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Parental age, genetic mutation, and cerebral palsy.

Parental age and birth order were studied in 251 patients with cerebral palsy. No parental age or birth order effects were observed in spastic quadriplegia or diplegia, but a paternal age effect was detected in those with athetoid/dystonic cerebral palsy and congenital hemiplegia. These observations indicate that some cases of athetoid/dystonic or hemiplegic cerebral palsy might arise by fresh dominant genetic mutation.

Adolescent↗

Neurosurgery in cerebral palsy.

Stereotaxic subthalamotomy of 55 patients with cerebral palsy gave a good result in 65% of the selected cases. The result was uncertain in 15 and poor in 20%. An independent socio-medical follow-up study confirmed the clinical finding. The more rapid the involuntary movements, the better was the effect of subthalamotomy. Intention tremor was the most suitable symptom for stereotaxic treatment. Rigidospasticity was alleviated in the distal muscles only. Speech and gait were often improved. Good results were obtained in patients with normal intelligence, while feeble-minded patients did not benefit from the operation. Subthalamotomy was followed by transitory side-effects in 20%, of which increased involuntary movements on the ipsilateral side and mental restlessness were the most frequent. The operation had no mortality. Spinal longitudinal myelotomy effectively relieved the spasticity of the lower limbs in the three patients operated upon.

Adolescent↗

Voluntary and reflex control of the biceps brachii muscle in spastic-athetotic patients.

A cross-correlation technique of analysis was used to measure the transmission characteristics of tonic stretch reflex (TSR) pathways in spastic-athetoid subjects sustaining a voluntary contraction in the biceps brachii muscle. A comparison was made with the transmission characteristics of normal subjects measured by the same technique. It was found that gain and phase characteristics of spastic patients did not display the large resonant peaks present in normals. It is proposed that the resonant peaks in the TSR transmission of normal subjects were caused by long loop pathways. The absence of these peaks in the spastic patients supports the hypothesis that short-circuiting of long loop pathways by hyperactive spinal reflexes is part of the mechanism of spasticity.

Arm↗

Voluntary control of arm movement in athetotic patients.

Visual tracking tests have been employed to provide a quantitative description of voluntary control of arm movement in a group of patients suffering from athetoid cerebral palsy. Voluntary control was impaired in all patients in a characteristic manner. Maximum velocity and acceleration of arm movement were reduced to about 30-50% of their values in normal subjects and the time lag of the response to a visual stimulus was two or three times greater than in normals. Tracking transmission characteristics indicated a degree of underdamping which was not presnet in normal or spastic patients. This underdamping could be responsible for a low frequency (0·3-0·6 Hz) transient oscillation in elbow-angle movements associated with sudden voluntary movement. The maximum frequency at which patients could produce a coherent tracking response was only 50% of that in normal subjects and the relationship between the electromyogram and muscle contraction indicated that the mechanical load on the biceps muscle was abnormal, possibly due to increased stiffness of joint movement caused by involuntary activity in agonist and antagonist muscles acting across the joint.

Athetosis↗

CSF studies on the relationship between dopamine and 5-hydroxytryptamine in Parkinsonism and other movement disorders.

In Parkinson's disease, the concentration of homovanillic acid (HVA) was reduced in lumbar CSF from patients with idiopathic Parkinsonism (n = 54, P less than 0.05) and post-encephalitic Parkinsonism (n = 19, P less than 0.01). The reduction in the concentrations of 5-hydroxyindolylacetic acid (5-HIAA) was not significant, and there was no alteration in the levels of 4-hydroxy-3-methoxyphenylethylene glycol (MHPG). Treatment with L-dopa increased the concentration of HVA in the CSF (P less than 0.05) but had no effect on the levels of 5-HIAA and MHPG. Carbidopa given in combinations with L-dopa produced similar CSF concentrations of dopa as did L-dopa alone but caused less than half the rise in HVA. Fourteen patients who became functionally independent on treatment with L-dopa had higher 5-HIAA levels than 23 patients who showed no such improvement (P less than 0.001), suggesting that intact 5-hydroxyltryptamine neurones may be important in the therapeutic response to L-dopa. In a variety of movement disorders, the levels of HVA, 5-HIAA, and MHPG were not significantly different from age-matched controls. Treatment with tetrabenazine did not significantly alter the metabolite levels in patients in whom it produced either improvement, or side effects.

Aged↗

Paroxysmal choreoathetosis as a presenting symptom in idiopathic hypoparathyroidism.

A patient with idiopathic hypoparathyroidism presenting with spells of paroxysmal choreoathetosis is described. The possible mechanisms by which hypoparathyroidism induces choreoathetosis and other extrapyramidal motor dyfunctions are discussed. The need for screening patients with extrapyramidal disease for hypoparathyroidism is stressed.

Adolescent↗

Abnormal head movements.

Abnormal head movements have been studied in a variety of diseases using objective recording techniques and the data analysed with respect to the frequency content of the movement. Flopping, nodding, tic, chorea, myoclonic jerks, and most head tremors involve frequencies of approximately 2 and 4 Hz which correspond to the natural fundamental and second harmonic resonances of the head as determined by the mechanical properties of the head/neck system. These findings provide a basis for classification of abnormal head movements as well as an explanation of the characteristics of those arising from hypotonia of the neck muscles. The similarities between tremor frequencies and natural resonances suggest that in the case of the head, tremor arises from disorders of neural mechanisms normally responsible for the fine control of voluntary head movement and for stabilisation of the head during disturbance of posture. Head movements in cases of congenital nystagmus were found to be of two types. Some were of bizarre waveform, in no way assisted vision, and were taken to be of primarily pathological origin and classified as tremors. Others were learned adaptive responses which assisted vision either by interrupting the nystagmus, as in the case of spasmus nutans, or by compensating for the nystagmus with an inverse waveform and were called nodding. A prerequisite for true compensatory nodding is modified vestibulo-ocular reflex.

Adult↗

Central nervous system complications of Mycoplasma pneumoniae.

Three cases of central nervous system complications of Mycoplasma pneumoniae infections are reported. There were several atypical findings: the first patient presented with an acute disseminated encephalitis with normal cerebrospinal fluid and hypogammaglobulinaemia. The second patient presented with a left sixth nerve palsy followed six weeks later by choreoathetoid movements and an acute psychosis. The third patient presented with an acute encephalitis associated with acute renal failure secondary to rhabdomyolysis and was the only patient who did not completely recover; he was discharged akinetic and mute, but with normal renal function. The diagnosis of complications of Mycoplasma pneumoniae may be difficult. In two of the three cases a raised red blood cell volume secondary to a high titre of cold agglutinins rapidly led to the correct diagnosis.

Abducens Nerve↗

Focal paroxysmal kinesigenic choreoathetosis.

Three cases of paroxysmal kinesigenic choreoathetosis are described in whom unilateral attacks were focally induced, together with a case in whom bilateral attacks only occured. Treatment with phenytoin was effective in all cases. The aspects of the literature relating to focal and generalised attacks in paroxysmal kinesigenic choreoathetosis are reviewed.

Adolescent↗

Cervical spondylotic radiculo-myelopathy in patients with athetoid-dystonic cerebral palsy: clinical evaluation and surgical treatment.

The acute onset of symptoms of severe cervical radiculo-myelopathy in four patients with athetoid-dystonic cerebral palsy is reported. Neurological and radiological examination showed that the spondylotic changes of the cervical spine were responsible for new neurological deficits leading to the patients being bedridden. Dystonic-athetoid neck movements may cause excessive axial neck rotation as well as flexion and extension movements of the spine. These repetitive exaggerated movements may result in early degenerative changes of the vertebrae which may enhance the radiculo-myelopathy. The four patients were treated with an anterior discectomy with interbody fusion. They were bedridden pre-operatively but all have since been able to walk with or without a cane. It is concluded that early anterior decompression with interbody fusion is a treatment of choice for cervical spondylotic radiculo-myelopathy in association with athetoid cerebral palsy.

Adult↗