Biomedical subjects
J G Heckmann
Publications and source records attributed to J G Heckmann.
Clinical outcomes of three Parkinson's disease patients treated with mandibular implant overdentures.
Parkinson's disease (PD) often affects the oro-pharyngeal musculature, leading to problems with speaking, chewing and swallowing. The inevitable reduction in food and fluid intake contributes to the further deterioration of neurological symptoms. Parkinson's disease patients have great difficulties in adjusting to the use of complete dentures. It is the purpose of this report to evaluate the benefit of using dental implants combined with overdentures to improve chewing and predigestion capacity in severely handicapped PD patients. Three edentulous PD patients (2 male, 1 female; mean age 75.7 years; mean PD duration 4.3 years; PD severity grade III according to Hoehn and Yahr; mean edentulousness 19.3 years) complaining of poor chewing ability were included in this evaluation. One-stage dental implants were placed in the interforaminal region of the mandible. After completion of healing, new overdentures were fabricated. Custom-made non-rigid (resilient) telescopic attachments were used for retention of the overdentures on the implants. Follow-up examinations of the 3 patients were made between 28 and 42 months after the completion of treatment, and peri-implant tissue conditions as well as the patients' self-assessed satisfaction level were recorded. A modified gastrointestinal symptoms questionnaire, Hoehn and Yahr Scale and body weight measurements were used to monitor gastrointestinal impairment and PD severity. The peri-implant parameters indicated healthy soft tissue conditions and all Periotest values were in the negative range. The patients judged their chewing abilities to be greatly improved. Since placing the implants, PD severity had deteriorated to grade IV (Hoehn and Yahr scale) in 2 patients and was stable in 1 patient. The body weight had improved slightly in all patients (mean 2.2 kg). On the gastrointestinal scale, all patients had improved from a mean score of 8.7 to 5.7. Non-rigid telescopic attachments for overdenture stabilization are particularly suitable for PD patients as they are easy to handle and to clean. The patients reported remarkable improvement in their chewing ability, an assessment which would seem to be supported by the improved gastro-intestinal index. The regimen described appears to be a useful adjunctive treatment in edentulous Parkinson's disease patients and may be considered for patients with diseases similarly affecting motor skills.
Progressive muscle pain, weight gain, fatique and unusual body shape change.
Multiple symmetrical lipomatosis (MSL) is a rare syndrome characterized by symmetric unencapsulated lipomas around the axial region and frequently associated with neurological involvement, particularly myopathy and neuropathy (Klopstock et al., 1994). Here we present a typical image of a patient with MSL, explaining this disease for neurologists who might care for these patients in an average day at the neuromuscular consultation unit.
[Mechanisms of cerebral autoregulation, assessment and interpretation by means of transcranial doppler sonography].
Cerebrovascular autoregulation assures constancy of cerebral perfusion despite blood pressure changes, as long as mean blood pressure remains in a range between 50-170 mmHg. Static and dynamic myogenic mechanisms dampen sudden blood pressure changes. Neurogenic influences of sympathetic, noradrenergic fibers modulate primarily proximal, large diameter segments of cerebral arteries, but also small 15-20 microns diameter vessels. Parasympathetic, vasodilating impulses are of less influence. Monoaminergic brainstem centers such as the dorsal raphe nucleus, locus coeruleus or nucleus reticularis pontis oralis also influence vessel tone. Metabolic, local parenchymal and endothelial substances have major impact on cerebral vessel tone. Particularly important are nitric oxide, calcitonin gene related peptide, substance P, endothelin, potassium channels and autocoids such as histamine, bradykinin, arachidonic acid, prostanoids, leucotrienes, free radicals or serotonin. The clinical examination of autoregulation is mostly based on brief blood pressure changes induced by drugs such as angiotensin, phenylephrine or sodium nitroprusside, or by challenge maneuvers. Frequently, blood pressure is challenged by a tilt-table maneuver, the "leg-cuff"-method according to Aaslid, or a Valsalva maneuver. The analysis of coherence and phase relation between spontaneous or metronomic breathing modulation of blood pressure and brain perfusion also assesses autoregulatory function. Cerebral blood flow is determined by means of transcranial Doppler sonography, mostly of the proximal segment of the mid-cerebral artery. There is some controversy whether a decrease of cerebral blood flow velocity measured at this segment indicates vasodilatation at the insonated segment or reflects blood flow reduction due to decreased perfusion of down-stream vessel segments. Various clinical and animal studies are presented demonstrating diameter constancy of the insonated mid-cerebral artery segment and thus indicating that slowing of mid cerebral artery blood flow velocity as assessed by transcranial Doppler sonography is due to a decrease of down-stream perfusion. Direct, intraoperative measurements of vessel diameter confirm this conclusion.
[The Susac syndrome--a retinocochleocerebral angiopathy].
INTRODUCTION: There has been a series of case reports of otherwise healthy patients suffering from microangiopathy of the brain, retina and cochlea. Most patients were young women presenting clinically with a subacute encephalopathy, branch retina artery occlusions, and hearing loss. In 1994 the name "Susac syndrome" has been proposed for this disease entity. METHOD: Case report and review of 64 published cases, identified through MEDLINE are given. CASE REPORT: We describe a 32-year-old otherwise healthy woman presenting with a subacute encephalopathy, multiple branch retinal artery occlusions and bilateral hearing loss. MRI of the brain revealed multiple small white and grey matter lesions without contrast enhancement. CSF protein was elevated, oligoclonal bands were negative. Immunological laboratory parameters, microbiology, virology, coagulation studies, SEP, AEP, VEP and cerebral DSA were normal. REVIEW OF THE LITERATURE: Of 64 identified patients 58 were women. The mean age of the patients was 30 years. 60 patients (94%) had arterial occlusions, which were bilateral in 39%. 48 patients reported hearing loss, 37 patients (58%) had a global encephalopathy, but other neurologic manifestations were common. CONCLUSION: This rare syndrome has a strong young female preponderance. MRI of the brain often shows lesions suggestive of multiple sclerosis. Fluorescein angiography may show arteriolar wall hyperfluorescence. Patients can be identified at an early stage with a careful history and physical examination. Early treatment with corticosteroids is often associated with a good prognosis. Cyclophosphamide and antiplatelet drugs may be added in complicated cases.
[Langerhans-cell histiocytosis of the orbit; diagnosis, treatment and outcome in three patients -- children and adults].
BACKGROUND: Langerhans cell histiocytosis usually affects children. Systemic Langerhans cell histiocytosis may be present in 37% of affected children. Ten per cent of all children die of the disease. CASE REPORTS: Patient 1. A 9-year-old boy presented with painful upper lid swelling OD. Ultrasonography revealed a space-occupying lesion adjacent to a lytic defect of the frontal bone as shown on coronal CT. MRI showed marked contrast enhancement of the mass which came close to the intracranial fossa. The vascularized tumor was excised using an anterior orbitotomy with upper lid crease incision. No evidence of local or systemic recurrence was present three years postoperatively. Patient 2. A 19-year-old male presented with foreign body sensation, double vision OS and numbness of the left face of six weeks' duration. The ocular findings revealed a sixth nerve palsy OS. Otherwise, the eyes were unremarkable. Sensitivity of cranial nerve V2 was decreased. CT revealed an osteolytic process in the lateral orbital wall and sphenoid wing. MRI demonstrated a space-occupying lesion involving the temporalis muscle laterally, the pterygopalatine fossa medially with intracranial extension into the temporal lobe. Surgical excision was performed using a transcranial approach. Signs and symptoms had completely resolved six months after surgery with no evidence of local or systemic recurrence. Patient 3. A 30-year-old male presented with right-sided headache, painful upper lid swelling and vertical diplopia. Coronal CT scan revealed a lytic defect in the frontal bone laterally. Curettage and complete excision were performed using an infrabrow incision. Follow-up examination four years later revealed an intact ocular motility with no evidence of local or systemic recurrence. In all three cases the tumor showed characteristic light-microscopy and immunohistochemical features of Langerhans cell histiocytosis. CONCLUSION: Langerhans cell histiocytosis of the orbit is an important differential diagnosis of osteolytic and/or space-occupying lesions not only in children, but also in adults. A diagnostic biopsy followed by surgical excision and/or local and systemic corticoid- and chemotherapy are essential regarding the prognosis of local and systemic disease.
Neurologic manifestations of cerebral air embolism as a complication of central venous catheterization.
UNLABELLED: OBJECTIVE, PATIENTS, AND METHODS: A severe case of cerebral air embolism after unintentional central venous catheter disconnection was the impetus for a systematic literature review (1975-1998) of the clinical features of 26 patients (including our patient) with cerebral air embolism resulting from central venous catheter complications. RESULTS: The jugular vein had been punctured in eight patients and the subclavian vein, in 12 patients. Embolism occurred in four patients during insertion, in 14 patients during unintentional disconnection, and in eight patients after removal and other procedures. The total mortality rate was 23%. Two types of neurologic manifestations may be distinguished: group A (n = 14) presented with encephalopathic features leading to a high mortality rate (36%); and group B (n = 12) presented with focal cerebral lesions resulting in hemiparesis or hemianopia affecting mostly the right hemisphere, with a mortality rate as high as 8%. In 75% of patients, an early computed tomography indicated air bubbles, proving cerebral air embolism. Hyperbaric oxygen therapy was performed in only three patients (12%). A cardiac defect, such as a patent foramen ovale was considered the route of right to left shunting in 6 of 15 patients (40%). More often, a pulmonary shunt was assumed (9 of 15 patients; 60%). For the remainder, data were not available. CONCLUSION: When caring for critically ill patients needing central venous catheterization, nursing staff and physicians should be aware of this potentially lethal complication.
Low incidence of stroke in the Chiquitanos tribe in the Bolivian lowlands.
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[Tuberculous meningoencephalitis].
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[Nosocomial pneumonias in a neurology intensive care unit].
BACKGROUND AND OBJECTIVE: Nosocomial pneumonia in patients in an intensive care unit (ICU) are a great problem as a cause of increased morbidity and mortality as well as the resulting high cost of treatment. This study was aimed at determining the incidence of nosocomial pneumonia and the risk factors for its occurrence in patients with severe neurological disease. PATIENTS AND METHODS: Between 1.1. and 31.12.1997, 217 patients (125 men, 92 women; average age 63.4 years) were prospectively included if they were treated for more than 48 hours in the ICU of the Neurology Department of Erlangen University. The occurrence of nosocomial pneumonia (NP) was noted, using the criteria of the Center of Disease Control and Prevention (CDC). Incidence of the diseases was related to age, sex, initial state of consciousness, type of ventilation, duration of stay in the ICU and any associated medical condition. RESULTS: NP was diagnosed in 68 patients (31.4%). Statistically significant relative risks were male sex (2.4 fold, P < 0.01), clouded consciousness with a Glasgow coma score < 8 (6.2 fold, P < 0.001), mechanical ventilation (8.4 fold, P < 0.001), time in ICU > or = 8 days (9.3 fold, P < 0.001) and associated medical condition (3.3 fold, P < 0.005). In 17.7% of cases no relevant pathogen was identified microbiologically. A mixed infection was present in 36.8% of cases. The most common Gram-positive organism was Staph, aureus (35.3%), the most common Gram-negative ones were Ps. aeruginosa (25%), Kl. pneumoniae and Kl. oxytoca (11.8%), E. Coli (10.3%) and Acinetobacter species (7.4%). There was also a high rate of infection or infestation with Candida albicans or glabrata (41.2%). NP played a clinically decisive role in the fatal course of 13 of the 47 patients who died. CONCLUSION: These data (incidence, relative risk) can, by taking into consideration various aspects of specialist and hospital hygienic practices, contribute to a continuing optimization of the prevention and treatment of disease.
[Detection of patent foramen ovale. Transesophageal echocardiography and transcranial Doppler sonography with ultrasound contrast media are "supplementary, not competing, diagnostic methods"].
BACKGROUND: The prevalence of patent foramen ovale (PFO) in healthy individuals is estimated to be about 25% and is elevated to 40% patients with stroke. To date transesophageal echocardiography (TEE) was considered to be the most sensitive method to detect PFO and was regarded as the gold standard. Transcranial Doppler sonography of the middle cerebral artery during contrast injection (c-TCD) has recently been proposed as an alternative method for the detection of PFO. We report our experience on 45 patients (age < 55 years) with stroke or transient ischemic attack (TIA) in whom both c-TCD and TEE were performed to detect PFO as a mechanism for embolic cerebral ischemia. PATIENTS AND METHODS: In 45 patients (21 women, 24 men, mean age 41.4 years ranging from 17 to 54 years) with cerebral ischemia, both standardized TEE and standardized c-TCD were performed separately. When any PFO was found by TEE and/or c-TCD, it was classified as positive. If c-TCD was positive but TEE negative, a second TEE was performed and vice versa. RESULTS: PFO was found epicritically in 26 patients (57.8%). First TEE detected PFO in 24 cases (sensitivity 92.3%). Separately performed c-TCD detected PFO in 22 cases of the PFO-positive cases (sensitivity 84.6%). However, c-TCD detected PFO in 2 cases, in which the first TEE had been negative, leading to a second TEE which confirmed PFO and demonstrated minimal shunt (7.7%). TEE detected PFO in 4 cases in which first c-TCD was negative. A second c-TCD confirmed in 2 of these 4 cases a positive right-to-left shunt. Neither method revealed false positive results (specifity 100%). The positive predictive value was 100% in both methods. The negative predictive value in TEE was 90.5% and in c-TCD was 82.6%. CONCLUSION: TEE and c-TCD are not concurrent diagnostic tools to detect PFO. Both supplement each other. If both methods are used in all PFO-suspected patients, PFO detection rate is higher than when using either method alone.
[Renal tubular acidosis with severe hypokalemic tetraparesis after ibuprofen intake].
HISTORY: A 72-year-old woman was admitted because of severe acute tetraparesis, more marked proximally. For six months she had been taking ibuprofen, up to 4800 mg daily, for a painful ulcer of the lower leg. INVESTIGATIONS: Biochemical tests revealed marked hypokalaemia (serum potassium 1.4 mmol/l) with a metabolic acidosis (pH 7.29). The ECG showed changes of hypokalaemia (ST-segment depression and U wave). TREATMENT AND COURSE: Within two days of administering potassium and bicarbonate the pareses completely regressed. Transitorily abnormal renal functions also rapidly normalized after ibuprofen had been discontinued. CONCLUSION: The biochemical findings suggest renal tubular acidosis, type 2, most likely caused by the excess intake of ibuprofen, a drug which can cause renal dysfunctions with life-threatening electrolyte abnormalities.
Nontraumatic seizure-associated bilateral fractures of the head of the humerus.
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Neurological manifestations of chronic hepatitis C.
Hepatitis C virus (HCV) infection is often associated with abnormal immunological responses. We describe four patients with vasculitic neurological signs and symptoms following HCV infection. A 56-year-old woman with HCV infection developed peripheral neuropathy characterized by asymmetric distal painful hypesthesia, dysesthesia and moderate motor weakness of the lower limbs. Serological examinations revealed cryoglobulinemia and low levels of complement C4. A biopsy of the sural nerve revealed vasculitic neuropathy. HCV infection associated immunomediated vasculitis was diagnosed. While steroid therapy was ineffective, treatment with interferon-alpha improved the neuropathy considerably without, however, eliminating HCV infection. A 62-year-old man with HCV infection developed peripheral sensory neuropathy. Complement C3 was slightly diminished. Nerve biopsy revealed vasculitic neuropathy. A 71-year-old woman developed chronic symmetric sensomotor polyneuropathy. HCV hepatitis followed blood transfusions. Cryoglobulins tested positive, consistent with type II cryoglobulinemia. Complements C3 and C4 were diminished. Inflammatory infiltrates in the sural nerve biopsy specimen led to the diagnosis of chronic vasculitic disorder. A 55-year-old woman with HCV infection developed vasculitis of the skin, connective tissue, visceral organs, and kidney, leading to hemodialysis. Neurologically she developed severe apathy and drowsiness, myoclonic jerks, exaggerated deep tendon reflexes, and positive pyramidal signs. Magnetic resonance imaging of the brain showed diffuse increased signal abnormalities involving supra- and infratentorial white matter suggesting cerebral vasculitis. Cryoglobulins were positive, complements C3 and C4 slightly diminished (54 mg/dl, 4.3 mg/dl). Supportive therapy resulted in neurological improvement. Treatment with interferon-alpha was discontinued because of agranulocytosis. In patients with peripheral neuropathy or signs of leucencephalopathy, a hepatitis C associated vasculitis should be considered in the differential diagnosis.
Ondine's curse in association with diabetes insipidus following transient vertebrobasilar ischemia.
Ischemic lesions of the brainstem can lead to complex neurologic deficits. Failure of the automatic control of ventilation (Ondine's curse syndrome) is a possible but rare syndrome following localized brainstem dysfunction. We report on a 49-year-old man with intermittent bradycardia, cranial nerves' dysfunctions and a slight right-sided hemiparesis. An acute brainstem ischemia was diagnosed and treated immediately with high-dose heparin. Cerebral angiography revealed a proximal occlusion of the left vertebral artery but a normal right vertebral artery and a hyperplastic right posterior inferior cerebellar artery. Cranial Computed Tomography and MRI scan demonstrated multiple ischemic lesions in the posterior circulation. During a 4-week treatment course the patient underwent six episodes of acute severe hypoxia and hypercapnia requiring orotracheal intubation twice and manual ventilation by air mask over a few minutes for four times after a tracheostomy had been performed. Twice a short-term episode of hypothalamic Diabetes insipidus was observed following hypoventilation. We conclude that both Ondine's curse syndrome and diabetes insipidus were due to transient vertebrobasilar ischemia.
Progression of optic neuritis to multiple sclerosis: an 8-year follow-up study.
OBJECTIVE: The relationship between acute monosymptomatic optic neuritis (AON) and subsequent multiple sclerosis (MS) is still doubtful. We investigated the risk of developing MS in patients from North Bavaria, who were suffering from AON. PATIENTS/METHODS: Twenty-nine patients with clinical evidence of AON were included in the study. Initial evaluation included brain resonance imaging (MRI) and a clinical neurological examination. Follow-up examinations were performed after 72-108 months (mean: 96 months) in 26 patients (three patients were lost to follow-up) and consisted of a second complete neurological examination. Diagnosis of MS was established according to the criteria of Poser CM, Paty DW, Scheinberg L. New diagnostic criteria for multiple sclerosis: guidelines for research protocols. Ann Neurol 1983:13:227-231. RESULTS: At follow-up, 14 of 26 patients (54%) had converted to clinically definite MS. Nine patients (64%) had developed MS within 2 years after the onset of AON. CONCLUSION: We observed the development of MS in 54% of the 26 investigated AON patients. The exceptional feature of the present study was the relatively long follow-up period of 8 years.
Comment on the paper: Batchelor TT, Platten M, Palmer-Toy DE, Hunter GJ, Lev MH, Dalmau J, Hochberg FH: Chorea as a paraneoplastic complication of Hodgkin's disease. Journal of Neuro-Oncology 36: 185-190, 1998.
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Vestibular evoked blood flow response in the basilar artery.
BACKGROUND AND PURPOSE: Monitoring of the basilar artery (BA) is difficult and has been sparsely performed. The aim of this study was to present physiological data of functional transcranial Doppler sonography (TCD) of the BA during caloric vestibular stimulation in healthy volunteers. METHODS: TCD of the BA was performed in 26 healthy volunteers (14 women, 12 men, age 25.1+/-3 years) during caloric vestibular stimulation. Vertigo was documented using electronystagmography (ENG) and a subjective vertigo scale ranging from 0 to 10 points. Simultaneously, capnogpraphy was performed. RESULTS: All subjects experienced vertigo, nausea and oszillopsia during vestibular irrigation. The average subjective vertigo was for a period of 106 s (+/-65.4); the average subjective estimated degree of vertigo was 6.7 points (+/-1.5). In all subjects, ENG demonstrated horizontal nystagm to the left non-irrigated side. In 14 subjects the subjective vertigo was rated by the individuals as extreme (point score > or =7) and in 12 subjects as low (point score <7). Mean flow velocity (MFV) in the BA increased significantly during vestibular irrigation, being more prominent in the initial irrigation and vertigo phase (5.8+/-5.9%, P<0.05) than in the second vertigo phase (2.2+/-8.8%, P<0.05). The calculated pulsatility index (PI), which indicates the condition of the small resistance vessels, decreased significantly (-4.9+/-8.1%; 4.3+/-8.9%, P<0.05) during both phases of vestibular activation. End tidal pCO2 did not change significantly (constant 5.4+/-0.4 Vol%), but respiration frequency was significantly increased during vestibular stimulation (12.3+/-3.8 min(-1) to 16.4+/-5.3 min(-1) and 16.3+/-4.8 min(-1), P<0.05) probably as a vegetative sign of vertigo. The observed MFV- and PI-changes were more prominent, although not quite significant, in the subgroup of subjects who experienced extreme subjective vertigo than in the subgroup who experienced low subjective vertigo. CONCLUSION: These observations indicate that MFV increase in the posterior circulation is due to activation of the vestibulocerebellum. In addition, it is possible that the previously elaborated MFV increase in the MCA might contribute to MFV increase in the BA via the posterior communicating artery. The difference in the 2 subgroups (extreme vertigo vs. low vertigo) may reflect the great variety of anatomical and physiological conditions of the peripheral vestibular organ, the brainstem anatomy and the corresponding blood supply. For clinical purposes this TCD-test may contribute to the investigation of the vasomotor reserve of the posterior circulation, e.g. in patients with vertebrobasilar ischemia, bilateral vestibular loss or local neurodegenerative disease.