Symptoms of degeneration of the pyramidal tracts in conventional magnetic resonance imaging and diffusion tensor imaging in a young woman with primary lateral sclerosis.

Primary lateral sclerosis (PLS) is one of the forms of motor neuron disease (MND), affecting only upper motor neurons. The diagnosis of PLS should be made on different diagnostic criteria, for example, Pringle or Gordon, but it is usually a diagnosis of exclusion. There are no characteristic findings in standard laboratory and electrophysiological assessment. We present details of a 31-year-old woman who had suffered from progressive paraparesis with right-side predominance. Conventional MRI of brain and spinal cord and diffusion tensor imaging (DTI) studies showed Hyperintense lesions in the upper part of the cervical spinal cord at the level C1 in lateral funicules, in the medulla oblongata at the pyramidal decussation and in the midbrain. Brain DTI revealed changes along the corticospinal tracts on fractional anisotropy (FA) maps. MRI of the thoracic spinal cord showed in T2-weighted images hyperintensive regions in the course of the lateral corticospinal tracts. This aided in PLS recognition.

The clinical and neuroimaging studies in Holmes tremor.

AIM: Holmes tremor (HT) is a combination of rest, postural and action tremor. A parallel dysfunction of cerebello-thalamic and nigrostriatal pathways seems necessary to produce this kind of tremor. We present the clinical and neuroimaging study verifying that hypothesis. MATERIAL AND METHODS: A total of 10 patients: five male, five female, fulfilling consensus criteria were included. Demographic, clinical and neuroimaging data (MRI = 9; CT = 1, SPECT with the use of 123-I-FP CIT: DaTSCAN in six patients to assess the presynaptic dopaminergic nigrostriatal system involvement, indices of asymmetry for ligand uptake for each striatum were calculated) were analyzed. RESULTS: Hemorrhage was the most frequent etiology and thalamus - the most commonly involved structure. Contrary to the previous reports, the visual assessment did not reveal remarkable interhemispheric differences of DaTSCAN uptake. Quantitative measurements showed only minimal differences. CONCLUSIONS: It is open to debate whether nigrostriatal pathway damage is crucial for the phenomenology of HT. Alternative hypothesis is presented that HT represents the heterogeneous spectrum of tremors with similar phenomenology, but different pathophysiology.

Autonomic dysfunction in primary Raynaud's phenomenon.

AIM: The pathogenesis of Raynaud's phenomenon is still unclear. Neural and intravascular mechanisms are thought to be involved in the pathological process. The role of the autonomic nervous system is continually discussed, with particular attention to over-reactivity of the sympathetic part. The aim of this study was the clinical and electrophysiological analysis of autonomic nervous system function in patients with primary Raynaud's phenomenon. METHODS: Thirty four patients with primary Raynaud's phenomenon and 31 sex and age-matched controls were examined. Neurological examination, modified Low's Questionnaire, orthostatic and sustained handgrip tests, conduction velocity study in three nerves, sympathetic skin response (SSR), and heart rate variability (HRV) during deep breathing and at rest with the fast Fourier transform were performed. RESULTS: In the clinical examinations, 35.3% of the primary Raynaud's patients presented sensory neuropathy, but this was not confirmed in the standard conduction velocity tests. The modified Low's Questionnaire revealed dysautonomy in 82% of the patients. Autonomic regulation during the orthostatic and handgrip tests were within the normal limits. HRV at rest and the E/I ratio were significantly lower in the patient group than in the controls, while HRV spectrum analysis revealed the predominance of the low-frequency band in the patients. CONCLUSIONS: These results indicate the presence of sympathetic dysregulation and impairment of parasympathetic modulation of heart function in primary Raynaud's patients. The different cardiovascular and sudomotor functions are not affected to the same degree. These observations might support the theory of a central impairment of autonomic function in primary Raynaud's phenomenon. Peripheral nerve lesion as a coexisting cause of the observed dysautonomy remains uncertain.

Diffusion tensor imaging findings in the multiple sclerosis patients and their relationships to various aspects of disability.

BACKGROUND: The aim of the study was to assess microstructural changes within strategic brain regions in multiple sclerosis (MS) patients, using diffusion tensor imaging (DTI), with regard to various aspects of disability. MATERIAL AND METHODS: The study comprised 50 patients with relapsing-remitting MS (37 women, 13 men, mean age 36.4 yrs) and 27 age- and sex-matched controls. Using DTI, fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values were obtained within corpus callosum (CC), both thalami (TH) and middle cerebellar peduncles (MCP). Disability was assessed using Expanded Disability Status Scale (EDSS), MS Functional Composite (MSFC), Symbol Digit Modalities Test (SDMT) and Fatigue Severity Scale (FSS). DTI indices were compared between the patients and controls and in the MS group - referred to disability measures. RESULTS: Significant decrease in FA and increase in ADC within CC and both TH were found in MS patients compared to the controls. DTI indices within CC and TH correlated significantly with SDMT score, and within TH and MCP - with MSFC manual dexterity measure. CONCLUSIONS: Changes in DTI measures in normal appearing white and grey matter in the MS patients indicate subtle alterations of the tissue integrity. An occult damage to the strategic brain regions may contribute to various aspects of disability due to MS.

[Multiple intracranial meningiomas in a patient with asymptomatic syringomyelia]. / Mnogie oponiaki sródczaszkowe u chorej z bezobjawowa jamistoscia rdzenia kregowego.

A rare coexistence of multiple intracranial neoplasms of mesodermal origin with asymptomatic syringomyelia is reported. The clinical findings were documented by NMR and CT results. The histological examination of the tumours removed surgically is included.

[Dysembryoplastic neuroepithelial tumor (DNT)--case report and literature review]. / Dysembrioplastyczny nowotwór neuroepitelialny (DNT)--opis przypadku i przeglad pismiennictwa.

Neuroepithelial dysembryoplastic tumour was first described by Daumas-Duport in 1988 and in WHO classification was included into the group of neuronal and mixed neuroglial tumours. This is a benign and very rare tumor with a good prognosis occurring in children and young adults. The tumour caused characteristic clinical symptoms: epileptic fits, supratentorial, intracortical localisation, most often in temporal lobe and specific nodular architecture with heterogenic cell composition. Oligodendrocyte-like cells, glial and neuronal elements are usually found. The authors present a case of a 24-years old female with partial epileptic sensorial symptomatology. CT examination revealed a tumour in the left parietal lobe. Histological findings showed a typical texture of DNT. The tumour has no tendency for recurrence even in case of incomplete removal and does not require chemotherapy nor radiotherapy which is significantly important for accurate diagnosis, in order to avoid an aggressive therapy in young patients.

Peripheral nerve changes assessed by conduction velocity distribution in patients with primary Raynaud's phenomenon and dysautonomia.

AIM: Different mechanisms (neural and intravascular) are thought to be important in the pathogenesis of Raynaud's phenomenon (RP). In a previous study we confirmed autonomic nervous system impairment in patients with primary RP, but the pathogenic role of peripheral nerves remained unclear. The aim of the current study was an electrophysiological analysis of peripheral nerves using both standard conduction velocity and the conduction velocity distribution (CVD) in patients with primary RP in order to investigate the causes of dysautonomia. METHODS: We examined 34 patients with primary RP and dysautonomia and 31 sex- and age-matched controls. Standard motor and sensory conduction tests in ulnar and peroneal (sural) nerves and a CVD test in the same nerves were performed. RESULTS: Clinically, none of the patients had motor symptoms, while 35.3% of them presented sensory neuropathy. Standard neurographic tests were within the normal limits except for the significant prolongation of mean sensory latency in both examined nerves. CVD revealed significant slowing of motor conduction velocity in all the conduction values, e.g. in the 10th, 50th, and 90th percentiles of velocity. There were no differences in the width of the velocity distribution in the patient group and controls. CONCLUSION: The results of CVD indicated the presence of generalized subclinical peripheral motor nerve impairment (subclinical polyneuropathy) in patients with primary RP and dysautonomia. Based on the present and previous studies, we conclude that the mechanism of autonomic dysfunction in primary RP is mixed, resulting from both central and peripheral neural abnormalities.

[Dyskinesis after a single dose of thiethylperazine during the first trimester of pregnancy]. / Dyskinezy po jednorazowym zastosowaniu tietyloperazyny w pierwszym trymestrze ciazy.

A case of a 22-year female patient with poly-symptomatic reversible dyskinetic syndrome following a single thiethylperazine (Torecan) dose is presented. The patient was 10 weeks pregnant. Detoxication abolished extrapyramidal symptoms within 7 hours. Possible mechanism of such an adverse reaction to thiethylperazine is discussed.