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El trabajo ha sido presentado parcialmente como presentación tipo póster en la XXXVII Reunión de la Sociedad Andaluza de Neurología, 2014., La neuropatía axonal aguda, motora o sensitivo-motora, es una forma grave e infrecuente del síndrome de GuillainBarré (SGB)1. Aunque suele relacionarse con una etiología infecciosa, de forma excepcional puede presentarse como un síndrome paraneoplásico2, por lo que consideramos de interés la comunicación de un nuevo caso de tales características.

Coeliac disease is a gluten sensitive enteropathy, autoimmune in origin, which has been traditionally regarded as a gastrointestinal disease. Years later it has been reported an extraintestinal affection. A huge number of neurological syndromes of unknown cause had been initially described in association with coeliac disease, with total or partial response to a gluten free-diet. A specific kind of occipital cerebral calcifications in relation to coeliac disease has been also described, and sometimes it means the existence of a syndrome called "Gobby's Syndrome". We show a patient with a mild unknown coeliac disease, a woman who had occipital cerebral calcifications in a TAC cerebral, which was made because of her intractable migraines and that it lead to the diagnosis. The migraine disappeared after a gluten free-diet, like similar cases reported by literature. The fact of existing neurological symptoms associated to coeliac diseases opens a therapeutic window of opportunity because they would respond to a gluten free-diet.

Reversible posterior leukoencephalopathy syndrome is a brain disorder characterized by headache, nausea, vomiting, visual disturbance, depressed level of consciousness, convulsions and occasionally focal neurologic deficits. It is commonly associated with malignant hypertension, toxemia of pregnancy or the use of immunosuppressive agents. Early diagnosis and specific treatment is essential. We report a case of reversible posterior leukoencephalopathy in the context of a hypertensive crisis in an habitual cocaine sniffer. Reversible posterior leukoencephalopathy must be suspected in every patient with hypertensive crisis and compatible clinic manifestation. Neuroimaging studies show characteristic features which confirm the diagnosis.

Two Spanish sibs with familial amyloidotic polyneuropathy (FAP) homozygous for the V30M-TTR gene, were diagnosed by DNA and protein analyses. Their clinical picture was very similar to the Majorcan FAP heterozygous patients except for the sensorimotor syndrome which was more aggressive. Noteworthy were clinical differences between the sibs concerning autonomic involvement, cranial neuropathy and kidney disturbances. These differences can be due to genetic and/or environmental factors.

A 53-year-old right-handed man acutely developed stuttering. On his examination there was blocks and repetitions exclusively on first syllables of words in conversational speech, improving in automatic tasks. There was neither aphasia, nor other neurological deficits. An MR imaging of the brain showed a circumscribed cortical infarct on the left precentral circunvolution. Cerebral angiography was consistent with atherosclerotic narrowing of the intracavernous segment of left internal carotid artery. Artery-to-artery embolism was the suggested mechanism for this stroke and the patient was treated with oral anticoagulants for six months. Stuttering improved progressively and the patient became asymptomatic a month after the stroke. Ictal acquired stuttering symptomatic of ischemic stroke without aphasia or other neurological deficit is exceptional. An small infarct on the left motor area as cause of isolated acquired stuttering adds new information about neural circuits involved in this phenomenon.

A 65-year-old woman developed a progressive action tremor on her left hand for the last two years. On examination an intentional left hand tremor with dysmetria and tendency to deviate to left on tandem were identified. There was no mental deterioration. CT scan and MR imaging disclosed a marked unilateral, left, cerebellar hypoplasia. MR also showed multiple lacunae. Neurological family disease and exposition to drugs or alcohol were not present. In some cases cerebellar hypoplasia could become symptomatic at advanced age as result of concurrent cerebral disease affecting the compensating structures for congenital defect. In this patient a vascular mechanism is suggested as possible late trigger.

The etiology of lumbosacral plexopathy is often due to compression. One of the less common causes of this is aneurysm of the iliac artery. However, 13% of the patients with this disorder initially have symptoms of plexus irritation or deficit.We describe the case of a 42 year-old-man, with no previous medical history, who complained of right-sided sciatica for the previous three months. On examination there were clinical signs of a lesion of the right lumbosacral plexus. On CT of the pelvis and MR of the lumbar spine there were images compatible with an aneurysm of the right iliac artery. This diagnosis was confirmed on arteriography. Treatment was surgical (aneurysmography and right iliofemoral by-pass). The pain disappeared and the motor deficit improved considerably.When a patient presents with lumbosacral plexopathy, an iliac aneurysm should be considered as a possible etiological factor. In such cases early diagnosis is essential, since surgical treatment will usually resolve the clinical problem.

Single-photon emission photometry (SPECT) images of the brains of 60 patients with Alzheimer's disease (AD) were compared with the brain scans of 16 healthy controls of the same sex and similar ages. SPECT was repeated one year later in 16 of the AD patients. Perfusion indices for frontal, parietal, temporal and occipital regions of patients and controls were compared (Student's tests). Flow alterations were located and symmetry examined. The correlation between perfusion index for each cortical area and MMSE score was assessed (Pearson's r). Perfusion alterations were present in all except one AD patient, and in 65% of AD patients such alterations were in the parietotemporal cortex. Severity of cognitive impairment in AD patients was related to perfusion changes in the left posterior temporal region. Forty-four AD patients showed asymmetrical distribution of tracer and in 73% perfusion changes were predominantly in the left hemisphere. Altered brain blood flow is common in AD and onset is early. The parietotemporal location of defective perfusion is of great diagnostic value. Asymmetry is common and the left hemisphere is most often affected.

We describe a patient with a diagnosis of Hansen's disease borderline type, presenting as cutaneous lesions and silent multineuritis. Samples of nasal mucus, earlobe and cutaneous lesions were positive for acid-fast bacilli. He was given treatment with rifampin, dapsone and clofazimine. Five years later, he developed fever, poliarthritis, orchitis and hepatic involvement. Searching for acid-fast bacilli in many cutaneous and mucosal locations was fruitfulness. Because of clinical suspicion of erythema nodosum leprosum, he was treated with steroids with improvement of his clinical picture, but subsequently he developed multineuritis with many sensitive symptoms. A high number of bacilli was seen in nerve biopsy. We comment on atypical features of clinical evolution and erythema nodosum leprosum, and emphasize the significance of large number of bacilli into peripheral nerve in contrast with their absence at other levels.

The clinical findings of basilar dolichoectasia (DB) are caused by compression of the cranial nerves, vertebrobasilar ischaemia, hydrocephaly and compression of the brainstem. Hypoacusia as an isolated finding in DB is very rare.We describe the case of a 48-year-old hypertensive man with bilateral hypoacusia as an isolated finding in DB. Diagnosis was made on MR, and arteriography showed changes characteristic of an anomalous blood vessel.In patients with hypoacusia of uncertain aetiology, specially if they are also hypertensive, one should rule out the possibility of DB using cerebral magnetic resonance or angioresonance.

Familial amyloidotic polyneuropathy type I (FAF-I) is caused by a specific genetic mutation that gives rise to a transthyretin anomaly whose presence in serum constitutes the biochemical marker for this disease. We studied the serum of 7 patients and 16 asymptomatic members of their immediate families using ELISA with FD-6 monoclonal antibody to detect the transthyretin anomaly. Positive results were found for the 7 patients, including the 2 patients whose disease was apparently sporadic, and 12 carriers were detected among the family members. This technique makes sural nerve biopsy unnecessary for establishing a diagnosis in patients whose clinical signs are consistent with FAP-I. Asymptomatic carriers are also detected, facilitating appropriate genetic counseling.

A 45-years-old woman developed palilalia without other neurologic symptoms or signs. Cranial MR and CT scan showed only bilateral thalamic infarcts; the SPECT showed bilateral frontoparietal hypoactivity. We conclude that involvement of the supplementary motor area by diaschisis after bilateral thalamic lesions may produce palilalia.

We report a patient that developed a sensorimotor polyneuropathy more than a year before the appearance of the typical clinical signs of progressive systemic sclerosis. A sural nerve biopsy showed epineural vasculitis with involvement of the basal membrane of the endoneural vessels, without proliferation of the connective tissue.

Primary antiphospholipid syndrome (PAS) is characterized by the isolated presence of antiphospholipid antibodies in the absence of other immunological changes. Its association with ischemic cerebrovascular disease is rarely reported. This study analyzes this association in 10 patients (8 women) first presenting with ischemic cerebrovascular accident (ICVA) that met the criteria for PAS. The average age of the patients was 50.4 years. Two patients had Sneddon's syndrome. Prolonged thromboplastin partial activation time was found in only three patients. Eight started aspirin therapy. The average time of follow-up was 17.2 months, during which there was one relapse. PAS should be included as a possible cause when examining individuals with ICVA, particularly when patients are young, when abortion has occurred or when no other cause is evident. Acetylsalicylic acid is effective for preventing relapse when other vascular risk factors are carefully controlled.

In young patients episodes of amaurosis fugax usually correspond to processes not related with atherosclerosis of the extracranial vessels such as migraine, cardiac embolism, vasospasm, vasculitis, oral contraceptives or hematologic disease. In 11% of the cases, however, no cause can be found. These idiopathic cases generally have a benign course and their frequency decreases spontaneously allowing them to be described as benign amaurosis fugax. We describe four cases of amaurosis fugax in which no responsible cause was found despite extensive clinical, laboratory ultrasonographic and angiographic studies. The episodes progressively remitted. In these cases of benign amaurosis fugax the risk of a permanent deficit is exceptional and treatment is not necessary.

Neoplastic lumbosacral plexopathy is a frequent and disabling complication in subjects with cancer. Its clinical presentation is characterized by pain, muscle weakness, sensory complaints in one or occasionally both limbs associated with the tumoral symptoms. The presence of autonomic symptoms is less frequent; one of these is the "hot and dry foot". We present two patients, one with prostatic cancer and the other with myxoid liposarcoma, who developed a lumbosacral plexopathy as a manifestation of the extension of the neoplastic process; in both cases there was in addition a clear difference in the temperature of the affected limb. Although infrequent, the "hot and dry foot" it constitutes an early sign of metastatic plexopathy which facilitates the differential diagnosis with preforaminal lumbosciatic radiculopathies.