Rdzeniowy zespół twarzowo-palcowy: częsty zespół w nietypowej lokalizacji

Background and purpose Cheiro-oral syndrome (COS) is an established neurological entity characterized by a sensory impairment confined to the mouth angle and ipsilateral finger(s)/ hand. The current understanding of localization is a concomitant involvement of the spinothalamic and trigeminothalamic tract between the cortex and pons. The cervical spinal cord has not been mentioned in this situation yet, and this unusual location may heretofore increase the risk of misdiagnosis. Material and methods Six patients who presented with unilateral COS due to cervical cord disorder are reported. Results All patients were women and their age ranged between 42 and 70 years. Their neurological deficits included unilateral paraesthesiae restricted to cheiro-oral distribution, positive radicular sign, and mild change of tendon reflex. Cervical spinal stenosis at middle/lower cervical spine with variable magnitude of cord compression and intrinsic cord damage was found. A diagnostic dilemma obviously arises from the lack of tangible neurological signs or typical pattern of myelopathy, in addition to the previous concept of cerebral involvement. A benign course ensued in all reported patients. Conclusions Cheiro-oral syndrome can be an early neurological sign for cervical cord disorder; it further suggests that it is a strong neurological but weak localizing sign. A reciprocal influence of multiple factors is considered to generate COS at the cervical cord. Therefore, an absence of brain pathology should lead to a thorough examination of the cervical cord in case of COS.Wstęp i cel pracy Zespół twarzowo-palcowy (ZTP) jest znanym zespołem neurologicznym, który cechuje się niedoczulicą ograniczoną do kącika ust i palców ręki lub ręki po tej samej stronie. Bieżący stan wiedzy dotyczący lokalizacji uszkodzenia w tym zespole wskazuje na jednoczesne zajęcie dróg rdzeniowo-wzgórzowej i trójdzielno-wzgórzowej pomiędzy mostem i korą mózgową. W tym kontekście nie wymieniano dotąd uszkodzenia rdzenia kręgowego w odcinku szyjnym, a taka nietypowa lokalizacja uszkodzenia może zwiększyć ryzyko błędnego rozpoznania miejsca uszkodzenia. Materiał i metody W pracy przedstawiono dane dotyczące 6 pacjentek z jednostronnym ZTP spowodowanym występowaniem nieprawidłowości w obrębie rdzenia kręgowego. Wyniki Zakres wieku pacjentek wynosił od 42 do 70 lat. Objawy neurologiczne obejmowały jednostronne parestezje ograniczone do obszaru twarzy i ręki, objaw korzeniowy i niewielkie nieprawidłowości w zakresie odruchów głębokich. Stwierdzono występowanie zwężenia kanału kręgowego w środkowej lub dolnej części odcinka szyjnego z uciskiem rdzenia kręgowego i uszkodzeniem wewnątrz rdzenia kręgowego. Wątpliwości diagnostyczne wynikały z braku typowych objawów neurologicznych mielopatii i stwierdzenia lokalizacji zmian nieodpowiadającej wcześniejszym opiniom na temat mózgowego pochodzenia zespołu. U wszystkich opisywanych pacjentek przebieg schorzenia był łagodny. Wnioski Zespół twarzowo-palcowy może być wczesnym objawem neurologicznym nieprawidłowości w obrębie rdzenia kręgowego. Stanowi istotny objaw neurologiczny, ale jego wartość lokalizacyjna jest niewielka. Powstawanie ZTP wskutek uszkodzenia rdzenia kręgowego wynika z wzajemnych oddziaływań wielu czynników. W razie braku uchwytnej patologii mózgu należy w przypadkach ZTP przeprowadzić szczegółowe badania rdzenia kręgowego w odcinku szyjnym

Cerebral hemorrhagic infarction following cranioplasty in a shunted patient with tension pneumocephalus resulting from depressed skull and craniodural defect

SummaryA 34-year-old female sustained a severe traumatic brain injury that was treated with decompressive craniectomy and subsequent cranioplasty, then with ventriculoperitoneal shunt about 10 years previously. However, the skull flap was found to be depressed ever since. She was admitted to our hospital for a headache and left hemiparesis with sudden onset. The computed tomography scan displayed tension pneumocephalus in the right frontoparietal region. First, she underwent emergency burr hole drainage and placement of a subdural drain with external ventricular drainage tube. Then her symptoms improved considerably. Unfortunately, 6 months later she was admitted again to our hospital because of headache and left hemiparesis with sudden onset, and the brain computed tomography showed tension pneumocephalus in the right frontoparietal region. She underwent craniectomy to remove the previous depressed skull and simultaneous cranioplasty with Ti-Mesh. On the day of her operation, generalized seizure occurred and her consciousness deteriorated. The magnetic resonance imaging showed hemorrhagic infarction on both sides of the thalamus and the right parieto-occipital region. We think it probable that a sudden increase of cerebral blood flow in the cerebral hemisphere where the cranioplasty had been performed caused reperfusion injury and resulted in hemorrhagic infarction

Multi-parametric neuroimaging evaluation of cerebrotendinous xanthomatosis and its correlation with neuropsychological presentations

<p>Abstract</p> <p>Background</p> <p>Cerebrotendinous xanthomatosis (CTX) is a rare genetic disorder. Recent studies show that brain damage in CTX patients extends beyond the abnormalities observed on conventional magnetic resonance imaging (MRI). We studied the MRI and ^{99 m}Tc-ethyl cysteinate dimer single photon emission computed tomography (SPECT) findings of CTX patients and made a correlation with the neuropsychological presentations.</p> <p>Methods</p> <p>Diffusion tensor imaging (DTI) and 3D T1-weighted images of five CTX patients were compared with 15 age-matched controls. Voxel-based morphometry (VBM) was use to delineate gray matter (GM) and white matter (WM) volume loss. Fractional anisotropy (FA), mean diffusivity (MD), and eigenvalues derived from DTI were used to detect WM changes and correlate with neuropsychological results. SPECT functional studies were used to correlate with GM changes.</p> <p>Results</p> <p>Cognitive results showed that aside from moderate mental retardation, the patient group performed worse in all cognitive domains. Despite the extensive GM atrophy pattern, the cerebellum, peri-Sylvian regions and parietal-occipital regions were correlated with SPECT results. WM atrophy located in the peri-dentate and left cerebral peduncle areas corresponded with changes in diffusion measures, while axial and radial diffusivity suggested both demyelinating and axonal changes. Changes in FA and MD were preceded by VBM in the corpus callosum and corona radiata. Cognitive results correlated with FA changes.</p> <p>Conclusion</p> <p>In CTX, GM atrophy affected the perfusion patterns. Changes in WM included atrophy, and axonal changes with demyelination. Disconnection of major fiber tracts among different cortical regions may contribute to cognitive impairment.</p

Clinical significance of serological biomarkers and neuropsychological performances in patients with temporal lobe epilepsy

<p>Abstract</p> <p>Background</p> <p>Temporal lobe epilepsy (TLE) is a common form of focal epilepsy. Serum biomarkers to predict cognitive performance in TLE patients without psychiatric comorbidities and the link with gray matter (GM) atrophy have not been fully explored.</p> <p>Methods</p> <p>Thirty-four patients with TLE and 34 sex - and age-matched controls were enrolled for standardized cognitive tests, neuroimaging studies as well as measurements of serum levels of heat shock protein 70 (HSP70), S100ß protein (S100ßP), neuronal specific enolase (NSE), plasma nuclear and mitochondrial DNA levels.</p> <p>Results</p> <p>Compared with the controls, the patients with TLE had poorer cognitive performances and higher HSP70 and S100ßP levels (<it>p </it>< 0.01). The patients with higher frequencies of seizures had higher levels of HSP70, NSE and S100ßP (<it>p </it>< 0.01). Serum HSP70 level correlated positively with duration of epilepsy (σ = 0.413, <it>p </it>< 0.01), and inversely with memory scores in the late registration (σ = −0.276, <it>p </it>= 0.01) and early recall score (σ = −0.304, <it>p </it>= 0.007). Compared with the controls, gray matter atrophy in the hippocampal and parahippocampal areas, putamen, thalamus and supplementary motor areas were found in the patient group. The HSP70 levels showed an inverse correlation with hippocampal volume (R square = 0.22, <it>p </it>= 0.007) after controlling for the effect of age.</p> <p>Conclusions</p> <p>Our results suggest that serum biomarkers were predictive of higher frequencies of seizures in the TLE group. HSP70 may be considered to be a stress biomarker in patients with TLE in that it correlated inversely with memory scores and hippocampal volume. In addition, the symmetric extratemporal atrophic patterns may be related to damage of neuronal networks and epileptogenesis in TLE.</p