Sweet's syndrome (acute febrile neutrophilic dermatosis) affecting the central nervous system.
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Aged↗
Biomedical subjects
D Claus
Publications and source records attributed to D Claus.
[Immunoglobulin therapy of chronic inflammatory neuropathies].
High-dose intravenous immunoglobulins (ivIg) are an effective therapy in chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). In both diseases, data regarding ivIg long-term treatment are sparse. Therapy with ivIg was performed in 18 patients with CIDP or MMN. Sixteen patients responded to ivIg therapy; they were treated for more than 6 months. Two of them had not previously shown any positive response to other immunosuppressive treatments. Response to ivIg therapy could be observed even after a long disease duration (maximum of 19.5 years). All 16 therapy responders now have no or only mild clinical symptoms. Treatment could eventually be completely stopped in 6 patients; they have now been in complete remission without therapy for a maximum of 63 months. Ten patients still receive regular ivIg infusions in different dosages. No severe side effects were observed in the whole group. IvIg therapy is an effective, safe and easily applicable treatment regimen even in the long-term course of CIDP and MMN.
Adolescent↗
Focal upper limb demyelinating neuropathy.
Observations are presented on nine selected patients with chronic upper limb demyelinating neuropathy to illustrate the range of manifestations that may be observed. In three, the involvement was purely motor, in five, mixed motor and sensory and, in one, virtually purely sensory; in seven the symptoms were unilateral and in two bilateral. The presence of reduced nerve conduction velocity and conduction block and the response to treatment in seven of the cases indicate that they represented examples of chronic inflammatory demyelinating polyneuropathy (CIDP) with focal involvement. This was confirmed by nerve biopsy in two cases. The presentation in one patient was accompanied by forearm swelling initially suspected of being a tumour but shown to be due to muscle hypertrophy. This was probably the consequence of recurrent muscle cramps and fasciculation and possibly neuromyotonia. The patient with predominant sensory involvement restricted to the upper limbs demonstrates that sensory CIDP can present focally. In one patient with monomelic motor and sensory involvement, nerve biopsy showed multifocal areas of hypertrophic demyelinating neuropathy distally in the ulnar nerve without inflammatory infiltration. This patient failed to respond to therapy. Response in the others was satisfactory, although one patient with a monomelic motor neuropathy showed a severe deterioration after being given corticosteroids; he subsequently improved with intravenous human immunoglobulin therapy.
Adolescent↗
A new familial short stature syndrome: Brussels type.
A new association of congenital familial short stature with facial dysmorphism and osteochondrodysplastic lesions is described in two siblings. Clinical abnormalities include severe prenatal and postnatal growth failure and facial dysmorphism. Radiographs show osteochondrodysplastic lesions with a narrow thorax, short ribs, epiphyseal maturation delay and slightly deformed metaphyses. Microscopic analysis of the skeleton shows pathological features.
Body Height↗
Large left ventricular thrombus in a patient with Friedreich's ataxia.
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Adult↗
Identifying delirious states and autonomic cardiovascular dysfunction associated with amitriptyline treatment by standardized analysis of heart rate.
Ninety-one patients treated with amitriptyline and 60 normal control subjects underwent a standardized heart rate analysis in supine posture. Tests included the determination of time- and frequency-derived measurements of heart rate variability. The patients differed significantly from the control subjects in all parameters investigated. Two-thirds of the patients treated with a tricyclic antidepressant (TCA) reached values that met the criteria for cardiovascular autonomic neuropathy. Our results provide evidence that heart rate analysis might be a more sensitive tool in diagnosing amitriptyline-associated anticholinergic delirium than determination of TCA plasma levels. Further research is needed to elucidate what implications the TCA-associated alteration of autonomic cardiovascular function might have for patients.
Adult↗
Prediction of response to total sleep deprivation by autonomic heart rate parameters?
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Adult↗
Botulinum toxin: influence on respiratory heart rate variation.
Remote adverse effects of local intramuscular botulinum toxin were investigated in a prospective follow-up study. Twenty-six patients with spasmodic torticollis were examined (18 women, eight men, 45 +/- 13 years). Respiratory heart rate variation (HRV) was investigated by a computerized method. Different parameters were recorded (beats per minute, coefficient of variation, root mean square successive difference (RMSSD), spectral analysis, difference and quotient between maximum and minimum RR intervals, mean circular resultant). After one intramuscular injection of 12.5 ng botulinum toxin (Porton Products Ltd., England), no significant influence on HRV was seen. After the second injection, a significant attenuation was seen of four parameters (coefficient of variation, Rmax - Rmin, Rmax divided by Rmin, mean circular resultant) that lasted up to several months. No clinically manifest remote side effects and no cardiac arrhythmia were seen for several months of botulinum toxin treatment. Our investigation proves an effect of local intramuscular botulinum toxin on autonomic cardiac innervation.
Adult↗
Function of thick and thin nerve fibers in carpal tunnel syndrome before and after surgical treatment.
Present studies evaluated the functions of thick and thin nerve fibers in carpal tunnel syndrome (CTS) to determine whether data of thin nerve fibers can add further information to the diagnosis of CTS. Measurements were performed in 22 patients and 16 age-matched control subjects. Motor and sensory nerve conduction, vibratory testing, thresholds of warmth and cold sensations, suprathreshold magnitude of pain and flare response, as well as sympathetic reflexes induced by noxious mechanostimulation were examined before and 1, 3, 6, 12, and 18 months after decompression of the median nerve. Nerve dysfunction in CTS was mainly indicated by the electrophysiological parameters of thick myelinated nerve fibers. Impairment of thin nerve functions in CTS and postoperative recovery was demonstrated by quantitative somatosensory thermotesting. In contrast, assessment of flare response and sympathetic reflexes using infrared thermography and photoplethysmography did not add further information for the diagnosis of CTS.
Adult↗
Myotonic dystrophy: correlation of clinical symptoms with the size of the CTG trinucleotide repeat.
An unstable DNA sequence of a gene encoding a protein kinase has been identified as the molecular basis of myotonic dystrophy. The correlation between different symptoms of myotonic dystrophy and the size of this unstable base triplet (CTG)n repeat was investigated in 14 patients. DNA was prepared from whole blood by standard procedures. Detailed clinical, psychological, electrophysiological (quantified measurement of myotonia, electrocardiography) and other laboratory examinations (muscle biopsy in 4 patients, slit lamp examination) were performed. Triplet size correlated significantly with muscular disability and inversely with age at onset of the disease. A greater frequency of mental and gonadal dysfunction could be observed in patients with a larger repeat size. Other symptoms, however, such as cataract, myotonia, gastrointestinal dysfunction and cardiac abnormalities were not correlated with repeat size. Somatic mosaicism with different amplification rates in various tissues might be one possible explanation for the variable phenotypes. Furthermore, other factors such as different expression of the myotonic dystrophy gene might contribute to the clinical variability of the disease at a given triplet size.
Adult↗
Ascending central nervous spreading of a spinal astrocytoma.
A 43-year-old man died from the complications of astrocytoma metastasis. He first noticed symptoms of a lumbar disc prolapse in 1979. In 1987 a pilocytic astrocytoma (grade I) of the spinal cauda was removed. In 1989 a tumor recidivation at the same site was partially removed. Histology showed a grade II astrocytoma. Two months later the patient developed symptoms of increased intracerebral pressure. CSF cytology showed polymorphic giant tumor cells with hyperchromatic nuclei and a glioblastoma of the cerebral ventricles was diagnosed. The patient died from cardiovascular complications. The post-mortem investigation revealed an astrocytoma of the conus medullaris with an anaplastic ventral area (grade IV). This area was inaccessible to the biopsy. It is believed that tumor metastases from anaplastic parts spread along the spinal cord and brainstem and finally invaded the brain and cerebral ventricles.
Adult↗
Transcranial magnetic stimulation as a diagnostic and prognostic test in amyotrophic lateral sclerosis.
Corticospinal stimulus conduction was investigated after transcranial magnetic stimulation of the motor cortex in 63 patients (20 female, 43 male, 59 +/- 12 years) with amyotrophic lateral sclerosis (ALS) and progressive bulbar palsy. Recordings were made bilaterally from the Abductor digiti minimi muscle (ADM) in the hand and the Tibialis anterior muscle (TA) in the leg. Thirteen patients were re-examined after 250 +/- 125 days. Eight patients were examined a third time after 552 +/- 165 days. At the first investigation central motor conduction time was abnormal to one or more target muscles in 51% (n = 32) of all patients. No significant delay in CMCT developed during follow-up. The average time of survival of patients with normal CMCT at the first investigation was 16.5 +/- 7.5 months, and 14.7 +/- 8.8 months in patients with abnormal CMCT. This is not a significant difference. It is therefore concluded that transcranial magnetic stimulation is not a sensitive tool in the diagnosis of ALS. Furthermore, CMCT does not provide significant prognostic information.
Adolescent↗
Parameters of thick and thin nerve-fiber functions as predictors of pain in carpal tunnel syndrome.
Pain intensity in carpal tunnel syndrome (CTS) was correlated with neuro- and psychophysiological parameters related to the function of different nerve fiber classes within the median nerve in 23 patients. Control data were obtained from 16 normal subjects. Mean intensity of all pain attacks which occurred 14 days before surgical treatment was assessed on visual analogue scales (average CTS pain). Functions of thick myelinated nerve fibers were determined by motor and sensory nerve conduction studies. Functions of thin myelinated and unmyelinated nerve fibers were evaluated by measuring thresholds of warmth, cold and heat pain on the index and little finger. Pain intensity and neurogenic vasodilatation following noxious mechano-stimulation on the interdigital web between index and middle finger provided additional information on the functioning of nociceptive nerve fibers. Sympathetic reflexes induced by these painful stimuli were assessed by means of infrared thermography and photoplethysmography. Mean intensity of pain attacks (40 +/- 19% VAS) correlated significantly with latency (r = 0.58, P < 0.01) and amplitude (r = -0.50, P < 0.01) of the compound action potential from abductor pollicis brevis muscle following distal median nerve stimulation. Thresholds of warmth, cold and heat pain on index finger were significantly increased during CTS when compared to the control subjects. The magnitude of neurogenic vasodilatation and sympathetic vasoconstrictor reflexes were not significantly different. Average CTS pain correlated inversely to the threshold of heat pain on index (r = -0.46, P < 0.05), but also on the little finger (r = -0.41, P < 0.05), which is not innervated by the median nerve.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Prolonged dysphagia caused by congenital pharyngeal dysfunction.
We describe two patients with severe, isolated, congenital dysphagia caused by paralysis of the pharyngeal muscles, who recovered at the ages of 40 months and 20 months, respectively. No other evidence of neurologic or muscular dysfunction was present except for a transient paralysis of the adductors of the vocal cords in one child. Radiocinematographic studies showed paralysis of the pharyngeal stage of swallowing, with minimal involvement of the oral stage. One child refused oral feeding for several months after apparent radiologic recovery. Two other patients with a similar disorder died of tracheal aspiration at the ages of 8 months and 4 months, respectively. Autopsies showed no abnormality of the central nervous system, and the cranial nerves involved in swallowing were normal. Only five other well-studied cases of this syndrome have been reported. These observations demonstrate the existence of a type of severe, idiopathic, congenital dysphagia related to paralysis of the constrictor muscles of the pharynx, with a propensity to recover after several months or years if properly managed. The cause of the disorder is obscure, but it is probably related to a dysfunction of the central nervous system.
Child, Preschool↗
[Improvement of temperature and vibration sense in chronic uremia after a single dialysis].
Pathophysiology and pathoanatomy of uremic neuropathy are not yet well understood. A single hemodialysis positively increases nerve conduction velocities of uremic patients, thus demonstrating a functional A alpha-fiber improvement by detoxification. This study tested whether non-invasive Vibrameter and Thermotest studies show a similarly positive effect for A beta-, A delta- and C-fibers and whether the psychophysical techniques might substitute for nerve conduction studies. 20 uremic patients depending on chronic intermittent hemodialysis were examined shortly before and after a hemodialysis. Using a scaled 128 Hz tuning fork, a Vibrameter and a "Marstock"-Thermotest, vibratory and warm and cold thresholds were assessed at both internal malleoli according to the method of limits. In addition, thermal thresholds were determined at the volar aspect of the non-shunted wrist. In nine patients the Vibrameter showed elevated thresholds before and after dialysis as did six patients with the tuning fork. Warm or cold thresholds were abnormal in four patients before treatment and in eight patients after dialysis. This was due to some patients reporting elevated thresholds after dialysis although they had had normal thresholds before the treatment. Still, the overall sum of abnormal thermal or vibratory thresholds at the different tested body sites had decreased after treatment. Moreover, mean values of thermal and vibratory thresholds of the 20 patients improved with dialysis (p < 0.05). Tuning fork results were too coarse and failed to show a dialysis effect.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Stimulation of sudomotor axon reflex mechanism by carbachol in healthy subjects and patients suffering from diabetic polyneuropathy.
Sudomotor axon reflex (SAR) mechanism was stimulated by percutaneous iontophoresis of carbachol in healthy subjects and patients suffering from diabetic polyneuropathy (PNP) of differing severity (stage I-III). SAR response was assessed by hygrometry and compared with histamine induced neurogenic vasodilation (laser-Doppler flowmetry) and itching sensation (visual analogue scale), which may provide information on neuro-secretion and afferent function of nociceptive unmyelinated nerve fibers. Carbachol induced long lasting SAR response with maximal sweating rates 20-30 minutes after stimulus onset. In diabetic patients the carbachol induced SAR response and the histamine induced itching sensation were significantly reduced in stage III of PNP. In contrast, histamine induced vasodilatation was significantly impaired in all stages of PNP. It is concluded that prolonged SAR response following iontophoresis of carbachol can simplify evaluation of sudomotor function when compared to established tests using intradermal application of acetylcholine. Furthermore, to demonstrate impairment of unmyelinated nerve fibers in PNP, histamine induced vasodilatation was found to be more sensitive than SAR response.
Adult↗
Thermal threshold determination in alcoholic polyneuropathy: an improvement of diagnosis.
Reports on the incidence of alcoholic polyneuropathies are variable depending on diagnostic tools. In this study, 50 chronic alcoholics with positive MALT (Munich Alcoholism Test) and greater than seven years history of excessive alcohol abuse were examined neurologically. Tibial and peroneal motor and sural nerve conduction velocities (NCV) were studied. Warm and cold perception was evaluated in the area behind the internal malleolus using a Somedic-Thermotest. Thresholds were determined by the method of limits. The effect of a slow, medium and fast temperature change rate on thermal perception was tested. Thirty-eight patients (76%) showed signs of neuropathy. Thermal perception was more often abnormal (62%) than NCV (42%) and clinical examination (56%). A medium temperature change rate of 2.0-2.5 degrees C/s was the most sensitive index of small fiber neuropathy. Thermal threshold measurement proved to be a reliable, sensitive and easy to perform method that should become standard in the examination of polyneuropathies.
Adult↗
[Primary tumor calcification in a case of Burkitt's lymphoma].
A case of abdominal Burkitt lymphoma in a 8-year-old boy is reported. The authors discuss a very unusual feature of this untreated lesion, intratumoral calcification remaining unchanged after 4 years of follow-up.
Antineoplastic Combined Chemotherapy Protocols↗