Respiratory function deterioration is not time-linked with upper-limb onset in amyotrophic lateral sclerosis.

Introduction: In amyotrophic lateral sclerosis (ALS), symptoms apparently spread following regional rules, and depending on the site of onset. We examined if respiratory function deterioration appears earlier or is more severe in patients with upper-limb onset.

Material & Methods: We compared the results of various pulmonary function tests (PFT) obtained at diagnosis depending on the site of onset in 49 ALS patients.

Hepatic myelopathy: a rare complication of portacaval shunt.

Myelopathy is a rare complication of chronic liver disease associated with extensive portal-systemic shunt of blood. Its main clinical feature is a spastic paraparesis without sensory or sphincteric impairment. We describe 3 patients who presented hepatic myelopathy following the performance of a portacaval shunt.

Oculomotor abnormalities in motor neuron disease.

To determine whether there are oculomotor abnormalities in motor neuron disease (MND), electro-oculographic recordings were performed prospectively in 16 MND patients and the results compared with age-matched healthy controls. Parameters analysed included random and fixed saccades (latency, velocity and accuracy), smooth pursuit (gain, total harmonic distortion and number of saccadic intrusions) and optokinetic nystagmus (maximal and mean slow component velocity). Increased saccadic latencies and decreased smooth pursuit gain were the main alterations in the MND group.

Spasms of amputation stumps.

Two patients are presented with muscle spasms in an amputation stump. Neither patient experienced neuropathic pain nor phantom sensations, though phantom sensory phenomena, severe pain, and lack of response to treatment is characteristic of reported cases. One patient, a 75 year old man, has had myoclonic activity of the stump for more than two years, and the other, a 79 year old woman, recovered spontaneously after three months and is symptom free after a one year follow up.

Clinical status of motoneuron disease does not correlate with serum neurotoxicity on cultured neurons.

To test the hypothesis that a serum factor may be toxic to motoneurons in Motoneuron disease (MND), we evaluated neuronal survival in dissociated cultures from chick ciliary ganglia neurons in which MND or control sera were applied. No neurotoxic activity was found when comparing neuronal survival at 1 and 3 days in MND and control group. To examine the influence of the clinical MND status on neuronal survival, we used the following parameters: age at onset, sex, months from onset of disease to sera sampling, MND form, rhythm of progression, and scoring with Appel scale.

Myelopathy of unknown etiology. A clinical follow-up and MRI study of 57 cases.

After a careful differential diagnosis of 264 consecutive cases of spinal cord syndrome seen over a period of 10 years, no diagnosis was reached in 72 patients. Of these cases, known as myelopathy of unknown etiology (MUE), 57 were followed-up for a mean of 2.33 years, 50 being chronic cases and 7 acute cases.