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PubMedDate not supplied

Stiff-Baby--an unusual manifestation of cytoplasmic body myopathy: report of one case.

A 2-month-old male baby was admitted to our hospital with episodic cyanosis and respiratory failure which required mechanical ventilation. He was found to have upper limb flexion rigidity and poor weight gain since one month old. Progressive muscle stiffness over the abdomen, chest wall, back and four limbs were also noted. He could not be weaned from the ventilator smoothly due to recurrent CO2 retention. Laboratory tests revealed a high serum creatine kinase level. Cytoplasmic body myopathy was confirmed by muscle biopsy. The unusual initial presentations of generalized stiffness and early onset of respiratory failure were quite different from those of patients reported in the literature, who had floppiness, muscular atrophy and weakness. Prednisolone and Vigabatrin were given and the patient showed slight improvement in muscle stiffness and spontaneous movement.

Humans↗
PubMed1995

Parkinson's disease rigidity: EMG in a small hand muscle at "rest".

The presence of excessive EMG at "rest" might be an important factor in the genesis of Parkinson's disease (PD) rigidity, and we studied it in the first dorsal interosseous muscle (FDI) of 8 idiopathic PD patients. We had 8 age- and sex-matched normal controls. In the PD group, the average area of the surface EMG at "rest" correlated significantly with the clinical evaluation of rigidity and remained abnormally enhanced for 10-15 min after a command to "relax." Later, it tended to decline, but its entity was still much greater than in controls. The EMG "at rest" consisted of unwilled motor unit (MU) firing. A larger MU number was recruited in patients than in controls at "rest." MU rate coding was similar in both groups. Eventually, patients could get periods of EMG silence which, however, were interrupted by short EMG bursts, even if there was no muscle stretch. These bursts were interpreted as residual fragments of the original excessive EMG at "rest." MUs first recruited during such bursts showed high, but not total, overlapping with those first recruited by a gentle voluntary contraction or by a weak transcranial magnetic stimulus to motor cortex. We conclude that EMG activity at "rest" was made up of the discharge of low-threshold MUs, with a recruitment order similar to that resulting from descending cortico-spinal volleys. However, we cannot exclude other possible input sources to the alpha-motoneurones at "rest."

Aged↗
PubMed1982

Intracranial pressure and muscle activity in patients with decerebrate rigidity.

The authors analyse the relationship between the muscle activity during paroxysms of decerebration and the intracranial pressure. These relationships differ, and depend upon the presence or absence of mesencephalic herniation and patency of the perimesencephalic cisterns, as well as on whether the decerebration posturing has been spontaneous or induced by external stimulus. It is suggested that the magnitude of ICP reaction during decerebration is connected with the possibility of compensating the increased intracranial volume and with the strain imposed upon the brain stem at the mesencephalic level. Close correlations between the vegetative variables occuring during spontaneous DR paroxysms suggest a common excitatory neuronal mechanism. In cases when decerebration is provoked by external stimuli the response becomes more complex and not uniform.

Adult↗
PubMed2003

Burst activity of spiny projection neurons in the striatum encodes superimposed muscle tetani in cataleptic rats.

Catalepsy of animals represents a condition of akinesia and high rigidity of muscles. Catalepsy is a model for Parkinson's disease. The activity of spiny projection neurons in the striatum is correlated with different groups of muscles in rats or primates. Burst activity is a typical characteristic of spiny projection neurons in the state of Parkinson's disease. Rats were treated with 0.5 mg/kg haloperidol to induce a cataleptic state for 180 s. Spiny projection neuronal activity in striatum was measured to detect changes in spike frequency. Triggers were set manually when the rats increased their muscle tone superimposed on the already existing tetanus of the forelimb muscles. The results showed a clear correlation of the occurring triggers and the burst activity in the striatum. During such a burst event, rats did not move but showed this typical superimposed muscle tetanus. A possible explanation is that the intention to move is encoded in the burst activity of spiny projection neurons in the striatum while the execution of the movement is blocked. But also bursts that are not correlated with the short-term superimposed muscle tetanus occurred.

Action Potentials↗
PubMed1965

Flexor rigidity.

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Animals↗