Subclinical rhythmic EEG discharge of adults: SREDA in two children.

Subclinical rhythmic electroencephalogram (EEG) discharge is an uncommon rhythmic EEG pattern that has been reported to occur in adults. It is thought to be a nonspecific finding with little clinical significance. This article reports this EEG pattern in two children and suggests it be called subclinical rhythmic EEG discharge of adults and children.

A study and review of developmental dysgraphia in relation to acquired dysgraphia.

A survey of developmental dysgraphia in a population of 259 normal 13- and 14-year-old schoolchildren has been carried out. The neurological characteristics of handwriting impairments in the 25 children with the poorest handwriting skills are described. These data form the basis of a clinical classification of developmental dysgraphia. A group of simple clinical tests of handwriting and associated functions are offered for routine use. Because they are based on neurological principles relating to acquired dysgraphia, the classification and testing format may be used for correlation with neuro-imaging in research studies. More importantly, they may also facilitate both counselling and remediation for a particular child. The test battery could be modified for use in epidemiological surveys of neurodevelopmental disorders.

Distinctive MRI findings in a case of neurocysticercosis.

OBJECTIVE: To report a case of cerebral neurocysticercosis, in which cranial magnetic resonance imaging (MRI) was performed at the time of probable death of the cysticercus. CLINICAL FEATURES: A 15-year-old Indian boy with a focal seizure disorder presented with a low-grade fever and an increase in seizure frequency. Cranial MRI scanning demonstrated a small, cortically based nodule surrounded by a low-intensity ring and extensive oedema. INTERVENTION AND OUTCOME: Carbamazepine therapy was continued (without steroids) and a further MRI scan after one month showed resolution of the oedema, and a subsequent cranial CT scan showed calcification of the cortically based nodule. Praziquantel therapy was administered. CONCLUSIONS: Diagnosis of neurocysticercosis must often rely upon clinical and distinctive radiological findings. Death of the cysticercus may provoke an inflammatory reaction that may result in an increase in seizure frequency.

Cavum septum pellucidum and obstructive hydrocephalus.

Five patients presented with symptoms related directly to pressure effects from their cavum septum pellucidum with persistent or intermittent obstructive hydrocephalus. The most characteristic presenting symptoms were intermittent postural headache and postural loss of consciousness. If cysts of the cavum septum pellucidum are symptomatic and stereotactic cyst puncture or fenestration are ineffective, ventriculoperitoneal shunting should be carried out before resorting to more radical excision of the cyst.

The clinical significance of type 1 fiber predominance.

In the course of investigating children with hypotonia, muscle biopsy of the vastus lateralis frequently demonstrates greater than 55% predominance of the aerobic type 1 fibers of "type 1 fiber predominance" (T1FP). The clinical significance of T1FP is not well known. T1FP can be associated with a variety of neurological disorders but a significant proportion has no apparent cause. We followed up 23 children with T1FP to establish whether a neurological disorder subsequently became apparent or whether a distinct clinical entity of T1FP could be identified. Sixty percent of the children were found to have a specific neurological disorder, while 40% did not and may represent a distinct clinical entity. The majority of children with the clinical entity of T1FP improved although some were left with mild motor disability. Such information is important for counseling when patients with T1FP are first assessed.

Familial cyanotic breath-holding spells.

Breath-holding spells can be a source of considerable parental anxiety and are a frequent cause of referral to paediatric services. This report is of a family of seven siblings all of whom have suffered breath-holding spells, including three who developed breath-holding spells within the neonatal period, with the other four developing them at 3-4 months of age. All of the children have on occasions exhibited convulsive activity at the termination of the episode. All had normal initial electroencephalogram's (EEG) and one had centrotemporal spikes on his EEG when repeated at age 6 years. Anticonvulsant therapy resulted in a reduced frequency of breath-holding spells. The epidemiology, pathophysiology and therapy of breath-holding spells is discussed with reference to this unique family. This report gives further credence to the notion that there often may be a familial predisposition to breath-holding spells.

Enigmatic trigeminal sensory neuropathy diagnosed by facial skin biopsy.

Facial paraesthesia due to perineural malignant infiltration is a well recognised complication of basal and squamous cell carcinomas of the head and neck. Perineural involvement was originally attributed to involvement of the perineural lymphatics; however subsequent studies have demonstrated conclusively that these lymphatics do not exist and that the invasion occurs along the line of least resistance. Previous studies on perineural spread of carcinomas of the head and neck have emphasised diagnostic biopsy of an involved nerve (e.g. the infraorbital, mental or major branches of the trigeminal nerve), or at times craniectomy with exploration of the gasserian ganglion. We suggest that in many cases the diagnosis can be obtained by biopsy of the anaesthetic skin alone, without recourse to more involved biopsy techniques. The following case report illustrates this point.

Benign vascular sexual headache and exertional headache: interrelationships and long term prognosis.

There is a definite relationship between the vascular type of benign sexual headache and benign exertional headache. Forty five patients with benign vascular sexual headache were reviewed. Twenty seven (60%) experienced benign vascular sexual headache alone and eighteen (40%) had experienced both benign vascular sexual headache and benign exertional headache on at least one occasion. The mean age was 34.3 years with a male:female ratio of 5.4:1. Thirty patients with a history of benign vascular sexual headache were followed for an average of 74 months. A personal history of migraine was found in 47% of cases and a family history of migraine in 30%. Forty one per cent of patients with benign vascular sexual headache alone had recurrences after diagnosis, and stress and fatigue were considered major contributing factors to the initial and recurrent headache. Nine patients had experienced benign vascular sexual headache and benign exertional headache within 72 hours of each other on at least one occasion, often with a residual headache between the two. Four patients experienced their benign vascular sexual headache and benign exertional headache separated by months to years. The prognosis of benign vascular sexual headache and the clinical and possible pathophysiological relationships between benign vascular sexual headache and benign exertional headache are discussed. Knowledge of the interrelationships of these varieties of headache is valuable in the counselling of patients.

Triphasic waves in accidental hypothermia.

Triphasic waves occur in a variety of metabolic, toxic and diffuse encephalopathies. We describe an elderly patient in whom triphasic waves accompanied moderately severe accidental hypothermia and disappeared on rewarming.

Mitochondrial encephalomyopathy with corticosteroid dependence.

A 12-year-old boy with corticosteroid-responsive mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) is described. His mother proved to have an asymptomatic mitochondrial myopathy on examination of a muscle biopsy specimen. Three weeks after the onset of vomiting, headache, ataxia and visual and speech impairment, he presented with a background of somatic growth retardation, deafness and school failure. Examination revealed disorientation, dysphasia, dyspraxia, optic atrophy, hemianopia, hemiparesis and sensory inattention. A cranial computed tomographic scan disclosed a large, low-density area, which was consistent with infarction, in the left posterior hemisphere and marked calcification of the basal ganglia bilaterally. Within two weeks of the commencement of corticosteroid treatment, the neurological dysfunction resolved. Attempts to decrease the dosage of dexamethasone caused an exacerbation of symptoms repeatedly. Two weeks after ceasing corticosteroid therapy, the patient developed a serious neurological relapse and a new, large, low-density area, which resembled an infarction, in the right posterior hemisphere on a computed tomographic scan. The reintroduction of corticosteroid therapy again resulted in the rapid resolution of all symptoms. It became evident that the patient had an exquisitely sensitive corticosteroid dependency, whereby a reduction in the dexamethasone dosage of even 0.25 mg a day caused confusion, headaches and increasing lactic acidaemia. Although it is difficult to assess the impact of various therapies in MELAS because of the episodic natural course of the disease, this remarkable corticosteroid responsiveness also has been noted in four previously reported patients with MELAS syndrome; therefore, it would seem reasonable to suggest that corticosteroid therapy now should be considered as standard treatment for this condition. However, corticosteroid therapy in other forms of mitochondrial disorders still awaits careful evaluation.

Long term survival with early childhood intracerebral tumours.

Three young adults are described who presented during early childhood with a seizure disorder due to an underlying intracerebral tumour. The tumours were excised incompletely 14-19 years later. The histological findings were those of a temporal lobe benign capillary haemangioblastoma (Case 1), parietal lobe subependymoma (Case 2), and parietal lobe ganglioglioma (Case 3). After a mean period of follow-up of 22 years (range 18-26), only mild residual physical disabilities exist in each patient. These three cases illustrate (1) the need promptly to investigate children who present with focal seizures or whose EEG shows definite focal abnormalities, (2) the relevant investigations should include cranial CT or MRI in such cases and (3) that certain supratentorial tumours have a favourable outcome due to their benign biological behaviour rather than their location.

Familial periodic ataxia.

A teen-age girl presented with recurrent stereotyped episodes of vertigo and ataxia and manifested constant horizontal gaze-evoked nystagmus. Cranial computed tomographic scans and metabolic biochemical screens gave normal results. The diagnosis of familial periodic ataxia was made from the family history and examination of the family members. Familial periodic ataxia is a rare disorder of cerebellar function which is inherited dominantly and has a benign prognosis. The underlying pathophysiology remains uncertain but a therapeutic response frequently is achieved with acetazolamide.

Compressive mononeuropathy of the deep palmar branch of the ulnar nerve in cyclists.

Two cyclists developed mononeuropathy of the deep palmar branch of the ulnar nerve due to ulnar nerve compression adjacent to the ulnar tunnel (of Guyon) by prolonged bicycle riding. A modification of hand grip on the bicycle handlebars resulted in rapid recovery in one patient.