Reversible ALS-like disorder in HIV infection.

OBJECTIVE: To describe the clinical features, treatment, and outcome of six cases of HIV-1-associated ALS-like disorder. METHODS: The authors reviewed patients with HIV infection with neurologic symptoms seen over a 13-year period. Patients were identified by using the El Escorial research diagnostic criteria defining three categories of certainty for definite, probable, or possible ALS. Clinical features, EMG, CSF, serum analyses, and imaging and virological studies were assessed. RESULTS: Six patients with immunodepression (mean CD4(+) cells = 86.2/mm(3); mean age = 34 years) developed distal motor weakness mimicking a monomelic amyotrophy that subacutely progressed regionally or assumed a symmetric distribution on more than one region. EMG was characteristic of motor neuron disease with no multifocal conduction block. Causes other than HIV-1 were ruled out. The unusual rapid extension of the disease and the positive response to antiretroviral therapy suggest that ALS syndrome and HIV infection are etiologically related. HIV-1 might cause an ALS-like disorder by several mechanisms-via neuronal infection, by secretion of toxic viral substance, by inducing the immune system to secrete cytokines, or by inducing an autoimmune disease. CONCLUSION: These cases suggest that the association between some motor neuron diseases and HIV infection is not coincidental but pathogenetically related and that ALS-like disorder should be considered an HIV-related neurologic complication.

[Postoperative combined medullary sclerosis revealing Biermer's disease: toxic effect of nitrous oxide]. / Sclérose combinée de la moelle post-opératoire révélant une maladie de Biermer: toxicité du protoxyde d'azote.

Combined medullary sclerosis developed suddenly postoperatively in a patient with unknown Biermer's disease. The neurological lesions were undoubtedly induced by nitrogen protoxide via an inactivation of vitamin B12.

[True neurological thoracic outlet syndrome]. / Syndrome du défilé thoraco-brachial à forme neurologique.

The thoracic outlet syndrome (TOS) encompasses various clinical entities affecting the neurovascular bundle crossing the thoracic outlet. Unfortunately, this term often proves to be confusing because many of these entities have little in common beyond their known or presumed lesion site. Neurogenic TOS (true TOS) is caused by compression of the lower trunk in the brachial plexus, the cervical ribs or fibrous band. This syndrome is extremely rare. We consider that this neurological form of TOS is a clearly defined neurological syndrome. We report 10 patients with true TOS. All were females. Stating the onset was difficult because symptoms were progressive and insidious. Pain was the most frequently reported symptom. Sensory deficit was slight or absent. All patients showed unilateral severe atrophy of the thenar muscles. Wasting and weakness developed later. A reduced amplitude of ulnar and median compound muscle action potential associated with a normal amplitude of median sensory nerve action and a reduced amplitude of ulnar sensory nerve action potential were indicative of a chronic axon loss in the lower trunk of the brachial plexus. In all cases, we performed medial antebrachial cutaneous sensory nerve action potential, a C8-T1 innervated nerve. The absence of the medial antebrachial cutaneous sensory nerve action potential in 9 patients and a reduction in amplitude of 50 p. 100 compared to the unaffected side in the other patient, indicated the diagnostic value of this easy and reproductible test. It confirmed a C8-T1 post-ganglionic radicular lesion or a lower brachial plexus neuropathy. Radiography showed a rudimentary bilateral cervical rib or an elongated C7 transverse process in all cases. Surgery was performed in the affected side in 7 patients and in each case the lower part of the brachial plexus was found to be stretched and angulated over a fibrous band, which was removed. Pain was relieved after 1 to 4 weeks. A minimal motor improvement was observed after one year. Electrophysiological results were unchanged.

True neurogenic thoracic outlet syndrome: electrophysiological diagnosis in six cases.

We report 6 patients with true neurogenic thoracic outlet syndrome. All were female and presented unilateral severe atrophy of the thenar muscles. Pain in the affected upper limb was frequently reported, but sensory deficit was slight or absent. Reduced amplitude of ulnar and median compound muscle action potential associated with a normal amplitude of median sensory nerve action potential (SNAP) and a reduced amplitude of ulnar SNAP was indicative of a chronic axon loss in the lower trunk of the brachial plexus. The absence of the medial antebrachial cutaneous SNAP in 5 patients and a reduction in amplitude compared to the unaffected side in the other patient indicated a C8-T1 postganglionic radicular lesion or a lower brachial plexus neuropathy. Radiography showed a rudimentary bilateral cervical rib or an elongated C7 transverse process in all cases. Surgery was performed, and in each case the lower part of the brachial plexus was found to be stretched and angulated over a fibrous band, which was removed. Pain was relieved after 1-4 weeks, but at 1 year, there was only minimal motor improvement and the electrophysiological results were unchanged.

[Multifocal motor neuropathies with conduction blocks. 39 cases]. / Neuropathies motrices multifocales avec blocs de conduction. 39 cas.

Clinical, biological and electrophysiological features from a cohort of 39 multifocal motor neuropathies with conduction blocks (NMM with CB) have been studied. There were 29 males and 10 females with an average of 47.3. At the first evaluation, the mean duration of the symptoms was of 8 years with extremes between 1 and 28. Pain and paresthesias were present in respectively 10 and 18 p. 100 of the patients. Fasciculations and cramps were observed in more than 2/3 of the cases. Three patients had tremor at rest. Upper limb muscular weakness was the predominant initial symptom (84.6 p. 100). The weakness always affected distal and unilateral muscles. Radial and cubital nerve distribution are mainly affected and in half of the cases an unilateral motor deficit in the lower limb was associated. Muscle atrophy was frequent (74 p. 100) and rapidly developed in the first 2 years. Reflexes were decreased or absent in 64 p. 100. In 78 p. 100 of cases, biological study showed normal serum immunoelectrophoresis and CSF. IgM anti-GM1 antibodies were found in 24/36 patients. Very high titres were found in 5 cases. All patients had CB in upper limbs. The preferential localizations of the CB were equally at the median and ulnar nerves. Only 7 patients had CB localized to the lower limbs. In many cases, marked reduction of the motor amplitude prevented the detection of CB, marked reduction of the motor amplitude prevented the detection of CB. Moderate fibrillation potentials were found in 28 p. 100 of patients. Giant muscular unit potentials were frequent (21/39). F-waves in nerve with CB were always abnormal with marked increased latencies. Late responses sometimes seemed to be repeater F-waves. Axon reflexes were detected in 5 cases. The late responses abnormalities could precede the block. Clinical, biological and electrophysiological described arguments could may distinguish NMM with CB from motor neuron disease and relate them to the group of chronic demyelinating neuropathies.

Multifocal motor neuropathy with conduction block: a study of 24 patients.

Twenty four patients with pure motor neuropathy are reported. The chronic motor involvement associated with fasciculations and cramps, mainly in the arms, led, in most patients, to an initial diagnosis of motor neuron disease. In some patients (nine of 24), there was no appreciable muscle atrophy. Tendon reflexes were often absent or weak. The finding of persistent multifocal conduction block confined to motor nerve fibres raises questions about the nature and the importance of this syndrome. Segmental reduction of motor conduction velocity occurred at the site of the block, but significant slowing of motor nerve conduction was not found outside this site. The response to intravenous IVIg treatment seems to be correlated with the absence of amyotrophy. Patients with little or no amyotrophy had an initial and sustained response to IVIg, and did not develop amyotrophy during the follow up study. They could be considered to have a variant of chronic inflammatory demyelinating polyneuropathy. Patients with pronounced amyotrophy independent of the disease duration did not respond as well to IVIg treatment, suggesting the existence of a distinct entity. Among the patients treated about two thirds who had an initial good response to IVIg had high or significant antiganglioside GM1 (anti-GM1) antibody titres, but there was no correlation between the high titres before treatment and long lasting response to IVIg treatment.

Nerve biopsy findings in different patterns of proximal diabetic neuropathy.

Besides distal symmetrical sensory polyneuropathy (DSSP), middle-aged diabetic patients may present with focal or multifocal neuropathies, including proximal neuropathy of the lower limbs, the pathophysiological features of which are uncertain. We studied 10 non-insulin-dependent diabetic patients, 45 to 72 years of age, who developed a painful proximal neuropathy of the lower limbs for which other causes of neuropathy were carefully excluded. The proximal neuropathy was asymmetrical in all patients, sensory in 4, motor and sensory in the others. Signs of DSSP were present in all. A sample of the intermediate cutaneous nerve of the thigh, a sensory branch of the femoral nerve, was taken by biopsy and examined by light and electron microscopy. Examination of the nerve specimens revealed ischemic nerve lesions in 3 patients. Nerve ischemia was associated with vasculitis and inflammatory infiltration in 2 of them. In the other patients the lesions of the cutaneous nerve of the thigh included a varying incidence of axonal and demyelinative lesions similar to those observed in DSSP, with mild inflammatory infiltration in 4 of them. The density of myelinated and of unmyelinated was variably decreased. This study shows that axonal and demyelinative lesions similar to those found in diabetic DSSP are present in proximal nerves in mild forms of proximal diabetic neuropathy; while nerve ischemia, inflammatory infiltration, and vasculitis are encountered in the most severe forms of proximal diabetic neuropathy.

Clinical and electrophysiological study of the peripheral nervous system in the elderly.

The effect of age on the peripheral nervous system was investigated by clinical examination and neurophysiological studies in 59 subjects aged 60-103 years and 23 young subjects. A full laboratory screen for factors which, though clinically silent, may constitute risk factors (RFs) for peripheral neuropathy was also performed in the elderly subjects. Our findings show that the presence of RFs affects exceptionally the electrophysiological parameters in a statistically significant way. The age-dependent changes in nerve conduction parameters were well predicted by non-linear models. The simultaneous electromyographical study demonstrates the re-innervation capacity of the motor system.

[Demyelinating nervous lesions in myalgia-eosinophilia syndrome induced by L Tryptophan]. / Lésions nerveuses démyélinisantes au cours du syndrome myalgies-éosinophilie secondaire à la prise de L-tryptophane.

A case of severe peripheral nerve lesions in a patient with eosinophilia-myalgia syndrome induced by L-Tryptophan is reported. Both superficial radial nerves were very tender on palpation. Biopsy of the radial nerve showed myelin abnormalities of the type observed in experimental chronic nerve entrapment. Nerve lesions included asymmetrical distortion of the myelin sheath, segmental demyelination, axonal degeneration and regeneration. Such abnormalities suggest that mechanical factors, presumably due to proliferation of connective tissue, play a role in the nerve lesions observed in this patient.

Gadolinium-enhanced MRI in central nervous system Behçet's disease.

Two cases of central nervous system Behçet's disease, studied by gadolinium-enhanced MRI, are presented. In one patient, whose clinical picture was dominated by a brain syndrome, the gadolinium enhancement resolved with clinical improvement, although the hyperintense areas in the mesencephalon on T2-weighted images persisted. In the second, who had a pseudobulbar palsy and a mild right hemiparesis, there were many abnormal areas, but an enhancing focus in the posterior limb of the left internal capsule was probably the lesion responsible for the hemiparesis.

[Type II carnitine palmitoyl transferase deficiency complicated by acute respiratory failure]. / Déficit en carnitine palmityl transférase type II complique d'une insuffisance respiratoire aiguë.

Carnitine palmitoyl transferase (CPT) deficiencies can realise distinct clinical presentations. The best known is the muscular form with episodic muscle necrosis and paroxysmal myoglobinuria after prolonged exercise, in young adults, and results from decreased CPT II activity. In this paper, we report on an observation of a patient with a severe CPT II deficiency who presented a respiratory failure during an attack of muscle necrosis. The severity of the symptomatology were associated with a conspicuous reduction of CPT II residual activity in leucocytes and in fibroblasts. Fasting test showed an hypoketogenesis. These results support the concept that CPT II deficiency is ubiquitous, even though injury is restricted to the skeletal muscle.

[Multifocal neuropathy with persistent conduction block]. / Neuropathie multifocale avec blocs de conduction persistants.

Multifocal neuropathy, recently individualized, is characterized by progressive, asymmetrical lesions predominant in the upper limbs and associated with multifocal and persistent conduction block. Two clinical presentations have been identified: sensorimotor multineuritis and pure motor form which may mimick an anterior grey horn disease. The pathophysiology of the syndrome and the significance of its frequent association with the antibody to GM1 ganglioside remain debated. Various treatments have been tried with inconstant results.

[Treatment using an endo-urologic approach of stenoses following uretero-intestinal anastomosis]. / Traitement par voie endo-urologique des sténoses aprés anastomose urétéro-intestinale.

Twenty-one strictures following uretero-digestive anastomoses were treated by percutaneous transrenal dilatation. In 20 cases, an Olbert type angioplasty balloon on a guidewire was used. Rigid coaxial dilators were used in one patient after failure of the preceding technique and an electroincision was performed prior to dilatation in the remaining case. Overall, percutaneous transrenal dilatation was successful in nine patients, whereas ten dilatations failed and two patients are undergoing continued modeling with a mean follow-up of 16 months (range 1-42 months). Success rates by type of anastomosis were as follows: Bricker 5/12; Coffey 1/4; enterocystoplasty 2/4 and ureteroileovesical anastomosis 1/1. The date of development of the stricture, duration of modeling, and caliber of the indwelling catheter were apparently without influence on results. Because morbidity is low with percutaneous transrenal dilatation, this technique is advocated as first-line treatment, with surgery being reserved to failures.

Prevention of the transient adverse effects of a gonadotropin-releasing hormone analogue (buserelin) in metastatic prostatic carcinoma by administration of an antiandrogen (nilutamide).

Gonadotropin-releasing hormone (GnRH) analogues administered for the treatment of advanced prostatic cancer induce a transient increase in plasma testosterone levels during the first week of treatment, often with a secondary rise in plasma levels of prostatic acid phosphatase and a flareup of disease. To determine whether the antiandrogen nilutamide (Anandron) blocks these effects, we carried out a multicenter, placebo-controlled study of nilutamide in men with prostatic cancer treated with the GnRH analogue buserelin. Thirty-six men with disseminated prostatic cancer and elevated plasma levels of prostatic acid phosphatase were randomly assigned to two groups. Group 1 included 17 men who received buserelin (500 micrograms daily subcutaneously) and nilutamide (300 mg daily by mouth); group 2 included 19 men treated with buserelin and placebo. Symptoms were assessed, and plasma was collected before treatment, daily for 14 days, and on days 18, 22, and 29 after the initiation of treatment. Bone pain appeared or worsened in 5 of the 17 men in group 1 and in 12 of the 19 men in group 2 (P less than 0.05). Acute urinary obstruction occurred in one man in group 2. Despite similar changes in the plasma testosterone levels in both groups, the median concentration of plasma prostatic acid phosphatase decreased almost immediately in group 1, but increased transiently, then decreased on day 14 in group 2. Median levels of prostate-specific antigen decreased immediately in group 1 and decreased on day 8 in group 2. We conclude that nilutamide can prevent the adverse consequences of the buserelin-induced transient rise in plasma testosterone levels in men with advanced prostate cancer treated with a GnRH analogue.

[Treatment of urethral stenosis using Olbert's balloon dilatation angioplasty. Re-evaluation of the results]. / Traitement des sténoses urétrales par dilatation au ballon d'angioplastie d'Olbert. Réévaluation des résultats.

Twenty-eight patients with an urethral stricture underwent Olbert's angioplasty balloon dilatation under local anaesthesia and fluoroscopic control. Four dilatations failed. Six were performed palliatively after failure of classical urethral catheterisation. Sixteen dilatations were performed curatively: thirteen patients developed a recurrent stricture after periods of between 3 days and 12 months. Three patients were cured. Our results suggest that angioplasty balloon dilatation is ineffective as a curative procedure and should be reserved for inoperable patients requiring and indwelling catheter, in whom classical catheterisation has failed.

[Early endoscopic treatment of a complete traumatic rupture of the membranous urethra]. / Traitement précoce par voie endoscopique d'une rupture traumatique complète de l'urètre membraneux.

A man presenting complete traumatic disruption of the membranous urethra, with a 1 cm gap between the proximal and distal urethra, underwent successful endoscopic reconstruction ten days after the injury. When the urethral catheter was removed after fifteen days, peak flow rate was 25 cc per second. One internal urethrotomy was necessary 8 months later. Twenty months after the injury, cystography and retrograde urethrography revealed satisfactory restoration of urethral continuity. We suggest that this treatment be considered in complete traumatic disruption without hematoma and with less than a 1 cm gap between the proximal and distal urethra.

[Endoscopically "impassable" urethral stenosis. Catheterization under fluoroscopic guidance. Apropos of 11 cases]. / Sténoses de l'urètre "infranchissables" par voie endoscopique. Cathétérisme sous guidage radioscopique. A propos de onze cas.

In eleven patients with impassable urethral stenoses, we attempted retrograde catheterisation with an angiographic guide under fluoroscopic control. The stenosis was able to be negotiated with the guide in 10 cases (91% success). After dilatation by dilators (coaxial or rigid) or by Olbert's angioplasty balloon, we were able to introduce a Foley catheter into the bladder. The only failure was caused by a very marked separation of the two ends of the urethra due to trauma. This simple and effective method constitutes an alternative to immediate surgical treatment of endoscopically impassable urethral stenoses.

[Technical problems raised by renal puncture in the percutaneous extraction of coralliform calculi]. / Problèmes techniques posés par la ponction du rein dans l'extraction percutanée des calculs coralliformes.

Initial puncture and dilation of nephrostomy track(s) remain difficult procedures in percutaneous extraction of staghorn calculi. Technical s adjuvants are necessary and are exposed in detail. The choice of the needle, of the wires and of the catheters for initial puncture and ureteral catheterization is explained. Techniques of "Y" tract and double puncture are exposed. Severe complications (3 cases) are reported. Extraction of up to 90% of the stone can be expected if these technical adjuvants are optimally used.