Transmission of Creutzfeldt-Jakob disease via a corneal transplant.

A 45 year old woman is reported who initially presented with a cerebellar syndrome, severe ataxia, and dysarthria. She rapidly deteriorated to coma vigile with bilateral myoclonic jerks, flexion rigidity, and immobility necessitating complete nursing. Her EEG showed generalised slow activity and periodic biphasic and triphasic waves. The CSF concentration of neuron specific enolase was very high. Consequently the diagnosis of Creutzfeldt-Jakob disease was established. Eight months later she died of respiratory complications. Thirty years earlier the patient had undergone corneal transplantation for keratoconus. Review of the organ donor's hospital records showed that death was caused by intercurrent pneumonia subsequent to subacute spongiform encephalopathy confirmed by necropsy. In view of two previous case reports in the literature it is presumed that the cadaveric cornea was the source of transmission of Creutzfeldt-Jakob disease in this patient.

An open and double-blind cross-over study on the efficacy of clomipramine (Anafranil) in patients with painful mono- and polyneuropathies.

82 patients with chronic pain resulting from mononeuropathy were treated with psychotropic drugs in an open therapeutic study. In this study we found that treatment with a combination of clomipramine (Anafranil) and small doses of neuroleptics was significantly superior to therapy with neuroleptics alone. In a subsequent double-blind study, it was attempted for the first time to determine the efficacy of clomipramine compared to that of acetylsalicylic acid in 48 patients with painful mono- and polyneuropathies. The test was carried out in a cross-over trial with two sequence groups. We evaluated assessments of pain by both patients and doctors, and were able to prove statistically that clomipramine possesses a significantly greater efficacy compared to that of acetylsalicylic acid. By taking into account recent anatomical, biochemical and pharmacological studies, it can be concluded that clomipramine probably has-in addition to the action on peripheral receptors-a direct effect upon pain modulation systems. It seems possible that clomipramine activates serotonin-containing neurons of the endorphin-mediated analgesia system that control pain transmission in the CNS.

Somatosensory evoked potentials following trigeminal nerve stimulation in trigeminal neuralgia.

The examination of somatosensory evoked potentials following trigeminal nerve stimulation in 20 subjects with idiopathic trigeminal neuralgia revealed a pathological increase in latency of the first positive component on the involved side in 7 (41%) of the 17 patients without antecedent surgical treatment. The mean difference in latency between the two sides was 1.3 msec (2p greater than or equal to 0.005). These results are indicative of microtraumatization of the trigeminal nerve in the posterior fossa by blood vessels, for example, leading to local demyelinization. The method seems to be of diagnostic value in selecting patients for more decompression by posterior fossa operations.

Vitamin B 1, B 2 and B 6 deficiency in neurological disorders.

The activities of the red blood cell enzymes transketolase, glutathione reductase, and glutamic oxaloacetate transaminase were measured with and without in vitro addition of their respective coenzyme components thiamine, riboflavin, and pyridoxine in a group of patients with neurological disorders which may have been caused by malnutrition, intestinal malabsorption, hepatic failure or neoplasms arising outside the nervous system. The incidence of thiamine deficiency was 31%, of riboflavin deficiency 22% and of pyridoxine deficiency 6%. Alcoholics in particular suffered from deficiencies of vitamin B 1, and B 2. There was a correlation of vitamin B 1 and B 2 deficiency and signs of a cerebellar and/or brainstem lesion. The most frequent symptoms in this connection were gait disturbances and oculomotor signs like spontaneous and gaze nystagmus, disturbed eye tracking, diminished optokinetic nystagmus, decreased ability to suppress vestibular nystagmus by fixation. These signs hardly ever occurred in alcoholic patients who showed no deficiency of vitamin B 1, B 2 or B 6. Whenever they do appear, a vitamin B supplementation has to be performed in order to prevent the manifestation of Wernicke's encephalopathy, cerebral or cerebellar atrophy. Alcoholics showed the same incidence of polyneuropathy, whether they suffered from a deficiency of B vitamins or not. Deficiencies of vitamin B 1, B 2 or B 6 were also found in patients with intestinal malabsorption and polyneuropathy, diabetic polyneuropathy, optic atrophy, myelopathy and cerebellar ataxia of unknown etiology, neurological manifestations of neoplasms arising outside the nervous system, B 12 myeloencephalopathy and Thévenard's syndrome.

[Teratogenicity of anticonvulsant drugs (author's transl)]. / Zur Teratogenität von Antikonvulsiva.

Investigations were done on 111 children of epileptic mothers who used anticonvulsants in 93 pregnancies and none in 18 pregnancies. Hydantoinbarbiturate embryopathy was found in 7.1% after hydantoin monotherapy, in 17.6% after combination of hydantoin and barbiturates or primidone. No embryopathy was seen in children of untreated epileptic mothers. Children of untreated and treated epileptic mothers had an approximately equal frequency of marked single malformations and cerebral damage without dysmorphia. However, malformation and cerebral damage without dysmorphia was found significantly more frequently in children of mothers on anticonvulsant drugs with convulsions during pregnancy as compared to children of mothers without convulsions. Single manifestations and cerebral damage without dysmorphia are probably not caused by anticonvulsants but by convulsions during pregnancy.

Somatosensory evoked potentials following stimulation of the trigeminal nerve in man.

Somatosensory evoked potentials following trigeminal nerve stimulation can regularly be recorded from the contralateral scalp on C5/C6 (10--20 system), a region which overlies the primary face region of the somatosensory cortex. From the first three peaks analyzed (N 13, P 19 and N 26), the first positive peak (P 19) is most prominent and reliable and therefore is recommended for the routine measurements of neurophysiological examination.

Comparison of phenytoin determinations in plasma, plasma dialysate and saliva for control of antiepileptic therapy in children.

Phenytoin was determined in plasma or serum, dialysate from plasma or serum and saliva in 45 children and 7 adults by gas chromatography. 200 microliter of plasma were necessary for dialysis. Close correlations were found between phenytoin concentrations in the different media (r greater than or equal to 0.95). Since in some cases the levels in saliva were extremely high in comparison to plasma and dialysate, precautions are advisable when using phenytoin determinations in saliva for therapeutic decisions. Clinical data from 15 children with high phenytoin levels suggest that there is no advantage in determining the unbound fraction of the drug in plasma for the control of antiepileptic therapy.

Retrograde changes of nerve fibers with the carpal tunnel syndrome. An electroneurographic investigation.

In the CTS the motor nerve conduction velocity proximal to the wrist is reduced in proportion to the degree of severity of the nerve lesion. Furthermore the evoked nerve action potential is significantly reduced when recordings are made from the median nerve at the elbow and when the compound nerve is stimulated proximal to the lesion at the wrist. The extent of the retrograde changes correlates with the degree of severity and duration of nerve compression. Measurement of the evoked nerve action potential in the proximal nerve segment enables us to estimate the extent of the retrograde nerve fiber degeneration and therefore might be important for prognosis.

[Brachial plexus paralysis after stellate blockade and plexus anaesthesia (author's transl)]. / Armplexusparesen nach Stellatumblockade und Plexusanästhesia.

Brachial plexus paralysis is a serious complication of stellate blockade (n = 2) and plexus anaesthesia (n = 6) as was observed in 8 patients. It is frequently characterised by localised motor and sensory defects in the affected arm and sometimes accompanied by a causalgia-like pain. Pathogenetically mechanical factors--needle trauma, injection pressure, volume, and velocity--are of decisive importance. Prophylactically intraneural injections must be avoided. The electrifying pain during insertion of the needle and (or) immediate anaesthetic effect require correction of needle position.

[The ultimate electromyographic patterns in reinnervated muscle (author's transl)]. / Elektromyographische Spätbefunde nach Reinnervation

We investigated 59 patients in later stages after section and suture (n =23) or transplantation (n = 36) of the ulnar or median nerve. The most typical change in electromyographic pattern consists in a markedly increase of mean amplitude, due to an increased number and concentration of muslce fibers in the reconstituted motor-units. The mean duration and the incidence of polyphasic potentials are only slightly increased, indicating a nearly synchronous excitement of the different muscle fibers of the various motor-units. In no case there is a complete recovery; the mean amplitude of evoked potentials in reinnervated muscles varied from 32% in poor-, to 62% in satisfactory reinnervation, compared with the corresponding normal muscle in the opposite hand.

[Changes in drug elimination under the influence of perazine therapy (author's transl)]. / Anderung des Arzneimittelabbaus in der Leber unter der Behandlung mit Perazin

In 8 male patients who were treated with perazine for a schizophrenic psychosis (200-800 mg/die), the elimination rate of phenazone was investigated. Simultaneously determinations of plasma levels of perazine and desmethylperazine were carried out. The average half-life of phenazone was 27.0 h in perazine-treated patients and 11.2 h in controls. Correspondingly, the clearance of phenazone decreased from 47.0 ml/min to 18.9 ml/min under perazine, both differences being highly significant. The amount of 4-OH-phenazone, the principal hydroxylated metabolite excreted in the urine, was 66 mg/24 h in the perazine group, and significantly different from the results obtained in the control group: 185 mg/24 h. In contrast the urinary excretion of the unchanged phenazone increased from 29 to 40 mg/24 h under perazine. The results are interpreted to demonstrate inhibition of drug hydroxylation in the liver by perazine treatment.

[Thin-layer chromatographic determination of plasma levels of tricyclic psychopharmacological drugs: first results on their relationship to the clinical activity of neuroleptics (author's transl)]. / Dünnschichtchromatographische Bestimmung von Plasmaspiegeln tricyclischer Psychopharmaka: Erste Ergebnisse über eine Beziehung zur klinischen Wirkung von Neuroleptika

Plasma levels of perazine, clozapine, amitriptyline and imipramine and of their demethylated metabolites can be measured in patients receiving therapeutic doses by UV reflectance photometry of thin-layer chromatograms of plasma extracts, Large inter-individual variations were observed in unselected psychiatric patients treated with comparable doses. An investigation into the relationship between plasma levels and therapeutc effect in acutely schizophrenic patients was carried out for perazine and clozapine. With perazine, a group of patients exhibiting an unsatisfactory response had a tendency to show lower plasma levels than a group with a good response; in some patients of the former group, increase of the dose with concomitant increase of the plasma level led to a satisfactory therapeutic effect. In patients treated with clozapine, such a relationship could not be demonstrated.