Biomedical subjects
Publications and source records attributed to P Berlit.
Miller Fisher's syndrome is characterized by the symptomtrias of ophtalmoplegia, ataxia and areflexia. Five cases of Miller Fisher syndrome are presented.; the nosological position of this disorder is reviewed. Involvement of brainstem is present in some cases--but obviously facultative.
We report findings of magnetic resonance imaging (MRI) in three patients with spinal cord ischemia. While myelography was normal in all three patients, MRI found pathological signal increase on the T2-weighted images in each case. Signal changes in T1-weighted images of hemorrhagic infarction were seen in one patient, and gadolinium-DTPA enhancement because of a disturbed blood-tissue barrier was noted in another. Motor evoked potentials after transcortical magnetic stimulation showed normal latencies with very low amplitudes as a sign of axonal loss in ischemia of the spinal cord.
Twenty patients with different types of muscular dystrophy (MD) were included in a cross-sectional study by means of electrocardiography and ultrasound cardiography. A manifest cardiomyopathy was detected in 8 patients; a latent cardiomyopathy was found in 4. A hypertrophic cardiomyopathy was especially frequent in facioscapulohumeral MD, a congestive cardiomyopathy in Becker-Kiener MD. The ECG showed a reduction in the QT interval and frequent block formers in the X-chromosomal inherited forms and the trunc-girdle form. Bradycardia and a prolonged QT interval were frequent in myotonic dystrophy and facioscapulohumeral MD. Signs of cardiac infarction in the ECG were most frequent in the trunc-girdle forms. A high cardiac output per minute in conjunction with increased left ventricular volume was frequent in Becker-Kiener and Landouzy MD. A left ventricular dysfunction with reduced ejection was characteristic of myotonic dystrophy and trunc-girdle MD. A mitral valve prolapse was more frequent with increasing severity of the muscle disease and was particularly frequent in myotonic dystrophic and Landouzy MD. The cardiac output per minute and the stroke volume were significantly lower (P less than or equal to 0.03) where a mitral valve prolapse was present.
We evaluated the hospital records of 412 patients with isolated or combined ocular nerve palsies in a retrospective study. Palsies of the oculomotor nerve (n = 172) and of the abducens nerve (n = 165) were more frequent than those of the trochlear nerve (n = 25). Combined ocular nerve palsies (n = 50) were generally combinations of the 3rd and 6th cranial nerves (n = 21) or pareses of all three ocular nerves (n = 17). 165 ocular nerve palsies were due to vascular causes: in 135 of these cases diabetes mellitus and hypertension were present. The oculomotor nerve was most frequently affected; in 63% there was no involvement of the pupil. In inflammatory disease and brain tumor the abducens nerve was most frequently affected, with aneurysm of the oculomotor nerve. The origin of ophthalmoplegia was unclear in 73 patients. Ocular nerve paralysis was most common with tumors, aneurysm, and vascular processes and in 206 cases was only partial. Pain was associated with tumor, trauma and aneurysm. In trochlear nerve palsies concomitant pain was much less frequent than in palsies of the other two ocular nerves. The clinical course was followed for 3 weeks in 352 patients; in 191 patients there was a complete regression of the pareses and in 59 only a partial recovery. The most favorable prognosis was with inflammatory and vascular lesions; in the latter the outcome was improved by the administration of non-steroidal anti-inflammatory drugs.
An overview is given over etiology and prognosis of cerebral ischemias until the age of 40. In a time period of 19 years, 168 patients were diagnosed with cerebral ischemia until the age of 40 (91 females, 77 males). The most frequent etiology is premature atherosclerosis in patients with vascular risk factors (up to 50%). Cardiogenic embolism is responsible for 1 to 34% of the cases: cardiac valve diseases and endocarditis being the most frequent sources. In 2 to 19% a vasculitis is diagnosed. While infectious arteritis is especially frequent in countries of the third world, immunovasculitides are common in Europe and the USA. Noninflammatory vasculopathies include spontaneous or traumatic dissection, fibromuscular dysplasia and vascular malformations. A migrainous stroke is especially frequent in female smokers with intake of oral contraceptives. During pregnancy both sinus thrombosis and arterial ischemia occur. Hematologic causes for ischemia are polycythemia, thrombocytosis and genetic diseases (sickle cell anemia, AT3-deficiency). Cerebral ischemia may occur in connection with the ingestion of ergot-derivates. The prognosis of cerebral ischemia in young adults is better than in older stroke-patients.
We report on 7 patients (2 women, 5 men) with chronic renal failure, who developed under a high dosage of the new diuretic muzolimine (range 240 to 1440 mg per day) fatal neuromyeloencephalopathy. Clinical neurophysiological and neuroradiological findings and finally neuropathological studies in 2 patients resembled those found in vitamin-B12-deficiency-syndrome with a predominant affection of the spinal posterior column and the corticospinal tracts. The first neurological symptoms like paraesthesia, severe hyperpathia of the legs and mild to heavy spinal ataxia occurred after an average time of treatment of 78 days and a mean dosage of 52 g. The most progressive neurological deficits like severe tetraspastic paresis, were seen only in the nondialytic renal insufficient group (3 patients), while the others had a more benign course of the disease. This lead to the hypothesis of a partially dialysable toxic metabolite of muzolimine. After a follow-up of more than 2 1/2 years no significant recovery was seen in these cases.
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We report the case of a patient with Brown-Séquard syndrome resulting from Borrelia burgdorferi infection. Antibiotic treatment led to complete recovery.
The case of a patient with a combined paresis of the peroneal and posterior tibial nerve caused by a phlegmone of the upper thigh is reported. The pathologic-histological findings after leg amputation are described.
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We report on 7 patients suffering from chronic renal failure (2 females, 5 males; aged 35-75 (phi 53.5) years) who showed severe neuromyeloencephalopathy (NME) after high doses of a new Henle's loop diuretic, Muzolimine. The temporal and phenomenological development of these systems was strikingly parallel. The neurological deficit was revealed on neurophysiological, neuroradiological and in 2 cases on neuropathological tests (gross demyelinisation of the posterior column, mainly of the fasciculus gracilis, less in the lateral corticospinal tract and in some spinal roots). The critical drug dose for first neurological impairment was 52 g on average; at this point the patients had been treated for 78 days. The maximal daily dose was 1.440 mg. Dominant clinical features were pallhypaesthesia, ataxia, signs of peripheral neuropathy in combination with hyperreflexia and progressive para- to tetraspastic paresis. Constellation of symptoms, course of disease and findings of additional investigations, especially those of neuropathology, very much resemble Vitamin B12 deficiency and SMON-(Subacute Myelo Optic Neuropathy) syndrome. The rare entity of Muzolimine-NME is discussed in respect to other endogenous and exotoxic neuromyelopathies. We present the hypothesis of a toxic, partially dialysable metabolite of Muzolimine.
In 9 patients with the clinical diagnosis of brain death, a HM-PAO-SPECT was performed to document the arrest of cerebral circulation. Besides brain-stem areflexia, negative apnea-test and an observation time of at least 12 hours neurophysiological examinations (EEG and evoked potentials) were performed. In 4 cases, an additional arterial digital subtraction angiography proved the arrest of cerebral circulation shown in SPECT. In 5 cases, there was cortical low activity still present in EEG with negative SPECT. HM-PAO-SPECT seems to be a reliable noninvasive radiological method in the determination of brain death. It is especially helpful in cases with persisting focal cortical EEG-activity.
Motor and sensory nerve conduction velocity (NCV) of median nerve and motor NCV of peroneal nerve were measured in 548 epileptic patients, aged 20 to 40 years, under long term treatment with antiepileptic drugs. Compared with a control collective of 70 healthy persons in the same age the epileptics showed a reduction of all NCVs. 19 percent of the epileptic collective had at least 1 diminished NCV. Negative correlations with total amount and average daily dose of antiepileptic drugs were found in both sexes for sensory NCV of median nerve and motor NCV of peroneal nerve, only in males also for motor NCV of median nerve. Only in females, duration of therapy correlated negative with sensory NCV of median and motor NCV of peroneal nerve. In patients under monotherapy the group with carbamazepine treatment showed the lowest NCV values, the difference being significant for motor NCV of median nerve in comparison to phenytoin and valproate sodium. It is concluded, that NCV generally is lowered under long term treatment with antiepileptic drugs and that phenytoin has no specific influence compared with the other drugs.
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An overview is given on the use of immunoglobulins in clinical neurology. While 5S-immunoglobulins may be employed in addition to virostatics in viral encephalitis, 7S-immunoglobulins can be used in autoimmune diseases like myasthenia gravis, multiple sclerosis, and the Guillain-Barré syndrome. Refractory childhood epilepsies like the Lennox-Gastaut syndrome responded to 7S-immunoglobulins. Hyperimmunoglobulins are to be given in bacterial infections in which toxins are formed and in viral infections caused by cytomegalovirus and tick-borne encephalitis virus. While some open studies report benefit from the use of immunoglobulins in neurological diseases, controlled evidence for their efficacy is still missing.
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