The neu gene, which encodes a putative tyrosine kinase growth factor receptor termed p185neu, was originally identified as a dominant transforming gene in neurogliomas and schwannomas induced by transplacental treatment of rat embryos with ethylnitrosourea. The present studies were undertaken to determine the expression pattern of the neu gene in peripheral nerve. Northern blot analysis of total RNA isolated from rat sciatic nerves demonstrated prominent neu mRNA expression on postnatal days 1 and 7, with substantially lower expression up to adulthood. Immunohistochemical studies confirmed expression of p185neu by Schwann cells (SC) in developing sciatic nerve and minimal p185neu immunoreactivity in adult nerves. However, neu mRNA and p185neu protein progressively increased following sciatic nerve transection in adult animals. In addition, neu mRNA and p185neu were found in neonatal rat sciatic nerve SC and several SC-derived cell lines. In resting SC, neu mRNA was expressed at a low level, but was greatly increased by treatment with forskolin and glial growth factor. These studies demonstrate that the neu gene and its protein product, p185neu, are expressed by SC both in vivo and in vitro and suggest that p185neu plays a role in the regulation of SC proliferation or differentiation.

We report two cases of pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré syndrome (GBS). The patients developed dysphagia and weakness of the neck and arms subsequent to Campylobacter jejuni infection. Oropharyngeal palsy recovered poorly. Electrophysiological findings demonstrated axonal conduction failure. Anti-GD1a immunoglobulin G (IgG) antibody was detected in one case, and anti-GM1b IgG antibody in another. Anti-GT1a IgG and immunoglobulin M (IgM) antibodies were negative in both cases. The current cases suggest that the PCB and axonal variants of GBS form a continuous spectrum from the viewpoint of electrophysiological studies as well as antiganglioside serology.

In previous studies, differential hybridization screening of an activated murine T-lymphocyte cDNA library identified an interleukin 2-responsive mRNA, designated F5, expressed in lymphoid tissues and brain only. We now report characterization of a full-length clone isolated from an adult mouse brain cDNA library. Neither the nucleic acid nor amino acid sequences demonstrated similarity to reported sequences. On Southern blotting, the protein coding sequence hybridized to genomic DNA from a variety of species. On Northern blotting, F5 mRNA was expressed in adult mouse brain, spinal cord, eye, and dorsal root ganglia but not in peripheral nerve. In situ hybridization studies demonstrated prominent expression by neurons in brain. F5 mRNA expression was undetectable in embryonic rat cerebral hemisphere and low until postnatal day 21. F5 is a novel mRNA selectively expressed by proliferating lymphocytes and mature neurons.

Abstract-The outcome of spontaneous intracranial hypotension has been unpredictable. The results of initial MRI were correlated to outcome of treatment in 33 patients with spontaneous intracranial hypotension. A good outcome was obtained in 25 (97%) of 26 patients with an abnormal MRI vs only 1 (14%) of 7 patients with a normal MRI (p ϭ 0.00004). These findings show that normal initial MRI is predictive of poor outcome in spontaneous intracranial hypotension. NEUROLOGY 2005;64:1282-1284 Wouter I. Schievink, MD; M. Marcel Maya, MD; and Charles Louy, MD Spontaneous intracranial hypotension is increasingly recognized as an important cause of new daily persistent headaches, although an initial misdiagnosis remains common.1 The cause of spontaneous intracranial hypotension is a spontaneous spinal CSF leak often associated with an underlying generalized connective tissue disorder.2 Most cases of spontaneous intracranial hypotension are believed to be selflimiting, and initial treatment is centered around a course of bedrest and hydration. Nevertheless, persistent symptoms are present in the majority of patients who come to medical attention; for those, a variety of treatment options are available, including epidural blood patching, 3 percutaneous fibrin sealant placement, 4 and surgical repair of the underlying CSF leak. 5 The reported results of these various treatments have generally been good, but the outcome of spontaneous intracranial hypotension is unpredictable and some patients have persistent and often incapacitating symptoms in spite of maximal medical and surgical treatments. The vast majority of patients with spontaneous intracranial hypotension undergo cranial MRI scanning early in their clinical course prior to any therapeutic intervention. It has been our experience that patients with recalcitrant symptoms generally have had normal MRI findings. We therefore investigated a large group of patients with spontaneous intracranial hypotension to determine whether abnormalities on initial MRI can predict outcome.Methods. The patient population consisted of a group of 33 consecutive patients with spontaneous spinal CSF leaks and intracranial hypotension who were referred to us for evaluation and treatment. The mean age of the 23 women and 10 men was 41 years (range 13 to 72 years). The presenting symptom was a positional headache in 31 patients, a nonpositional headache in one patient, and nonpositional neck pain in one patient. Cranial MRI was available for all patients, which was reviewed for features of intracranial hypotension. The presence of a spinal CSF leak was confirmed by CT myelography in all patients. Radionuclide cisternography was performed in only a few patients because CT myelography has almost completely replaced this nuclear medicine study in our practice. None of the patients had a cranial CSF leak. Treatment consisted of 1) conservative measures such as bedrest, oral hydration, oral caffeine, and use of an abdominal binder; 2) high-volume epidural blood patching (up to 80 mL) injected at th...

Lewy bodies are filamentous neuronal inclusions characteristic of Parkinson's disease, and neurofilament triplet proteins are the major components of the filaments in Lewy bodies. Since the neurofilament proteins found in Lewy bodies are abnormally phosphorylated and partially degraded, the formation of Lewy bodies may be due to the defective metabolism of these proteins, and this could lead to impairments in the structure and function of neurofilament rich neuronal processes (i.e., large caliber axons). To gain further insights into the metabolism of neurofilaments in Parkinson's disease, we evaluated neurofilament mRNA levels by semi-quantitative in situ hybridization histochemistry in postmortem tissues from Parkinson's disease and control subjects. Substantia nigra pars compacta neurons were examined with digoxigenin-UTP labeled cRNA probes to the heavy and light neurofilament mRNAs. The relative abundance of these mRNAs was measured by videodensitometric image analysis of chromogenic reaction product. Using this approach, we demonstrated that the levels of both heavy and light neurofilament mRNAs were reduced in Parkinson's disease substantia nigra pars compacta neurons. Additionally, the levels of heavy neurofilament mRNA were lowest in Lewy body containing neurons in the Parkinson's disease cases. These results suggest that the formation of neurofilament-rich Lewy bodies in substantia nigra pars compacta neurons is associated with reduced levels of the heavy and light neurofilament mRNAs in Parkinson's disease. Thus, it is possible that the accumulation of abnormal neurofilament proteins in Lewy bodies and diminished neurofilament mRNAs contribute to the degeneration of substantia nigra pars compacta neurons in Parkinson's disease.