Estrogen receptors localization in the spinal trigeminal nucleus: an immunohistochemical study in humans.

There is increasing evidence for estrogenic modulation of neurotransmission within the trigeminal pain pathway. It is also likely that the effects of estrogens may be influenced by the presence and localization of estrogen receptors (ERs) in a given brain area. To date, human data on the localization of ERs in the spinal trigeminal nucleus (STN), a key brain region in craniofacial nociception, are lacking. To ascertain whether ERs are expressed in the human STN, we performed immunohistochemical analysis on medulla oblongata samples taken from eight adult subjects (three men and five women; age range, 23-71 years) who had died from causes unrelated to neurologic or endocrine diseases. Paraffin-embedded sections at the level of the subnucleus caudalis and interpolaris were incubated with anti-estrogen receptor alpha (ERα) and anti-estrogen receptor beta (ERß) antibodies. ERα immunoreactivity was detected in the nucleus and cytoplasm of neuronal and glial cells in the STN and in the nerve fibers within the spinal trigeminal tract in all eight subjects; ERß immunoreactivity was observed in the cytoplasm of neuronal cells in five subjects. This study is the first to provide evidence in humans that ER immunoreactivity is detectable on neuronal and glial cells of the STN. The two ER subtypes exhibited different expression patterns, with higher expression levels of ERα than ERß. The presence of ER-containing cells in the STN suggests that estrogens may directly affect trigeminal neuron excitability in humans.

Adult-onset migraine-related ophthalmoplegia and omolateral fetal-type posterior cerebral artery.

A 33-year-old woman with a long history of typical migraine without aura developed a pupillary-involving right third nerve palsy, after a typical migraine attack. The right pupil was 5 mm and showed delayed direct and consensual photomotor responses; the left pupil was 3 mm and reactive. Pupillary reaction to convergence was slow on the right eye. Ptosis, impaired elevation of the eye and weakened adduction were noted in the right eye. CT scan of the brain showed no abnormalities, whereas a CT digital cerebral angiography revealed a fetal-type right posterior cerebral artery (PCA). MRI disclosed thickening and contrast-enhancement of the cisternal portion of the right oculomotor nerve. A lumbar puncture, performed 5 days after the onset of ocular symptoms, yielded acellular cerebrospinal fluid (CSF) with normal protein and glucose levels. Ptosis and diplopia recovered within a week, whereas blurred vision, anisocoria and accommodation deficit subsided after 10 weeks.

Ataxia and migraine-like headache in a girl with a cerebellar developmental venous anomaly.

Cerebral developmental venous anomalies (DVAs) are generally considered as anatomical variants of the venous system without clinical importance. We report the case of a 15-year-old girl with recurrent episodes of migraine-like headache who presented with subacute vertigo and ataxia associated with intense occipital pain. Magnetic resonance imaging (MRI) showed a DVA with signal modifications of the surrounding brain parenchyma in the left cerebellar hemisphere. The patient's ataxia regressed completely within about 2 months. On a follow-up MRI 4 years later the venous malformation and the parenchymal abnormalities were unchanged. We attribute the patient's focal neurological dysfunction to regional changes in the brain parenchyma, possibly secondary to venous hypertension. Our report provides evidence that also uncomplicated DVAs can become symptomatic and supports the role of the venous congestion within the DVA territory in pathogenesis of some brain parenchymal abnormalities associated with DVAs.

Endothelial adhesion molecule expression is unaltered in the peripheral nerve from patients with AIDS and distal sensory polyneuropathy.

Some evidence suggests that endothelial dysfunction, including altered expression of cell adhesion molecules contributes to pathophysiology of nervous system disorders in the course of HIV infection. In this immunohistochemical study we investigated and compared the expression of E-selectin, intercellular adhesion molecule-1 (ICAM-1), platelet endothelial cell adhesion molecule-1 (PECAM-1) in peripheral nerve blood vessels from patients with distal sensory polyneuropathy (DSP) and control patients with other axonal neuropathies. Similar quantitative pattern of immunoreactivity was found in patients with DSP and controls. E-selectin and PECAM-1 immunostained vessels tended to increase in number only in patients with major CD4 cell depletion. The vascular endothelium of the peripheral nerve in HIV-infected subjects with DSP shows no changes facilitating the migration of infected or activated monocytes/macrophages into the nerve. These phenomena probably do not play a critical role in the development of axonal damage in DSP.

Enhanced expression of E-selectin on the vascular endothelium of peripheral nerve in critically ill patients with neuromuscular disorders.

Neuropathic complication often occurs in critically ill patients, and changes in the microcirculation of the peripheral nerve have been suggested to play a role in the pathogenesis of the nerve lesion. We report the results of a pathological and immunohistochemical study of superficial peroneal nerve biopsy specimens in a series of 22 critically ill patients with sepsis and neuromuscular disorders. Eight patients had histopathological features of axonal neuropathy compatible with critical illness polyneuropathy (CIP). The nerve lesions ranged in severity from mildly reduced myelin-fiber density with sporadic axonal degeneration to marked fiber loss with abundant degenerative changes. In no patient did we detect evidence of primary demyelinization or inflammatory infiltrates. We analyzed the immunohistochemical expression of E-selectin, vascular cell adhesion molecule-1 (VCAM-1), intercellular adhesion molecule-1 (ICAM-1) and tumor necrosis factor alpha (TNF-alpha) in nerve microvessels. Expression of E-selectin was significantly increased in endothelium of epineurial and endoneurial vessels, suggesting endothelial cell activation. These findings again confirm axonal degeneration as the major pathological feature of CIP. Our immunohistochemical data provide first evidence that activation of the endothelial cells of the microvessels in the endoneurium of human peripheral nerve does occur during sepsis. This specific activation might have implications with the mechanisms responsible for the axonopathy in critically ill patients.