Abstract:
:Leigh syndrome (subacute necrotizing encephalomyopathy, MIM 256,000) is a progressive neurodegenerative disorder of infancy and childhood, with characteristic pathological hallmarks including symmetric necrotizing lesions in the brainstem, basal ganglia, thalamus and spinal cord. It may result from several defects of mitochondrial enzyme complexes, including pyruvate dehydrogenase complex, and respiratory chain complexes I, II, III, IV, V. Clinical presentation mostly includes failure to thrive, developmental delay, muscle weakness, hypotonia, disorders of ocular movements, abnormal respiratory rate and bulbar dysfunction. Symptoms usually start after a few months of normal development and the course is typically rapid and relentless. Affected patients usually die before 5 years of life due to central ventilation failure. Leigh syndrome occurs with an estimated frequency of 1:77,000-1:34,000 live births. The disease demonstrates maternal, X-linked, and autosomal recessive inheritance.
journal_name
Neurol Neurochir Poljournal_title
Neurologia i neurochirurgia polskaauthors
Piekutowska-Abramczuk Dsubject
Has Abstractpub_date
2008-05-01 00:00:00pages
238-50issue
3eissn
0028-3843issn
1897-4260pii
10784journal_volume
42pub_type
杂志文章,评审abstract::A new quantitative motor unit potential (MUP) analysis method, called Multi-MUP analysis, is described. Multi-MUP analysis is a type of decomposition analysis of the EMG signal. At each recording site six different MUPs may be recorded during an epoch of 4.8 sec. A study of 20-30 MUPs including editing takes 3-7 minut...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章,评审
doi:
更新日期:1996-01-01 00:00:00
abstract:OBJECTIVE:Among pineal region lesions meningiomas are extremely rare and include falcotentorial and velum interpositum meningiomas. It is very difficult to discriminate between these two lesions and description of the clinical presentation and the surgical technique in approaching these tumors is limited. We respective...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:10.1016/j.pjnns.2014.09.009
更新日期:2014-01-01 00:00:00
abstract::Involuntary movements are rare ischemic stroke complication. Hemichorea, hemibalism and dystonia cases were described, especially during haemorrhage into basal ganglia and thalamus. The case of 73 years old woman is presented who was admitted to our Clinic cause of sudden choreatic involuntary movements occurrence wit...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:2003-01-01 00:00:00
abstract::From 1983 to 1988 25 infected shunt systems were found in 205 hydrocephalic children. Four children were treated successfully with antibiotics given into the shunt system and intravenously. Seventeen shunt systems had to be removed and Rikham reservoir or external drainage implanted. Antibiotics were given both into t...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1991-05-01 00:00:00
abstract::The authors report a case of sporadic secondary amyloidosis with polyneuropathy in a patient aged 50 years. The disease developed during chronic pyelonephritis. The case is described in view of its rare occurrence, diagnostic difficulties and interesting histological findings. ...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1977-01-01 00:00:00
abstract::The value of S-100 protein as a biochemical marker of the extension of brain damage was studied in a rat model of ischaemic stroke. Increased S-100 levels in cerebrospinal fluid were found between the second and fifth day stroke, and the highest values were noted in animals with largest infarcts. In the acute phase is...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1989-05-01 00:00:00
abstract::The authors discuss a case of a 46 year-old man with bilateral internal carotid artery and right vertebral artery occlusion in unexpectedly good clinical state. The clinical diagnosis is documented by angiographic and doppler findings and brain CT scan. ...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1997-05-01 00:00:00
abstract::It has been recently shown that small variations in cerebral temperature critically determine the extent of neurological damage following an ischaemic insult. These reports spurred a new interest in brain temperature among clinicians--neurologists and neurosurgeons. The information about this subject is very sparse an...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章,评审
doi:
更新日期:1994-07-01 00:00:00
abstract::The placement of tungsten coils into aneurysm sack in patient after SAH is a method of treatment used in many neurosurgical departments. The main indications for coiling procedures are severe neurological status (IV i V according to WFNS) and posterior circulation aneurysms. Coiling can be offered to a patient who doe...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:2001-11-01 00:00:00
abstract:BACKGROUND:Compared with surgical resection, endoscopic transsphenoidal surgery (TSS) for cholesterol granuloma (CG) in the petrous apex (PA) is associated with local recurrence due to obstruction of the drainage route. We present a detailed procedure of an endoscopic TSS using pedicle vascularized nasoseptal flap (PVN...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:10.1016/j.pjnns.2016.08.006
更新日期:2016-01-01 00:00:00
abstract::The author reports a case of Behcet's disease in a girl aged 18 years with recurrent lesions of the oral and vulvar mucosa, eyes and nervous system. The disease had a recurrent character with a tendency for progressing deterioration. Treatment was unsuccessful and the patient died. ...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1985-01-01 00:00:00
abstract::In a group of 15 patients with multiple sclerosis and in a control group of 20 healthy subjects visual evoked potentials produced with flashes or checkerboard pattern, auditory evoked potentials after stimulation with three different tones, and somatosensory evoked potentials after stimulation of the left, right or bo...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1979-01-01 00:00:00
abstract::In the Department of Neurosurgery of the Medical University of Warsaw 11 patients with primary central nervous system lymphomas were diagnosed and treated between 1990 and 1999. The patients comprised of 6 females and 5 males, aged 23 to 86 years, with most patients presenting in the sixth decade. The time from first ...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:2001-01-01 00:00:00
abstract::Every year several thousands of operations on the cervical spine from anterior approach are performed in the world for decompression of spinal cord and nerve roots. It is estimated that in 0.2 to 12% of the operations serious intraoperative and postoperative complications occur. Such cases have been reported in the wo...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章,评审
doi:
更新日期:1997-01-01 00:00:00
abstract::A 23 year old woman with dissecting aneurysm of the left posterior inferior cerebellar artery located distal to the branch feeding medulla oblongata is described. Trapping of the aneurysm with the aid of 2 clips was without any influence on neurological state of the patient and prevented recurrent hemorrhage. ...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1992-11-01 00:00:00
abstract::The authors describe a case of CJD in a man aged 45 years. The disease began with sluggishness of movements and speech difficulties, followed by development of pyramidal system damage and dementia. EEG findings were normal throughout the whole duration of the disease that is 3.5 years. Neuropathological examination di...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1982-01-01 00:00:00
abstract::A giant, unclippable right ICA aneurysm located partially intracavernously is reported with neck deriving at C3 level. The aneurysm caused optic chiasm compression, progressive left eye vision loss and exophthalmus as well as persistent headache. The presence of the aneurysm was first found in CT and confirmed by angi...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:2000-11-01 00:00:00
abstract::A 41-year-old man is described in whom after a febrile disease with disturbances of sleep rhytm and exessive somnolence, extrapyramidal signs appeared increasing progressively in intensity, with a hypertonic-hypokinetic syndrome with evidence of right-sided spastic hemiparesis and hypaesthesia. After treatment with Vi...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1977-09-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Synovial cysts of the spine occur most frequently in the lumbosacral region. Methods of treatment vary, but in cases of chronic pain or neurological deficits surgical intervention is undertaken. The aim of this paper is to present indications, surgical technique and efficacy of surgical treatment...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:10.5114/ninp.2012.31356
更新日期:2012-09-01 00:00:00
abstract::The authors present the results of surgical treatment of 48 aneurysms situated on the anterior communicating artery operated upon by the classic and microsurgical methods. The latter method has made it possible to raise the proportion of very good and good results from 23% in the classic method to 70%, and to reduce t...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1980-01-01 00:00:00
abstract::Complications of transpedicular stabilizations of thoraco-lumbar burst fractures are presented on the basis of literature review and own experiences. Unstable thoraco-lumbar burst fractures create the most difficult biomechanical conditions for a stabilizer. A literature review was done to estimate the effectiveness o...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章,评审
doi:
更新日期:2006-03-01 00:00:00
abstract::Idiopathic Parkinson Disease accounts for approximately 75% of all cases of parkinsonism. The described case of Corticobasal Degeneration (CBD), until now not presented in Polish medical literature is a relatively rare example of so-called "parkinson plus" syndrome. The authors present the case of a 56 years old woman...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1998-11-01 00:00:00
abstract::One of the most common children's ailments are headaches--based on literature--about 75% of the population under the age of 15 suffers from them. The aim of the study was to evaluate the influence of headache on the quality of life in children and adolescents. The study group was made up of 86 persons between the age ...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1999-01-01 00:00:00
abstract::An original method is presented used for production of experimental aneurysms in rats. The method may be useful in training for microneurosurgical treatment of aneurysms. ...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1992-01-01 00:00:00
abstract::The word "pain" is ambiguous, symbolic and it is understood differently by the patient and by the doctor or physiologist. It describes a kind of sensation evoked by harmful stimuli which is a physiological phenomenon indispensable for protection and also suffering caused by injury or disease. The second component of t...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章,评审
doi:
更新日期:1999-01-01 00:00:00
abstract:BACKGROUND AND PURPOSE:We present detailed results of using Neuroform stent-assisted coil embolization to treat complex cerebral aneurysms over a three-year period. MATERIAL AND METHODS:Only patients who underwent Neuroform stent-assisted coil embolization were included in this study. We assessed patients' history, an...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:10.1016/s0028-3843(14)60296-6
更新日期:2010-07-01 00:00:00
abstract::The authors describe a case of spinal intracanal cysticercosis. The cysts were situated intradurally, provoking radicular symptoms: pain, paresis. CT investigations showed herniation of discs L4/L5 and L5/S1 interpreted as the cause of the mentioned symptoms. Surgical treatment included discectomy L4/L5 and L5/S1. Rec...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:2000-07-01 00:00:00
abstract::On the basis of personal investigations including 1) electrophysiological methods (EEG, averaged evoked potentials), 2) behavior methods, 3) destruction of various parts of the brain (mesencephalic reticular formation, auditory pathways) and 4) pharmacological methods, conducted in the years 1953-1974 the author put f...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:1976-07-01 00:00:00
abstract::Acute lung injury (ALI), including acute respiratory distress syndrome (ARDS), is a critical condition consisting of acute hypoxaemic respiratory failure with bilateral pulmonary infiltrates of non-cardiogenic origin. Occasionally it develops in patients with acute or subacute central nervous system pathologies, such ...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:2009-11-01 00:00:00
abstract::140 patients with stroke (69 men and 71 women) aged 71.7 +/- 12.4 treated in the general neurology were compared with 102 patients (46 men, 56 women) aged 71.5 +/- 11.2 treated in the stroke unit during the period 1.11.95 to 31.10.97. Neurological status and state of environmental dependency during the discharge time ...
journal_title:Neurologia i neurochirurgia polska
pub_type: 杂志文章
doi:
更新日期:2000-05-01 00:00:00