The intrathecal delivery of r-metHuBDNF in doses of up to 150 microg/day was well tolerated and appears feasible. The reversible CNS effects with higher dose indicate that BDNF can be delivered cranially against CSF flow. The small number of patients and the design of the study did not permit conclusions to be drawn about the efficacy of the treatment.

SUMMARY Twenty eight patients with severe, intractable spasticity have been treated by chronic intrathecal administration of baclofen. An implantable programmable drug-administration-device (DAD) was used with a permanent intrathecal catheter. Infusion of 50 to 800 ug//day of baclofen completely abolished spasticity. Follow-up was up to two years. Therapeutic effect was documented by clinical assessment of tone, spasms and reflexes and by electrophysiological recordings of monoand polysynaptic reflex activity. Complications and untoward side-effects of the procedure were few. This procedure is recommended for spasticity of spinal origin refractory to physiotherapy and oral medication. It is a preferable alternative to ablative surgical intervention.After an upper motoneuron lesion in man, a spastic syndrome often develops with a delay of weeks or months. This is characterised by hyperactive monoand polysynaptic reflexes and a velocity dependent increase in muscle tone. Depending on the site of the lesion, the features of the motor disorder can vary considerably. In spinal cord transection the powerful inhibitory action of the vestibulo-spinal pathway (projecting from the lateral vestibular nucleus) on the tonic innervation offlexors is eliminated, leading to an increase in flexor muscle tone of the legs. Lesions of the reticulo-spinal pathways cause loss of inhibition of the flexion reflex, resulting in spontaneous and sometimes painful flexor spasms. On the other hand, extensor spasms can also occur. All these patients suffer frequently from severe disability having their legs in a fixed, usually flexed, position causing nursing problems and severe handicap in daily life.Flexor spasticity is often resistant to drugs in tolerable doses. Microsurgical procedures like selective peripheral neurotomy, posterior rhizotomy' and longitudinal myelotomy have been used. These interventions are designed to interrupt the spinal reflex arc or to reduce the afferent input to the dorsal horn.

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Cortical motor neurone loss and corticospinal tract (CST) degeneration are typical of amyotrophic lateral sclerosis (ALS). It is a matter of debate whether qualitative assessment of the CST by MRI is useful in the diagnosis. It is also an open question whether quantitative determination of the T2 relaxation times can improve its value. Signal intensity along the CST on 14 consecutive slices was assessed using arbitrary visual rating on double-echo T2-weighted and proton-density spin-echo images of 21 patients with ALS and 21 age- and sex-matched controls. T2 was determined quantitatively. On the T2-weighted images the patients' ratings did not differ from that of controls. The T2 of patients and controls showed no statistical difference in any slice. There was no correlation between T2 and patient age, duration of the disease, or predominant bulbar, lower or upper motor neurone signs. The only correlation between MRI findings and disease was on the proton-density images: all cases in which the CST was poorly seen were controls; a clearly high-signal CST was seen only in the patients. High conspicuity of the CST was thus specific but not sensitive for the diagnosis of ALS. T2-weighted images and measurement of T2 were not useful for diagnosis.

Maximal voluntary isometric contraction (MVIC) is a standard tool for assessment of muscle strength in treatment trials for amyotrophic lateral sclerosis (ALS). There is need for more practical bedside techniques especially for severely disabled patients. Hand‐held dynamometry (HH‐Dyn) is an inexpensive and easy‐to‐handle device. MVIC was measured in five proximal muscle groups bilaterally and compared with HH‐Dyn in 43 ALS patients. After a training period we found good intrarater correlation for HH‐Dyn (r = 0.99), with a low coefficient of variation. Measurements tended to become more accurate after repeated testing due to practice effects in examiners and patients. Overall correlation between HH‐Dyn and MVIC was good [r = 0.85 (P < 0.01)]. Strength‐range‐specific analysis showed a significant linear correlation up to 20 kg (44 lbs.) [r = 0.57 (P < 0.01)]. However, we found a tendency to underestimate muscle strength above 10 kg by HH‐Dyn as compared with MVIC, but this became meaningful only above a force of 20 kg. HH‐Dyn provides a strength estimate with a precision close to MVIC in weak muscle groups (MRC grade 4). With standardization and appropriate training, HH‐Dyn is a useful bedside test, providing an alternative to MVIC for follow‐up assessment in ALS. © 1999 John Wiley & Sons, Inc. Muscle Nerve 22: 1265–1270, 1999.