Technical aspects and current clinical applications of the skin and skeletal muscle biopsy in neurological diagnostics: an overview.

This short overview recalls the basic principles and technical aspects of skin and skeletal muscle biopsies in humans with paying special attention to the stages of these procedures essential for further correct morphological diagnosis. Some of these principles may also be useful in animal experimental studies. The authors emphasize the important role of proper thickness of the skin fragment, proper orientation of muscle fibres and a scalpel during skin biopsy, and proper concentration of fixatives. They recommend avoiding anaesthesia of the skeletal muscle itself and using forceps carefully so as not to crush the epidermis.

Usefulness of the FOUR scale to assess the state of consciousness of the patients treated in the Intensive Care Unit.

The Glasgow Coma Scale (GCS) is a widely adopted clinical scale which fails to assess a verbal component in intensive care units (ICUs) patients who are ventilated mechanically. AIM: The aim of the study was to evaluate the validity of the FOUR scale (Full Outline of UnResponsivness) when applied by staff of an ICU. MATERIALS AND METHODS: This prospective cohort study included 65 consecutive intubated patients treated in the ICU with brain damage. The study design included the simultaneous assessment of the patients' consciousness by two scales - the FOUR scale and the GCS. All assessments were made under strict inclusion and exclusion criteria. The patients were assessed by a randomly selected staff pairs (neurointensivist/ nurse, neurointensivist/resident, neurointensivist/neurointensivists). The assessments made by experienced neurointensivists were regarded as basic examinations and by other members as control examinations. The study design allowed for the assessment of the interrater reliability. RESULTS: In 65 enrolled patients, a total of 763 double assessments were made using the FOUR score and by the GCS. It has been shown that in 751 out of 763 assessments the patients' consciousness was accurately assessed, representing 98.43 % accuracy of the FOUR scale. In contrast, the patients' consciousness was reliably assessed by the GCS in only 88 assessments, representing 11.5 % of accuracy. CONCLUSIONS: The study showed excellent interrater reliability among the groups of examiners for the FOUR scale as well as the GCS. The main advantage of the FOUR scale in intubated patients is excellent accuracy. The FOUR scale is easy to learn. It is functional and it has no limitations typical of verbal scales. It is a valuable and recommended clinical tool for the assessment of consciousness of the ICU patients.

Paraneoplastic brainstem encephalomyelitis and atypical form of chronic inflammatory demyelinating polyneuropathy in patient with testicular germinal tumor-is this an overlap syndrome? a case report.

Paraneoplastic neurologic syndromes are diagnosed when neurologic symptoms are associated with neoplasm and other causative factors are excluded. They may precede or be simultaneous to various types of neoplasms, mainly malignant. In men up to 45-50 years old the most common cancer causing the paraneoplastic syndrome is testicle tumor, manifesting usually as limbic/brain stem encephalitis and myelitis. Usually effective treatment of underlying neoplasm brings resolution of neurologic symptoms. But corticosteroids and intravenuous immunoglobulins are also used. In the presented case a 37-year-old man was primarily diagnosed and treated for progressive tetraparesis with signs of both upper and lower motor neuron dysfunction, associated with bulbar symptoms. Having various diagnostic procedures performed an atypical form of chronic inflammatory demyelinating polyradiculoneuronopathy was primarily suspected, but eventually a discovery of endodermal sinus tumor in the testicle enabled to state the diagnosis of possible paraneoplastic syndrome. In spite of chemotherapy the patient died shortly after the diagnosis because of infectious complications. Histopathology displayed intense inflammatory changes in the brain stem as well as in cranial nerves and cervical spinal cord. The same immunological process evoked by various pathogenetic factors (infection vs. neoplasm) may cause similar clinical picture and hinder the diagnosis. Most importantly it may delay the proper way of treatment.

Multiple metastatic intracranial lesions associated with left atrial myxoma.

BACKGROUND: One of the most common cardiac tumors is myxoma. Despite its predominantly benign course, diverse cardiological, systemic as well as neurological complications have been reported. CASE REPORT: We are the first from Poland to present the case of a patient with multiple central nervous system metastases associated with the left atrial myxoma. Various diagnostic, neuroradiological and histopathological procedures were described. The patient underwent cardiac surgery. CONCLUSIONS: Follow-up studies excluded the recurrence of the heart tumor and confirmed partial resolution of brain metastases. Nevertheless, subsequent neurological assessment was advised according to the literature data and possible late relapses mainly due to cerebral emboli.

Enlargement of the Nissl substance as a manifestation of early damage to spinal cord motoneurons in amyotrophic lateral sclerosis.

AIMS: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of motoneurons. Recent studies indicate that in ALS, degeneration of motoneuron body is late in comparison to degeneration of axons. The morphological consequence of the axonal damage is chromatolysis. Therefore, loss of tigroid in motoneurons as a morphological manifestation of chromatolysis should be a prominent feature seen in an early stage of the disease. To verify that assumption we examined morphologically spinal cord motoneurons in patients with sporadic ALS. MATERIAL AND METHODS: In anterior horn motoneurons of 33 patients tigroid were assessed at light microscopy and morphometrically analyzed. Material was divided into an "acute" ALS group with a duration of the disease of up to 1 year, and a "chronic" ALS group with a clinical course lasting for 4 - 9 years. RESULTS: In the "acute" ALS group, loss of motoneurons was slight, and only a part of them showed central chromatolysis. Instead of chromatolysis the enlargement of the tigroid was found. This phenomenon was observed only in "acute" ALS and confirmed by morphometric analysis. CONCLUSIONS: In ALS, enlargement of the tigroid seems to be an early morphological feature - occuring earlier than central chromatolysis. Its presence may be connected with endoplasmic reticulum stress, disturbed axonal transport or functional compensation of the neuronal deficit.