Progress is usually good and it responds favourably to symptomatic treatment, if needed.

[Sarcoid myopathy. Report of two cases and review of the bibliography]. / Miopatía sarcoidea. Presentación de dos casos y revisión de la bibliografía.

INTRODUCTION: 5% of patients with sarcoidosis are affected by neurological complications, of which myopathy is one of the least frequent. We report the clinical course and therapeutic response of two patients with sarcoid myopathy. CASE REPORTS: We observed two females aged 63 and 55 who were previously diagnosed with sarcoidosis, which in one case was cutaneous and ophthalmic and in the other pulmonary. Both were asymptomatic from a systemic point of view when they visited the Neurology department. Both patients developed chronic myopathy (with a history of 9 months and 6 years), which was predominantly proximal, painful and both of them had normal or slightly high levels of creatine phosphokinase. The EMG was clearly myopathic in the two patients. A muscle biopsy showed a mononuclear-cell infiltrate with the formation of non-caseating granulomas. Both patients were treated with prednisone, although the therapeutic response was different in each case. One of the patients showed an important improvement in just a few days, while in the other case the disease remained stable, despite adding, first, azathioprine and later methotrexate to the treatment. CONCLUSIONS: Sarcoidosis can cause chronic, predominantly proximal, myopathy, and chiefly affects females over 50 years of age. The therapeutic response to steroids and immunosuppressants varies from case to case.

Neuroimaging in the evaluation of patients with non-acute headache.

Available studies offer only limited guidance on neuroimaging of non-acute headache patients. The aim of this study was to estimate the frequency of significant intracranial lesions in patients with headache and to determine the clinical variables helpful in identifying patients with intracranial lesions. All patients aged >or= 15 years attending the Neurology Clinic with non-acute headache were included in the study and followed prospectively. Every patient was investigated by neuroimaging studies, either computed tomography or magnetic resonance imaging. Neuroimaging results were classified as 'significant abnormalities', 'non-significant abnormalities' or 'normal'. Significant abnormalities included neoplastic disease, hydrocephalus, vascular malformations, Chiari malformation, large arachnoid cysts, intracranial haemorrhage, and acute cerebral infarcts. Consecutive patients (n=1876; 1243 women and 633 men) were included. Their mean age was 38 years (range 15-95 years). Neuroimaging studies detected significant lesions in 22 patients [1.2%, 95% confidence interval (CI) 0.7, 1.8]. The rate of significant intracranial abnormalities in patients with headache and normal neurological examination was 0.9% (95% CI 0.5, 1.4). The only clinical variable associated with a higher probability of intracranial abnormalities was neurological examination. The proportion of patients with headache and intracranial lesions is relatively small, but neither neurological examination nor the features in the clinical history permit us to rule out such abnormalities.

[Transient cerebral oedema associated to hypoglycaemia]. / Edema cerebral transitorio asociado a hipoglucemia.

INTRODUCTION: In the adult, hypoglycaemia is documented as a consequence of overdose of insulin or oral hypoglycaemic agents. Neonatal hypoglycaemia is common but rarely symptomatic due to protective mechanisms. Very few reports on hypoglycaemic injury are available in adults and most of them in patients with poor outcome. CASE REPORT: Woman, 45 years old. She is referred to emergency room due to insulin overdose and coma of unknown duration. Glucose level was 15 mg% without other metabolic anomalies. Computerized tomography revealed brain oedema most obvious in the parieto-occipital lobes. Therapy with manitol and glucose was started with total functional recovery. CONCLUSIONS: Brain imaging in neonatal hypoglycaemia shows similar findings. In the acute phase oedema in the parieto-occipital cortex and underlying white matter and atrophy or malacic cysts in the chronic phase have been described. Chronic changes are related to long-term sequelae that vary from development delay and epilepsy to persistent vegetative state. In adults lesions involve hippocampus and basal ganglia. The reasons of different sensitivity of these regions are not fully clear. Differences in regional blood flow autoregulation or in excitotoxins receptors of aspartate have been postulated. Hypoglycaemia may induce blood-brain-barrier permeability and subsequently brain oedema.

[The importance of neurological examinations in the age of the technological revolution]. / Importancia de la exploración neurológica en la era de la revolución tecnológica.

INTRODUCTION: Neurologic practice and care have been modified in many important ways during the past ten years, to adapt to the explosion of new information and new technology. Students, residents and practicing physicians have been continuing programs to a model that focuses almost exclusively on the applications to neurologic disorders of the new knowledge obtained from biomedical research. On the other hand high demand for outpatient neurologic care prevents adequate patient's evaluation. CASE REPORTS: Case 1: 65 years old female. Occipital headache diagnosed of tensional origin (normal computerized tomography). Two months later is re-evaluated due to intractable pain and hypoglossal lesion. An amplified computerized tomography revealed a occipital condyle metastasis. Case 2: 21 years old female. Clinical suspicion of demyelinating disease due to repeated facial paresis and sensitive disorder. General exploration and computerized tomography revealed temporo-mandibular joint. Case 3: 60 years old female. Valuation of anticoagulant therapy due to repeated transient ischemic attacks. She suffered from peripheral facial palsy related to auditory cholesteatoma. CONCLUSIONS: Neurologic education is nowadays orientated to new technologies. On the other hand, excessive demand prevents adequate valuation and a minute exploration is substituted by complementary evaluations. These situations generate diagnostic mistakes or iatrogenic. It would be important a consideration of the neurologic education profiles and fulfillment of consultations time recommendations for outpatients care.

Clusterlike headache as first manifestation of a prolactinoma.

We report a case of cluster headache in a patient with a macroprolactinoma. Symptomatic cluster headache was suspected because of an unsatisfactory response to medications that are usually effective in idiopathic cluster headache. The neurological examination was normal. However, magnetic resonance imaging demonstrated a large pituitary tumor. One year after starting treatment with cabergoline, the patient remains asymptomatic. Symptomatic cluster headache should be suspected when the clinical features of the headache are atypical. By inducing vascular mechanisms, the parasellar lesion may have played a role in initiating the cluster headache.