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Biomedical subjects

D A Nowak

Publications and source records attributed to D A Nowak.

17 recordsLinked to original sources

Spontaneous cerebrospinal fluid hypovolaemia: a therapeutic dilemma?

Spontaneous intracranial hypotension is characterized by orthostatic headaches in conjunction with reduced cerebrospinal fluid volume (CSF) and characteristic imaging findings. We report the clinical course of six consecutive patients with spontaneous intracranial hypotension who were followed between 4 months and 2.5 years. The characteristic orthostatic headaches were present in five patients. Diffuse pachymeningeal enhancement on brain magnetic resonance imaging (MRI) was evident in all cases. CSF detected elevated protein content in three of six patients. In only two of our six patients a first epidural blood patch resulted in complete symptom resolution lasting 4 months and 1 year. Four patients received a second epidural blood patch and one patient also received a third. In four patients, follow-up brain MRI revealed re-occurrence of the typical MRI features and all of them suffered from orthostatic symptoms at this time. Only four patients are free of complaints after an average follow-up period of 10 months. Symptom relief within 7 days from an epidural blood patch is accepted to be diagnostic for spontaneous intracranial hypotension. However, our data illustrate that the clinical course of the syndrome is very unstable and the epidural blood patch is less effective than widely accepted.

Adult↗

Sensorimotor dysfunction of grasping in schizophrenia: a side effect of antipsychotic treatment?

BACKGROUND: Antipsychotic treatment in schizophrenia is frequently associated with extrapyramidal side effects. Objective behavioural measures to evaluate the severity of extrapyramidal side effects in the clinical setting do not exist. OBJECTIVES: This study was designed to investigate grasping movements in five drug naive and 13 medicated subjects with schizophrenia and to compare their performance with that of 18 healthy control subjects. Deficits of grip force performance were correlated with clinical scores of both parkinson-like motor disability and psychiatric symptom severity METHODS: Participants performed vertical arm movements with a handheld instrumented object and caught a weight that was dropped into a handheld cup either expectedly from the opposite hand or unexpectedly from the experimenter's hand. The scaling of grip force and the temporospatial coupling between grip and load force profiles was analysed. The psychiatric symptom severity was assessed by the positive and negative symptom score of schizophrenia and the brief psychiatric rating scale. Extrapyramidal symptoms were assessed by the unified Parkinson's disease rating scale. RESULTS: Drug naive subjects with schizophrenia performed similar to healthy controls. In contrast, medicated subjects with schizophrenia exhibited excessive grip force scaling and impaired coupling between grip and load force profiles. These performance deficits were strongly correlated with the severity of both extrapyramidal side effects related to antipsychotic therapy and negative symptoms related to the underlying pathology. CONCLUSIONS: These data provide preliminary evidence that deficits of sensorimotor performance in schizophrenia are, at least in part, related to the side effects of antipsychotic treatment. The investigation of grasping movements may provide a sensitive measure to objectively evaluate extrapyramidal side effects related to antipsychotic therapy.

Adult↗

Disturbances of grip force behaviour in focal hand dystonia: evidence for a generalised impairment of sensory-motor integration?

BACKGROUND: Focal task specific dystonia occurs preferentially during performance of a specific task. There may be an inefficiently high grip force when doing manipulative tasks other than the trigger task, possibly reflecting a generalised impairment of sensory-motor integration. OBJECTIVE: To examine how well subjects with writer's cramp (n = 4) or musician's cramp (n = 5) adapted their grip force when lifting a new object or catching a weight. METHODS: Nine patients with focal hand dystonia and 10 controls were studied. Experiments addressed different motor behaviours: (A) lifting and holding an object; (B) adjusting grip force in anticipation of or in reaction to a change in load force by catching a small weight dropped expectedly or unexpectedly into a hand held receptacle. RESULTS: In (A), patients produced a grip force overshoot during the initial lifts; force overflow was most pronounced in those with writer's cramp. Patients and controls adjusted their grip force to object weight within one or two lifts, though patients settled to a steady force level above normal. In (B), patients with focal hand dystonia and normal controls showed similar predictive grip force adjustments to expected changes in object load, suggesting that this aspect of sensory-motor integration was normal. Patients had a shorter latency of grip force response than controls after an unexpected load increase, reflecting either a greater level of preparatory motor activity or a disinhibited spinal reflex response. CONCLUSIONS: The overall increased grip force in patients with focal hand dystonia is likely to be a prelearned phenomenon rather than a primary disorder of sensory-motor integration.

Adult↗

Digit cooling influences grasp efficiency during manipulative tasks.

A commonly experienced effect of cold is a sensation of numbness and loss of sensibility in the fingers. Intact tactile sensibility of the grasping digits is essential for the efficient scaling of grip force level during the manipulation of hand-held objects. We investigated whether or not cooling of the grasping digits affects scaling of the grip force magnitude in relation to the loads resulting from continuous vertical arm movements performed with a grasped instrumented object. Maxima and minima of load force occurred at the lower and upper turning point of the movement cycle, respectively, and were accompanied by maximum and minimum peaks in grip force occurring close in time prior to and following digit cooling, respectively. Thus, digit cooling did not influence the ability to adjust the grip force profile in anticipation of movement-induced fluctuations in load force. However, subjects established significantly higher grip forces against the hand-held object following digit cooling and generated a 10-70% higher ratio between grip and load forces at the upper and lower turning points of the movement cycle. It is thought that the impaired economical scaling of grip force level is the result of reduced sensory feedback from the grasping fingers during digit cooling. The results provide further evidence to support the suggestion that cutaneous afferent input plays a subordinate role in the predictive temporal regulation of the grip force profile, but is used to adapt economically the force level to the actual loading requirements during dynamic object manipulation.

Acceleration↗

Grip force control during object manipulation in cerebral stroke.

OBJECTIVE: To analyze impairments of manipulative grip force control in patients with chronic cerebral stroke and relate deficits to more elementary aspects of force and grip control. METHODS: Nineteen chronic stroke patients with fine motor deficits after unilateral cerebral lesions were examined when performing 3 manipulative tasks consisting of stationary holding, transport, and vertical cyclic movements of an instrumented object. Technical sensors measured the grip force used to stabilize the object in the hand and the object accelerations, from which the dynamic loads were calculated. RESULTS: Many patients produced exaggerated grip forces with their affected hand in all types of manipulations. The amount of finger displacement in a grip perturbation task emerged as a highly sensitive measure for predicting the force increases. Measures of grip strength and maximum speed of force changes could not account for the impairments with comparable accuracy. In addition to force economy, the precision of the coupling between grip and load forces was impaired. However, no temporal delays were typically observed between the grip and load force profiles during cyclic movements. CONCLUSIONS: Impaired sensibility and sensorimotor processing, evident by delayed reactions in the perturbation task, lead to an excessive increase of the safety margin between the actual grip force and the minimum force necessary to prevent object slipping. In addition to grip force scaling, cortical sensorimotor areas are responsible for smoothly and precisely adjusting grip forces to loads according to predictions about movement-induced loads and sensory experiences. However, the basic feedforward mechanism of grip force control by internal models appears to be preserved, and thus may not be a cortical but rather a subcortical or cerebellar function, as has been suggested previously.

Adult↗

A retrospective clinical, laboratory and outcome analysis in 43 cases of acute aseptic meningitis.

Forty-three consecutive cases of acute aseptic meningitis (AAM) presenting within a 24-months period were retrospectively analysed with respect to clinical symptomatology, cerebrospinal fluid (CSF) findings, clinical course, treatment and outcome. Nineteen of the 43 AAM cases (44%) were caused by enterovirus, one by HIV (2%), two by Varicella zoster virus (5%), three due to herpes simplex virus I (7%), two due to herpes simplex virus II (5%), one due to Central European encephalitis virus (2%), and in 15 patients (35%) the aetiology of AAM remained unknown. Headache (100%) and fever (93%) were the presenting symptoms in the majority of cases. Signs of preceding infection were predominantly gastrointestinal in the enterovirus subgroup, but were inconsistently observed in the other subgroups. CSF findings at the first lumbar tap on admission generally revealed lymphomonocytic pleocytosis of less than 500 cells per micro l, mild to moderately elevated protein and normal lactate and glucose levels. Initial therapy consisted of an empirical antiviral and antibiotic regimen until a serological diagnosis was available. Acyclovir, effective only in herpes family viruses, was initially administered to all AAM cases. Effective therapy for other viral pathogens are not broadly available and treating AAM of unknown aetiology imposes a particular problem. The average hospitalization time ranged from 16 to 31 days. Patients were either discharged home (72%) or transferred to a rehabilitation centre (28%). The outcome was good (40%) to fair (51%) in the majority of cases.

Acute Disease↗

Reversible segmental cerebral vasoconstriction (Call-Fleming syndrome): are calcium channel inhibitors a potential treatment option?

The Call-Fleming syndrome is characterized by sudden onset of thunderclap-like headache and focal neurological deficits. The pathophysiological correlate is a reversible segmental cerebral vasoconstriction frequently associated with focal cerebral ischaemia. The syndrome has been described in a variety of clinical conditions, and recently an association between the syndrome and exposure to vasoactive drugs was observed. Effective treatment options are not known. A 63-year-old female developed sudden 'worst ever' headache. Initial neurological examination, laboratory blood tests, CSF examination and brain magnetic resonance imaging (MRI) were normal. Previous medical history was unremarkable and she did not take vasoactive drugs. Eleven days after the onset of headache she developed visual field impairment and a right-sided hemiparesis. Brain MRI revealed bilateral posterior and left parietal ischaemic strokes. Cerebral catheter angiography showed segmental arterial vasoconstriction. A vasodilative therapy with calcium channel inhibitors was started and serial transcranial Doppler ultrasonography demonstrated resolution of cerebral arterial vasoconstriction. The present case illustrates that calcium channel inhibitors may be an effective therapy for segmental cerebral arterial vasoconstriction. However, more clinical data are needed to prove this observation.

Calcium Channel Blockers↗

[Isolated intracerebral cystic echinococcosis. Unusual presentation of a rare disease].

HISTORY AND CLINICAL FINDINGS: A 46-year-old woman was admitted with a first generalised tonic-clonic seizure. Past medical history was unremarkable. She reported no travels abroad within the past five years. INVESTIGATIONS: Brain MRI demonstrated disseminated cerebral and cerebellar focal lesions. Cerebrospinal fluid analysis showed a mild eosinophilic pleocytosis. Extensive imaging investigations and serological tests revealed no hints for a systemic parasitic disease. A stereotactic brain biopsy detected no pathogenic agent. 10 months following the initial admission, brain MRI showed multiple cystic lesions with ring-like enhancement following Gd-DTPA administration. At that time serological tests were positive for an infection with Echinococcus granulosus. DIAGNOSIS: The diagnosis was based on positive sequential serological tests and the typical MRI findings in conjunction with an eosinophilic cerebrospinal fluid pleocytosis. TREATMENT AND CLINICAL COURSE: An antihelmintic therapy with Albendazole was initiated. Following two-months of oral Albendazole administration, brain MRI revealed a clear reduction in size and number of the hydatid lesions. The patient was free of complaints and the clinical examination was unremarkable. CONCLUSION: Isolated intracerebral manifestation of cystic echinococcosis is very rare. Compared to the typical presentation with a solitary cerebral hydatid cyst, the dissemination of hydatid cysts is quite uncommon in cystic echinococcosis. Diagnosis should be based on the typical MRI findings and serological tests. The present case demonstrates that the later may be negative over a long period within the clinical course of the disease offering a real challenge to the clinician.

Albendazole↗

Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm?

OBJECTIVES: Dysplastic gangliocytoma (Lhermitte-Duclos disease) is a rare disorder, characterized by a slowly progressive unilateral tumour mass of the cerebellar cortex. The fundamental nature of this apparently benign entity and in particular its pathogenesis remain unknown. The debate, whether it represents a neoplastic, malformative or hamartomatous lesion, is still in progress. Lhermitte-Duclos disease was recently encountered to be part of a multiple hamartoma-neoplasia complex (Cowden's syndrome). METHODS: The present account gives a review of the pertinent literature with emphasize on clinical presentation, radiological findings, surgical procedures, histopathological features and pathogenetic considerations of dysplastic cerebellar gangliocytoma. RESULTS: Dysplastic cerebellar gangliocytoma clusters within the third to fourth decades of life. Cranial nerve palsies, unsteadiness of gait, ataxia and sudden neurological deterioration as a result of occlusive hydrocephalus are frequent signs and symptoms. Associations with other congenital malformations, such as megalencephaly, polydactylia, multiple haemangioma and skull abnormalities are common. Magnetic resonance imaging (MRI) is the diagnostic modality of choice and reveals characteristic non-enhancing gyriform patterns with enlargement of cerebellar folia. Surgery is the therapeutic procedure generally performed and complete resection was attempted in the majority of cases. The histopathological findings of Lhermitte-Duclos disease include widening of the molecular layer, which is occupied by abnormal ganglion cells, absence of the Purkinje cell layer and hypertrophy of the granule cell layer. CONCLUSIONS: Dysplastic gangliocytoma of the cerebellum is of benign behaviour and its incidence is extremely rare. The disease should be considered when confronted with a young adult presenting with clinical signs of progressive mass effect in the posterior fossa. The lesion is hypointense on T1- and hyperintense on T2-weighted magnetic resonance images. Recognition of the disease is of particular importance, as the frequent but under-reported coexistence with Cowden syndrome, should prompt thorough clinical and apparative investigation to detect or exclude concomitant malignancies.

Adult↗

Neurosarcoidosis: a review of its intracranial manifestation.

Sarcoidosis is a multisystem disease of unknown cause and with a worldwide distribution. Involvement of the central nervous system occurs in a relatively small number of patients with sarcoidosis. Isolated neurosarcoidosis without signs of systemic disease is a rarity. Because of its non-specific clinical presentation and neuroradiological imaging characteristics, intracranial neurosarcoidosis remains a very difficult diagnosis, particularly in the absence of systemic signs of the disease. Intracranial neurosarcoidosis has a predilection for the basal leptomeninges commonly affecting the cranial nerves, but any part of the brain may be involved, resulting in a wide spectrum of clinical syndromes. Cranial nerve involvement is the most common single symptom. Intracranial sarcoid manifests as nodular or diffuse leptomeningeal thickening and extraor intra-axial parenchymatous lesions. Intracranial sarcoid may mimic various forms of meningitis, including carcinomatous and intracranial mass lesions such as meningioma, lymphoma and glioma, based on neuroradiological imaging. Magnetic resonance imaging is a very sensitive diagnostic tool for detecting intracranial abnormalities due to neurosarcoidosis. Lumbar puncture is useful in ruling out other neurological disorders, in particular infectious, but cerebrospinal fluid findings are not specific. Angiotensin-converting enzyme in serum and cerebrospinal fluid may be increased, decreased or normal. Therapy consists of immunosuppressive agents and should be initiated with corticosteroids. Other immunosuppressive drugs should be added in severe cases or after frequent recurrences. We review here all aspects of intracranial neurosarcoidosis from the clinical point of view, with special emphasis on presentation, diagnostic procedures, differential diagnostic considerations and treatment.

Brain Diseases↗

Spinal intradural capillary haemangioma: a review.

Capillary haemangioma is a benign tumour frequently encountered in the skin and other soft tissues. Histologically, these vascular lesions are characterised by nodules of capillary-sized vessels lined by flattened endothelium, each of which is subserved by a feeding vessel. Capillary haemangioma of the central and peripheral nervous system is extremely rare. Less than 20 of these lesions have been described as occurring within the confines of the spinal dura mater, in close relation to the conus medullaris and nerve roots of the cauda equina. The presenting symptoms are similar to those of more common intradural tumours at the conus-cauda region. Magnetic resonance imaging is the imaging modality of choice, and homogeneous enhancement following administration of Gd-DTPA is a useful clue to the diagnosis. Complete resection is the treatment of choice, and during surgery the vascular tumour is usually found encapsulated and sharply bordered from the surrounding parenchyma of the spinal cord and affected nerve roots. In the present account we give an overview of the clinical features, neuroradiological findings, therapeutic options and histopathological differential diagnostic aspects of spinal intradural capillary haemangioma. In general, vascular lesions of this entity are preoperatively misdiagnosed as neoplasms, and a higher level of clinical and radiological suspicion may avoid surgical overtreatment of these benign tumours.

Adult↗

Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum).

OBJECTIVE: Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is a rare hamartomatous lesion of the cerebellar cortex. The pathogenesis of the disease is still poorly understood. Lhermitte-Duclos disease was recently considered to be part of a multiple hamartoma-neoplasia syndrome (Cowden disease). We add two further cases to this rare entity. PATIENTS: A 24-year old woman presented with occipital headaches, blurred vision, diplopia and ataxia of gait. Physical examination revealed turricephaly. The second patient was a 37-year old woman, who presented with progressive occipital headache with nausea and vomiting. Physical examination revealed congenital facial asymmetry. Computed tomography and NMR-imaging, respectively demonstrated a space occupying mass of a cerebellar hemisphere in both cases. RESULTS: Suboccipital craniotomy and complete removal of the infratentorial tumour were performed in both patients. Histopathological findings clinched the diagnosis of Lhermitte-Duclos disease. Postoperative course was uneventful in the first and complicated by progressive occlusive hydrocephalus in the second patient, necessitating permanent surgical shunt drainage. Both patients were discharged free of complaints. CONCLUSIONS: Dysplastic cerebellar gangliocytoma is commonly associated with progressive mass effects in the posterior fossa and typically presents with headaches, cerebellar dysfunction, occlusive hydrocephalus and cranial nerve palsies. The disease usually manifests in young adults, but the age at presentation ranges from birth to the sixth decade. There is no sex predilection. NMR-imaging became a useful clue to the diagnosis within the last decade. Therapy consists of decompression of the posterior fossa by total surgical removal of the tumour mass.

Adult↗

The effects of digital anaesthesia on predictive grip force adjustments during vertical movements of a grasped object.

Grip force adjustments to fluctuations of inertial loads induced by vertical arm movements with a grasped object were analysed during normal and impaired finger sensibility. Normally grip force is modulated in a highly economical way in parallel with fluctuations of load force. Two subjects performed vertical up and down movements of a grasped object, both with normal finger sensibility and then cutaneously anaesthetized finger sensibility. Short breaks were taken in between single movements, during which the object was held stationary. After digital anaesthesia was applied to the grasping fingers, both subjects substantially increased the grip force. The grip force amplitude and timing still anticipated changes in load force, although the established grip force had already overcome movement-induced load force peaks. This implies that the increase of grip force and consequently the elevated force ratio between maximum grip and maximum load force are not processed to alter the feedforward system of grip force control. Cutaneous afferent information from the grasping digits appears to be necessary for economic scaling of the grip force level, but it plays a subordinate role in the precise anticipatory temporal coupling of grip and load forces during voluntary object manipulation.

Adult↗

Effects of changing gravity on anticipatory grip force control during point-to-point movements of a hand-held object.

We investigated the quality of predictive grip force control during gravity changes induced by parabolic flight maneuvers. During these maneuvers gravity varied: There were 2 periods of hypergravity, in which terrestrial gravity nearly doubled, and a 20-s period of microgravity, during which a manipulated object was virtually weightless. We determined grip and load forces during vertical point-to-point movements of an instrumented object. Point-to-point movements were a combination of static (stationary holding) and dynamic (continuous movements) task conditions, which were separately analyzed in our previous studies. Analysis of the produced grip forces revealed that grip adjustments were closely linked to load force fluctuations under each gravity condition. In particular, grip force maxima coincided closely in time with load force peaks, although these occurred at different phases of the movement depending on the gravity level. However, quantitative analysis of the ratio of maximum grip force to the corresponding load force peak revealed an increased force ratio during microgravity when compared to that during normal and hypergravity. We hypothesize that the impaired precision of force coupling with respect to force magnitude during microgravity results from reduced feedback information about the object's mass during the stationary holding of the object in between each movement. The results indicate that the temporal grip force regulation is highly automatized and stable, whereas economical planning of force magnitude is more flexible and might reflect changes of the external loading condition.

Adaptation, Physiological↗

Intraneural growth of a capillary haemangioma of the cauda equina.

Solitary intraneural haemangiomas are very rare. A case of intraneural capillary haemangioma involving two nerve roots of the cauda equina is reported. The patient was a 63-year-old woman with a three years history of intermittent lumbalgia and numbness of the ventral surface of the left thigh. Magnetic resonance imaging detected an intradural extramedullary nodular space occupying mass at the level of the conus medullaris. Laminectomy of T12 and complete removal of the tumour were performed. Histopathological analysis demonstrated a capillary haemangioma. The tumour was located within the sheaths of a spinal nerve root. The lesion consisted of a myriad of small and very small vessels, reticularly arranged with normal nerve fascicles dispersed within the nodules of clustered capillaries. The present case of an intraneural capillary haemangioma of the cauda equina appears to be one of the first reported examples of this entity in the world's literature. The clinical presentation, diagnostic procedures and therapeutic options of intraneural haemangiomas of the conus medullaris and cauda equina are discussed. The current literature is reviewed.

Cauda Equina↗