Atypical Perfusion Manifestation in Migraine with Aura.

Migraine with aura may be confused with a stroke. Magnetic resonance imaging is an important tool for the differential diagnosis. Cerebral hypoperfusion has been described in classic migraine, mainly during the aura. A 47-year-old male had an unremarkable past medical history. After sneezing, he developed a left hemi hypoesthesia, bitemporal vision loss, photopsia, and some distortion in the position of letters and words. This lasted <1 h, and it was followed by a severe headache. A magnetic resonance angiography was performed during the headache. It showed a left hemispheric hypoperfusion that did not correlate with the symptoms described by the patient. It is believed that during the aura, cerebral blood flow decreases, leading to hypoxia and decreased cellular energy generation, and these metabolic alterations define the symptoms of the patient. In our case, we documented brain hypoperfusion during the headache in the ipsilateral brain hemisphere to the symptoms, which has no clinical correlation. This condition could be due to spasm in the capillary arteries, and it may persist and influence the clinical manifestations during the headache phase in migraine with aura. A state of generalized cerebral hyperperfusion has been suggested, and there may be a coexistence of both phenomena for some period. This may open a new line of research regarding the pathophysiology and vascular changes of migraine with aura.

Impaired glucose tolerance with neurological manifestations in insulinoma.

Insulinoma may have an atypical presentation and it should be suspected in patients with neurological manifestations in spite of an atypical insulin and proinsulin levels associated with hypoglycemia. Fast test is an important tool to reach the diagnosis.

Stiff Person Syndrome and Acetylcholine Receptor Ganglionic Neuronal Antibodies.

The association between stiff person syndrome and paraneoplastic syndromes has been described, linking intracellular or extracellular antibodies. We describe the case of a 64-year-old woman with stiff person syndrome and positivity for acetylcholine receptor ganglionic neuronal antibodies, which can also be seen in muscular hyperexcitability conditions, as well as other paraneoplastic syndromes. The relevance of this report is the possible direct elevation of this antibody due to a direct immunological cause. Acetylcholine receptor ganglionic neuronal antibodies have been reported with autoimmune autonomic neuropathy and paraneoplastic syndromes linked to lung cancer and myastheniform syndromes, as well as in some muscular hyperexcitability states, but not in stiff person syndrome.

Treatment with Botulinum Toxin for Refractory Fever Caused by Severe Spasticity: A Case Series.

INTRODUCTION: Brain and spinal cord injuries may cause very severe spasticity that occasionally may be associated with persistent fever. CASE SERIES: We present 14 patients with spasticity and persistent fever, treated with botulinum toxin type A. Their spasticity improved and the fever resolved within a period no greater than 48 h. In all cases, infectious and other non-infectious causes were ruled out. CONCLUSIONS: When sustained tonic muscular activity is associated with a significant increase in body temperature and is refractory to the usual drugs used for hyperpyrexia, type A botulinum toxin may be an effective treatment option to control both spasticity and fever.

Parkinson's disease and genetics.

BACKGROUND: Idiopathic Parkinson disease (IPD) is a condition of unknown cause. Several factors are believed to contribute to its onset, and many studies have been conducted in search of the possible etiology of Parkinson disease. REVIEW SUMMARY: Genetic factors have become relevant when trying to explain the onset of Parkinson disease. The studies are divided into 2 categories: epidemiological and studies that analyze twins from families with members suffering from Parkinson disease, thus looking for the responsible genetic mutations. In this article we address this controversial topic, reviewing some of the most significant studies trying to provide evidence which relates genetics to Parkinson disease. CONCLUSION: We present current epidemiological studies and the most important genetic factors related to Parkinson disease, including the latest information currently available on each issue.

Treatment of primary progressive freezing of gait with high doses of selegiline.

We report the case of a 76-year-old, right-handed woman with progressive primary freezing of gait. Despite several therapeutic strategies, she continued to worsen to the point that she became confined to a wheelchair. Treatment with selegiline in doses up to 20 mg/d led to marked improvement of the gait disorder. This case illustrates that selegiline can be an option for patients with freezing of gait other than those with Parkinson's disease.

Wyburn-Mason syndrome.

Wyburn-Mason syndrome is an uncommon condition in which arteriovenous malformations coexist in the visual brain area, in the retina, and in some cases, in the face. As a consequence, it has recently been defined as a brain-retino-facial angiomatosis. The syndrome results from an embryonary insult in the development of the optic nerve path and the related vessels from its origin in the mesencephalon all the way to the projection to the retina where it usually causes unilateral manifestations. Here the authors present an interesting case of this ailment and a review of the literature.