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Biomedical subjects

J G Heckmann

Publications and source records attributed to J G Heckmann.

At least 55 records · Page 3Linked to original sources

[Cerebral tuberculosis in a patient with Sharp's syndrome].

HISTORY AND ADMISSION DIAGNOSIS: A 57-year-old female patient with fever and impaired consciousness was admitted to the department of neurology after her first epileptic seizure. She had a 9-year history of mixed connective tissue disease (MCTD, Sharp's syndrome) predominantly presenting with pulmonary symptoms and destructive arthritis. Endoprothetic surgery had to be performed several times and she was given long-term immunosuppressive therapy. INVESTIGATIONS: Cerebrospinal fluid analysis showed pleocytosis (59/mm3), high protein concentration (2540 mg/l) and low glucose level (31 mg/dl) compared to blood glucose level (122 mg/dl) the indicating possible tuberculous meningoencephalitis. DIAGNOSIS, TREATMENT AND COURSE: Tuberculostatic therapy was initiated, but despite extensive testing Mycobacterium tuberculosis could initially not be detected by microscopy, culture or amplification techniques (TMA; transcription mediated amplification). Clinical response to antituberculous therapy was poor and the patient developed cerebral ischaemia and hydrocephalus. Because of earlier histological findings from the synovialectomy showing epitheloid cell granuloma a knee joint specimen from a wound drainage was tested and extracerebral tuberculosis was finally confirmed by mycobacterial culture so that tuberculosis as the reason for the meningoencephalitis became highly probable. Despite slight improvements the patient still had hemiparesis and lethargy as neurological sequalae at the end of therapy. CONCLUSION: The case demonstrates the difficulties in the diagnosis of tuberculosis in patients with signs and symptoms similar for those caused by other multisystemic diseases. When tuberculous meningitis is considered, therapy should be initiated even in cases with negative microbiological tests because of severe consequences when treatment is delayed.

Brain Ischemia↗

Oral mucosal blood flow in patients with burning mouth syndrome.

The pathophysiology of burning mouth syndrome (BMS) is largely unknown. Thus, the aim was to study oral mucosal blood flow in BMS-patients using laser Doppler flowmetry (LDF). Thirteen BMS patients (11 female, two male; mean age+/-SD 64.3+/-7.9 years, mean disease duration 18.9+/-6.2 months) and 13 healthy non-smoking controls matched for age and gender (11 female, two male; mean age 64.7+/-8.1 years) were investigated. Using the LDF technique mucosal blood flow (mBF) was measured at the hard palate, the tip of the tongue, on the midline of the oral vestibule, and on the lip. Measurements were made at rest and over 2 min following dry ice application of 10 s duration using a pencil shaped apparatus. In addition, blood pressure (BP), heart rate (HR), peripheral cutaneous blood flow, and transcutaneous pCO(2) were continuously recorded. Mucosal blood flow (mBF) increased at all measurement sites in response to dry ice application (P<0.001) with peak flow at 0.5--1.5 min after stimulation onset. During the following 1.5--2 min, blood flow decreased at all sites with a tendency to return to baseline towards the end of the observation period. Except for BP and peripheral blood flow, all of the cardiovascular changes exhibited significant changes during the observation period; no differences between groups were detected. When compared to healthy controls BMS patients generally exhibited larger changes in mBF. These changes were significant for recordings made on the hard palate (F[1,24]=13.9, P<0.001). Dry ice stimulation appears to be an effective, non-invasive and reasonably tolerable means to investigate mucosal blood flow at different mucosal sites. In general, vasoreactivity in BMS patients was higher than in healthy controls. BMS patients exhibited a higher response on the hard palate compared to controls. These changes in oral blood flow appear to be specifically related to BMS symptoms indicating a disturbed vasoreactivity.

Aged↗

Cerebral vein thrombosis and prothrombin gene (G20210A) mutation.

Recently, prothrombin gene mutation G20210A has been associated with elevated thrombosis risk and rarely with cerebral vein thrombosis (CVT). Three patients are described who had this genetic predisposition and who developed CVT in an unusual constellation with other factors. In the first patient, the intake of valproic acid (VPA) may have played an aggravating role in the development of CVT; in the second patient diagnosis of coagulation disorder was made during pregnancy consultation 6 years after CVT; in the third patient the CVT occurred at the age of 78 years. In patients with CVT, coagulation-examinations should include tests for the prothrombin gene (G20210A) mutation.

Adult↗

Disease latency in Creutzfeldt-Jakob disease via dural grafting: a case report.

The case of a 57-year old man is reported who had been operated upon for cerebellar angioblastoma more than 19 years previously. Having received a lyophilized dural patch he developed Creutzfeldt-Jakob disease of which he died 19 months later. This is the longest latency with dura-associated Creutzfeldt-Jakob disease reported so far.

Cerebellar Neoplasms↗

Transcranial doppler sonography-ergometer test for the non-invasive assessment of cerebrovascular autoregulation in humans.

Cerebrovascular hemodynamics during physical stress have been sparsely investigated, mostly through risky invasive techniques. The aim of this study was to determine the effect of ergometer stress on cerebrovascular hemodynamics in humans using the non-invasive and thus clinically-applicable method of transcranial Doppler sonography (TCD) combined with simultaneous non-invasive measurements of cardiovascular parameters. In eighteen healthy subjects (six women, twelve men; 29.3+/-4.6 years old) left midcerebral artery blood flow velocities (CBFVs) were continuously monitored using TCD during 3 min at rest, 3 min during ergometry and 3 min recovery. Simultaneously, systolic, diastolic, mean CBFVs, pulsatility index (PI), heart rate, beat-to-beat blood pressure (BP) and transcutaneous p(CO(2)) were measured. The subjects were supine with elevated trunk. Ergometry was performed by pedalling a Mühe-ergometer. In eight volunteers, the procedure was repeated within the next day to test the repeatability of the results. Heart rate increased significantly during ergometry (from 65.2+/-11 to 105. 3+/-12.3/min; P<0.05). The systolic BP increased significantly slightly later during ergometry (from 118.9+/-8.6 to 141.6+17.9 mmHg; P<0.05). Transcutaneous p(CO(2)) was initially within physiological ranges, but increased significantly after a delay during the 3rd min of cycling (from 39.7+/-3.7 to 41.1+/-4.7 mmHg; P<0.05). MFV started to rise significantly after 1 min of the exercise period (from 59.6+10.9 to 68.3+13.9 cm/s; P<0.05). PI increased immediately and significantly at the start of exercise (PI at rest 0.93+0.11; PI ergometry 1.1+0.13; P<0.05). The results were found to be reproducible in the eight volunteers. The cerebrovascular changes during ergometer exercise may reflect the combined activation of the cerebrovascular autoregulative mechanisms (neurogenic, myogenic and metabolic). The TCD-ergometer test presented here is non-invasive and would seem to present a low risk for patients who are judged fit enough for mild exercise. The test may contribute to the detection of cerebrovascular abnormalities in various diseases.

Adult↗

Hypoglycemic sensorimotor polyneuropathy associated with insulinoma.

Hypoglycemia-induced peripheral neuropathy due to insulinoma is unusual and, as far as we know, has previously been reported in only 34 patients. In this case report, we describe the clinical features, electrophysiological features, and pathological findings in a 37-year-old patient with polyneuropathy from repeated hypoglycemic episodes over a 9-year period that related to an insulinoma. The literature is discussed. The reported case is of special interest because the peripheral neuropathy led to the correct diagnosis.

Adult↗

Oral mucosal blood flow following dry ice stimulation in humans.

The aim of the current pilot study was to establish a procedure that would allow the investigation of microcirculatory changes in the oral cavity. The authors studied the effects of painful stimulation using dry ice (CO2). To investigate potential regional differences in the change of blood flow, recordings were made for the tongue and at the mucosa of the hard palate, lip, and oral vestibule. The authors investigated 26 patients divided into groups of younger subjects (10 men, 3 women; age range 21-31 y) and older patients (2 men, 11 women; age range 54-74 y). Mucosal blood flow (mBF) was obtained at the hard palate, at the tip of the tongue, on the midline of the oral vestibule, and at the lip. Measurements were made during rest and for 2 minutes after application of dry ice for a 10-second duration, using a pencil-shaped apparatus. Blood pressure, heart rate, cutaneous blood flow, transcutaneous partial pressure of carbon dioxiode (PCO2) and partial pressure of oxygen (PO2) were recorded. Mucosal blood flow increased at all sites in response to application of dry ice (p <0.001), with peak flow at 0.5 minute to 1.5 minutes after onset of stimulation. During the 1.5 minutes to 2 minutes, blood flow decreased at all measurement sites with a tendency to return to baseline. Heart rate, blood pressure, pCO2, PO2, and cutaneous blood flow did not show significant changes. Overall, responses in older patients showed more variance when compared with younger patients. Stimulation by dry ice appears to be an effective, noninvasive, and tolerable means to investigate mucosal blood flow at different mucosal sites. Preliminary data indicate different levels of responsiveness to painful cold stimulation at different sites on the oral and perioral mucosa; particularly, mucosal blood flow response at the tongue was least pronounced. Therefore, assessment of stimulated mucosal blood flow appears to be a promising tool to investigate the pathophysiology of a number of neurologic symptoms, eg, the burning mouth syndrome.

Adult↗

[Generalized brain edema in non-purulent meningoencephalitis. The anti-edema effect of therapy with dexamethasone].

We report about two female inpatients aged 58 and 24, suffering from non-purulent meningoencephalitis, in the first case caused by varizella-zoster-virus, in the second case probably due to viral infection. Both patients developed a diffuse brain edema associated with a progressive loss of consciousness. The adjunctive treatment with dexamethasone led to rapid improvement of clinical symptoms. Computed tomography revealed a significant reduction of brain edema. These results of these two cases support the efficacy of corticosteroids as adjunctive treatment of diffuse brain edema caused by non-purulent meningoencephalitis. The pathophysiological mechanisms are discussed.

Adult↗

["Ghost and mimicry-tumor"--primary CNS lymphoma].

In recent years, the frequency of primary cerebral lymphoma (PCNSL) has increased, even among immunocompetent patients. In order to treat the disease optimally, early diagnosis is important. We present three patients with atypical courses of this disease and stress the importance of PCNSL in the differential diagnosis for optimal treatment. In a 75-year-old man, a space-occupying, radiopaque, enhancing CNS lesion disappeared completely after biopsy and short steroid therapy. One year later, the tumor recurred on the other side and again regressed after steroid therapy. The first biopsy showed signs of a papillary tumor, so a choroid plexus papilloma was suspected initially. A 57-year-old woman developed progressive bilateral hearing dysfunction. Lymphocytic pleocytosis led to a primary diagnosis of chronic lymphocytic meningitis. During the further course of disease, the patient developed multiple space-occupying cerebral lesions. Stereotactic biopsy revealed PCNSL. Despite combined chemo- and radiotherapy, a relapse occurred. A 49-year-old woman rapidly developed memory and concentration disturbances. Computed tomography revealed diffuse edema in both hemispheres and MRI detected severe, diffuse, white matter lesions. The CSF revealed lymphocytic pleocytosis including plasma cells. Herpes encephalitis and, after lack of clinical improvement and progression of the MRI changes, acute disseminated encephalomyelitis (ADEM) were suspected. Corticosteroid treatment was initiated. After enormous clinical improvement, a clinical relapse occurred and MRI detected bitemporal and singular space-occupying lesions in the corpus callosum and hypothalamus. Finally, open biopsy showed PCNSL. However, on examination of the treatment history of patient 1, the initial diagnosis must be revised; a PCNSL seems most probable. The phenomenon of tumor remission under steroid administration is rare. In patients 2 and 3, atypical clinical signs and symptoms delayed diagnosis of PCNSL. This tumor can mimic diverse neurological diseases and remit following corticosteroid treatment alone. In unclear cerebral disease, biopsy should be performed early for exact diagnosis and optimal treatment.

Aged↗