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[Involvement of the peripheral nervous system in Crohn disease].

In medical literature peripheral neurological disorders are associated with Crohn's disease only in particular cases. We report on two patients who suffering from Crohn's disease developed an acute Guillain-Barré syndrome or a polyneuropathy associated with myositis. Two weeks after a 65-year old female patient developed a serious Guillain-Barré syndrome, a haemorrhagical Crohn's colitis was diagnosed. Within a few weeks the specific therapy led to a satisfying recovery. A 45-year old female had suffered from Crohn's disease since 12 years. Within 8 weeks she developed a serious sensorimotor mixed axonal and demyelinating neuropathy and a granulomatous myositis. After treatment with steroids her signs constantly decreased. These observations as well as a literature review show that polyneuropathy and Guillain-Barré syndrome can be associated with chronic inflammatory bowel diseases. Our results suggest that peripheral neurological signs could be regarded as a possible extraintestinal manifestation of inflammatory bowel disease.

Aged↗

Changes in fibronectin distribution in the developing peripheral nervous system of the chick.

The relationship of peripheral nerves with fibronectin was examined at different stages of the chick embryo using double immunofluorescent staining. Neurons were stained with a monoclonal antibody (E/C8) against intermediate filaments in neuronal processes, and fibronectin was stained with polyclonal antibodies. Prior to axonal outgrowth, fibronectin was distributed in a meshwork throughout the mesenchyme. However, soon after the initiation of axonal outgrowth, fibronectin began to disappear along neuronal pathways. Thus, during the period of active axonal growth, all neural tissues were marked by the striking absence of fibronectin. Interestingly, fibronectin reappeared along peripheral pathways soon after projection patterns were established. The presence of fibronectin in the substrate on which axons grow suggests that fibronectin may provide a permissive substrate for axon extension. The disappearance of fibronectin upon axon arrival suggests that neurons may modify the substrate of their pathway during outgrowth.

Animals↗

Wolman's disease: ultrastructural evidence of lipid accumulation in central and peripheral nervous systems.

We report the first case of Wolman's disease in which the fine structure of either the peripheral or the central nervous system has been examined. We confirm ultrastructurally the presence of lipid within endothelial and pericytic cells. Several cell types previously believed to be uninvolved in this storage process demonstrate lipid inclusions characteristic of Wolman's disease: perineural, endoneurial and Schwann cells of peripheral nerve, and oligodendrocytes and astrocytes of the central nervous system.

Astrocytes↗

P2 receptors in the central and peripheral nervous systems modulating sympathetic vasomotor tone.

Arterial pressure depends on the level of activity of sympathetic vasoconstrictor outflow to blood vessels. This activity is generated in the central nervous system, and involves inputs from a variety of brain regions projecting to sympathetic preganglionic neurones. Of especial interest are a group of neurones in the rostral ventrolateral medulla (RVLM), as they have been demonstrated to have a fundamental role in reflex regulation of the cardiovascular system, and in generation of tonic drive to sympathetic outflow. Sympathetic outflow to blood vessels is additionally modulated at sympathetic ganglia, and at the peripheral terminals of sympathetic nerves. This review considers the role of P2 purine receptors in this neural pathway. Ionotropic P2X receptors are expressed in the RVLM, in sympathetic ganglia, and at the sympathetic neuromuscular junction, and mediate fast excitatory neurotransmission, indicating a general role for ATP as a regulator of sympathetic vasomotor tone. P2Y receptors couple to G proteins and mediate slower signalling to ATP; they have been reported to inhibit prejunctionally neurotransmission at the peripheral terminals of sympathetic nerves, but little is known about their possible role in the central nervous system and in sympathetic ganglia.

Adenosine Triphosphate↗

[POEMS Syndrome with central and peripheric nervous system demyelination].

POEMS syndrome is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. This polyneuropathy belongs to the chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) group. Sporadic reports have emphasized the clinical association between CIDP and central nervous system (CNS) demyelination, but as far as we know not in the context of POEMS syndrome. We report the case of a 67 years old patient who developed POEMS syndrome associated to Castleman's disease, characterized by demyelinating polyradiculoneuropathy, hepatosplenomegaly, mediastinal and supraclavicular adenopathies and monoclonal IgG lambda gammapathy. Cranial magnetic resonance imaging disclosed diffuse alteration of the cerebral white matter highly suggestive of demyelination. CNS demyelination can be another feature of POEMS syndrome. Cranial MRI should be performed in patients with POEMS syndrome in order to verify this new feature.

Aged↗

[Sarcoidosis of the central and peripheral nervous system].

The authors report two cases of Besnier -Boeck- Schaumann sarcoidosis with a neurological presentation. The first case was a 25 year old woman with a 5 year history of polyneuritis affecting the cranial and spinal nerves with very low nerve conduction times and without any detectable visceral disease. The diagnosis was made by distal bronchial biopsy. The second case was of an elderly man with tremor, difficulty in keeping his balance and deafness with chronic meningitis. The diagnosis was made by proximal bronchial biopsy, chest X-Ray showing a benign hilar lymphoma. In the first case, therapy comprised two three months courses of high dose intravenous steroids followed by oral corticosteroid therapy tailing off over three weeks. In the second case, the same intravenous steroid therapy was relayed by continuous oral steroids. The therapeutic effect was spectacular in the first case, but incomplete in the second, the intravenous steroids seeming to be more effective than the oral steroids.

Adult↗