23 research outputs found
Ecchordosis physaliphora – opis przypadku i omówienie patologicznych pochodnych struny grzbietowej
Some notochord cells remain along the axis of the vertebral column after embryogenesis. These ‘notochordal remnants’ have some similarities, but their biological behaviour varies considerably. They can give rise to benign lesions such as ecchordosis physaliphora (EP) and ‘benign notochordal cell tumour’ (BNCT), or aggressive ones like chordoma.
We review the problems of the differential diagnosis of notochordal remnants apropos of a case of the incidental autopsy finding of EP in a 78-year-old man, who died due to heart infarction. The 6-mm asymptomatic gelatinous lesion was fixed to the basilar artery on its ventral aspect.
Small EPs can be easily overlooked in autopsy. Ecchordosis physaliphora and intradural chordoma share some similarities that may be misleading and may even result in the wrong diagnosis and therapy. The recently reported new entity BNCT poses a similar problem. We review the literature illustrating the most important features of notochord-derived lesions and discuss the relationships between these lesions with regard to molecular genetics.Podczas formowania się jąder miażdżystych na pozostałościach komórek struny grzbietowej rozwijają się zmiany podobne do siebie, mające jednak różny przebieg kliniczny. Niektóre z nich, takie jak ecchordosis physaliphora (EP) i notochordal benign cell tumour (NBCT), mają charakter łagodny, inne (np. struniak) – agresywny. Celem pracy jest przegląd najważniejszych cech zmian wywodzących się ze struny grzbietowej i ich diagnostyka różnicowa na podstawie przypadku EP stwierdzonego podczas autopsji mózgu 78-letniego mężczyzny zmarłego na atak serca. Galaretowaty guz o największym wymiarze 6 mm, przytwierdzony do brzusznej części tętnicy podstawnej nie dawał żadnych objawów.
Ecchordosis physaliphora to zazwyczaj mały, bezobjawowy guzek, łatwy do przeoczenia podczas autopsji. Struniak i EP wywodzą się z tych samych komórek, lecz ze względu na podobieństwa mogą być ze sobą mylone, co skutkuje niewłaściwym rozpoznaniem i ewentualnym leczeniem. Podobny problem występuje w przypadku NBCT. W pracy poruszono również kwestię ich wzajemnego związku na podstawie badań genetycznych
"Ecchordosis physaliphora" : opis przypadku i omówienie patologicznych pochodnych struny grzbietowej
Podczas formowania się jąder mia¿d¿ystych na pozostałościach komórek struny grzbietowej rozwijają się zmiany podobne do siebie, mające jednak różny przebieg kliniczny. Niektóre znich, takie jak ecchordosis physaliphora (EP) i notochordal benign cell tumour(NBCT), mają charakter łagodny, inne (np. stru- niak) - agresywny. Celem pracy jest przegląd najważniejszych cech zmian wywodzących się ze struny grzbietowej iich diagnostyka różnicowa na podstawie przypadku EP stwierdzonego podczas autopsji mózgu 78-letniego mężczyzny zmarłego na atak serca. Galaretowaty guz o największym wymiarze 6 mm, przytwierdzony do brzusznej części tętnicy podstaw- nej nie dawał żadnych objawów.
Ecchordosis physaliphora to zazwyczaj mały, bezobjawowy guzek, łatwy do przeoczenia podczas autopsji. Struniak iEP wywodzą się z tych samych komórek, lecz ze względu na podobieństwa mogą być ze sobą mylone, co skutkuje niewłaściwym rozpoznaniem i ewentualnym leczeniem. Podobny problem występuje w przypadku NBCT. W pracy poruszono również kwestię ich wzajemnego związku na podstawie badań genetycznych.Some notochord cells remain along the axis of the vertebral
column after embryogenesis. These ‘notochordal remnants’
have some similarities, but their biological behaviour varies
considerably. They can give rise to benign lesions such as
ecchordosis physaliphora (EP) and ‘benign notochordal
cell tumour’ (BNCT), or aggressive ones like chordoma.
We review the problems of the differential diagnosis of notochordal remnants apropos of a case of the incidental autopsy
finding of EP in a 78-year-old man, who died due to heart
infarction. The 6-mm asymptomatic gelatinous lesion was
fixed to the basilar artery on its ventral aspect.
Small EPs can be easily overlooked in autopsy. Ecchordosis
physaliphora and intradural chordoma share some similarities that may be misleading and may even result in the wrong
diagnosis and therapy. The recently reported new entity
BNCT poses a similar problem. We review the literature illustrating the most important features of notochord-derived
lesions and discuss the relationships between these lesions
with regard to molecular genetics
Saccadic eye movements in juvenile variant of Huntington disease
Background and purpose
Huntington disease (HD) is a neurodegenerative disease leading to involuntary movements, cognitive and behavior decline. The juvenile variant of HD (JHD) manifests in people younger than 21 and is characterized by a different clinical presentation, i.e. rigidity and bradykinesia. Rapid eye movements were not extensively studied in patients with JHD. Aims of our study were to describe the saccadic eye movements in JHD patients and to find a correlation between the saccade abnormalities, severity of the disease and cognitive and behavior deterioration.
Materials and methods
We studied 10 patients with JHD and 10 healthy subjects. Reflexive and volitional saccades were assessed with the Saccadometer Advanced. The battery of cognitive and behavior tests was performed as well.
Results
We found a prolonged latency, slowness and decreased velocity of reflexive and voluntary saccades and reduced amplitude of voluntary saccades. Moreover, patients with JHD executed a significantly lower number of volitional saccades and made more incorrect cued saccades than controls. We noted a significant correlation between prolonged latency of reflexive saccades with gap task and disease severity and significant inverse correlation between prolonged latency of reflexive saccades with overlap task, an increased number of incorrect saccades made on a cue and impairment in working memory.
Conclusion
Abnormalities of saccade eye movements in patients with JHD were similar to those reported in patients with HD. Our findings did not confirm abnormalities previously reported in patients with early onset HD. Abnormal saccade parameters correlated also with a disease severity and cognitive deterioration
Small volume of the posterior cranial fossa and arterial hypertension are risk factors of hemifacial spasm
Objectives
So far, there are only two studies evaluating the relation between the small volume of the posterior cranial fossa (VPCF) and the occurrence of HFS, both on Asian population. The aim of the study was to determine small VPCF and arterial hypertension (AH), as risk factors for hemifacial spasm (HFS) and their relation to neurovascular conflict (NVC) in Polish Caucasian-origin patients.
Materials and methods
The study included 60 patients with idiopathic HFS and 60 healthy volunteers matched by sex and age. AH was defined according to WHO. The VPCF measured the volume of the prepontine, prespinal and both cerebellopontine angle cisterns in MRI scans.
Results
There were no significant differences between occurrence of AH and the VPCF of patients and controls but the mean VPCF in women was significantly smaller than in men, In the multivariate regression analysis model only NVC was the statistically significant. In the subgroup of >50-year-old patients the most dominant risk factor was NVC (OR 71.09; 95% CI 21.08–239.77; p=0.0000), followed by the AH duration (OR 1.07; 95% CI 1.00–1.16; p=0.047). In the subgroup of <50 years, NVC was also the dominant risk factor, followed by the lower VPCF (Walad test: OR 0.4; 95% CI 0.16–1.04; p=0.045).
Conclusion
There was no significant difference in VPCF and in frequency of AH diagnosis in HFS patients and age- and sex-related controls, but the logistic regression analysis showed that small VPCF and AH duration are risk factors of HFS in younger and older patients respectively
Saccadic eye movements in juvenile variant of Huntington disease
Background and purpose: Huntington disease (HD) is a neurodegenerative disease leading to
involuntary movements, cognitive and behavior decline. The juvenile variant of HD (JHD)
manifests in people younger than 21 and is characterized by a different clinical presentation,
i.e. rigidity and bradykinesia. Rapid eye movements were not extensively studied in patients
with JHD. Aims of our study were to describe the saccadic eye movements in JHD patients
and to
find a correlation between the saccade abnormalities, severity of the disease and
cognitive and behavior deterioration.
Materials and methods: We studied 10 patients with JHD and 10 healthy subjects. Reflexive
and volitional saccades were assessed with the Saccadometer Advanced. The battery of
cognitive and behavior tests was performed as well.
Results: We found a prolonged latency, slowness and decreased velocity of reflexive and
voluntary saccades and reduced amplitude of voluntary saccades. Moreover, patients with
JHD executed a significantly lower number of volitional saccades and made more incorrect
cued saccades than controls. We noted a significant correlation between prolonged latency
of reflexive saccades with gap task and disease severity and significant inverse correlation
between prolonged latency of reflexive saccades with overlap task, an increased number of
incorrect saccades made on a cue and impairment in working memory.
Conclusion: Abnormalities of saccade eye movements in patients with JHD were similar to
those reported in patients with HD. Our
findings did not confirm abnormalities previously
reported in patients with early onset HD. Abnormal saccade parameters correlated also with
a disease severity and cognitive deterioration
Small volume of the posterior cranial fossa and arterial hypertension are risk factors of hemifacial spasm
Objectives: So far, there are only two studies evaluating the relation between the small
volume of the posterior cranial fossa (VPCF) and the occurrence of HFS, both on Asian
population. The aim of the study was to determine small VPCF and arterial hypertension
(AH), as risk factors for hemifacial spasm (HFS) and their relation to neurovascular conflict
(NVC) in Polish Caucasian-origin patients.
Materials and methods: The study included 60 patients with idiopathic HFS and 60 healthy
volunteers matched by sex and age. AH was defined according to WHO. The VPCF measured
the volume of the prepontine, prespinal and both cerebellopontine angle cisterns in MRI
scans.
Results: There were no significant differences between occurrence of AH and the VPCF of
patients and controls but the mean VPCF in women was significantly smaller than in men, In
the multivariate regression analysis model only NVC was the statistically significant. In the
subgroup of >50-year-old patients the most dominant risk factor was NVC (OR 71.09; 95% CI
21.08–239.77; p = 0.0000), followed by the AH duration (OR 1.07; 95% CI 1.00–1.16; p = 0.047).
In the subgroup of <50 years, NVC was also the dominant risk factor, followed by the lower
VPCF (Walad test: OR 0.4; 95% CI 0.16–1.04; p = 0.045).
Conclusion: There was no significant difference in VPCF and in frequency of AH diagnosis in
HFS patients and age- and sex-related controls, but the logistic regression analysis showed
that small VPCF and AH duration are risk factors of HFS in younger and older patients
respective
Deep brain stimulation in the treatment of Holmes tremor – A long-term case observation
We present the patient with Holmes tremor secondary to the infarction of thalamus, successfully treated with the deep brain stimulation (DBS) of the area between ventralis oralis anterior and zona incerta for a long time, in whom the severe tremor reappeared after removal of the DBS lead. This is the first presentation of the effective DBS on this location. Our case does not support the hypothesis that the DBS treatment could lead to sustained relief of symptoms after cessation of stimulation
Is hypertension a risk factor of hemifacial spasm?
Objectives
The published data on the relation between arterial hypertension (AH) and hemifacial spasm (HFS) are controversial. The aim of the study was to determine the prevalence of AH in HFS patients and the relation of AH and compression of the brainstem at the region of vasomotor center.
Materials and methods
The study included 60 of primary HFS patients and 60 healthy controls matched by age. AH was defined according to WHO criteria. The vessel compression of the brainstem was measure on MRI scans in selected region of vasomotor center located in the ventro-lateral medulla (VLM), between the pontomedullary junction, retro-olivary sulcus and the root entry zone (REZ) of the IX and X nerves. Modeling and compression severity of the VLM was graded in the 0–3 scale.
Results
The prevalence of AH in HFS patients did not differ significantly from the control group (61.6% vs 45.0%, p=ns). VML compression by vessel was frequently found in HFS patients with AH than without AH (97.2% vs 60.9%, χ2=11.0, p=0.0009). A similar relation was also found in the control group. The higher rate of VML vascular compression was related to the presence of AH in both, HFS patients and control group.
Conclusion
The prevalence of AH in HFS patients does not differ from controls. The VLM compression in HFS patients and controls is related to AH diagnosis. The association between AH and VLM compression is stronger in patients with higher degree of VLM compression
Deep brain stimulation in the treatment of Holmes tremor : a long-term case observation
We present the patient with Holmes tremor secondary to the infarction of thalamus,
successfully treated with the deep brain stimulation (DBS) of the area between ventralis
oralis anterior and zona incerta for a long time, in whom the severe tremor reappeared after
removal of the DBS lead. This is the first presentation of the effective DBS on this location.
Our case does not support the hypothesis that the DBS treatment could lead to sustained
relief of symptoms after cessation of stimulatio
Assessment of patient knowledge and awareness of the occurrence of addiction
Introduction. Drug treatment and substance abuse treatment centers are mostly involved in the treatment of alcohol or drug addicts. At present, an increasing number of patients depend on several psychoactive substances simultaneously. These people have a phenomenon called mixed addiction.Aim. The aim of the study was an attempt to answer the question about the state of mind of psychiatric hospital patients about the occurrence of addiction.Material and methods. The study included a group of 100 patients in the Neuropsychiatric Hospital's Neuropsychiatric Ward and the SPZOZ Psychiatric Day Ward in Kraśnik. The survey was conducted between February and August 2017. The author's questionnaire method was fully anonymous.Results. Misuse of psychoactive substances is a serious social problem. The phenomenon of addiction is more common among men living in rural areas, working professionally in the age range of 31 to 40 years. The respondents acknowledge that their knowledge of narcotics is based on their experience and their friends.Conclusion. Respondents are aware of the occurrence of mixed dependency