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In a 59-year-old female patient, a World Health Organization (WHO) grade II astrocytoma had been diagnosed 16 years ago, which finally progressed into WHO grade III. Several right frontal neurosurgical resections, local radiation and a local radioimplant had been applied. Despite this long record, she was reported alert with a Karnofsky index of 90% until admission. Within a few weeks she rapidly developed akinetic mutism. Upon admission, computed tomography (CT) scan showed a large cystic right frontal defect and a suggested small tumor recurrence. White matter of the frontal lobe appeared to be translucent and compatible with previous radiation. The severe mental changes were initially attributed to a delayed radiation encephalopathy. Neuropathologically, the white matter of the frontal lobe showed mild elevated cell density consistent with gliosis; however, a tumor recurrence invading the tip of the corpus callosum and invading the entire length both fornices appeared. From the neuropathological findings of massive local tumor recurrence in both fornices, together with the acute clinical onset, it seems unlikely that the sequel of radiotherapy caused akinetic mutism, but the symmetric and severe involvement of the limbic system. We conclude that the rapid progression from a state of alertness to a full clinical picture of akinetic mutism was because of infiltration of both fornices.

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Acquired neuromyotonia is characterized by hyperexcitability of motor nerves resulting in continuous muscle fiber activity. It occurs most often as a paraneoplastic syndrome in patients with cancers of the immune system. Antibodies against voltage-gated potassium channels (VGKCs) have been detected in some patients. Peripheral neuropathy is sometimes present. We report on a patient with Hodgkin's lymphoma in complete remission who developed paresthesias followed by neuromyotonia with bulbar involvement. Peripheral sensorimotor neuropathy was diagnosed electrophysiologically and evidence of axonal degeneration and demyelination was detected by sural nerve biopsy. The patient's complaints, including dysarthria, improved after carbamazepine treatment.

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To clarify the nosology of focal myositis (FM), we report the clinical and pathologic features of eight patients presenting with focal enlargement of one muscle. Most patients improved without immunosuppressive therapy, and none developed polymyositis. Pathologic features were those of an inflammatory myopathy, with muscle fiber hypertrophy and moderate to severe inflammation. In most cases, a clustering of tightly packed muscle fibers, enveloped by a thick bundle of fibrosis, was associated with the diagnosis of FM. Immunohistochemistry showed T cell predominance within the interstitial infiltrates in all cases. No evidence of vasculitis was present. Our findings suggest that FM is a benign condition that has certain clinical features separating it from other inflammatory myopathies. Pathologic changes, such as large clusters of nesting muscle fibers surrounded by thick fibrosis, are more characteristic of FM than polymyositis.

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A 57-year-old woman presented with subacute sensory, ataxic neuronopathy. Clinical investigation revealed a right-sided non-small-cell lung cancer. Serum investigation for specific antineuronal antibodies was negative. Histology showed T lymphocytic infiltrates in dorsal root ganglia. The observed histological pattern is similar to that described in antibody-positive cases. Thus, these findings suggest similar pathways in specific antineuronal antibody-negative and -positive cases of paraneoplastic subacute sensory neuronopathy.

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We analyze neuronal cytopathology and secondary reactions in spinal-muscular atrophy (SMA) in comparison with amyotrophic lateral sclerosis (ALS). In a series of SMA and ALS cases, immunohistochemistry was performed on spinal cord sections for neuronal, astroglial and microglial antigens, ubiquitin and tau proteins. Swollen motoneurons staining for phosphorylated neurofilament proteins are seen in most SMA but few ALS cases. Ubiquitinated inclusions are found only in ALS. In SMA, glial bundles are prominent in anterior roots, to lesser extent in posterior roots. In ALS, glial bundles are seen only in some cases. While basic histopathologies are similar in both types of motor neurone diseases, neuronal cytoskeletal pathology is more prominent in SMA, possibly reflecting a more acute degenerative process. The presence of axon spheroids and glial bundles also in posterior horns/roots of both types of motor neurone disease suggests spread of degenerative pathology beyond the motor system.

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Descriptions on patients with multifocal motor neuropathy with conduction block (MMNCB), including histology, nerve fiber teasing and electron microscopy on nerve biopsy, are rare. We report a 64-year old female patient with progredient weakness of the upper extremities without sensory deficit. Electrophysiology shows conduction blocks up to 80% in several examined motor nerves. A biopsy of the sensory sural nerve reveals features of a mixed axonal and de- and remyelinating neuropathy. Thus sural nerve biopsy contributes here significantly to differential diagnosis and supports an immune mediated pathogenesis of MMNCB.

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