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Biomedical subjects

M B Connolly

Publications and source records attributed to M B Connolly.

16 recordsLinked to original sources

Encephalopathy complicating Henoch-Schönlein purpura: reversible MRI changes.

A 10-year-old boy with Henoch-Schönlein purpura complicated by encephalopathy, transient cortical blindness, and a secondary generalized seizure is reported. Reversible changes in the posterior white and gray matter were seen on magnetic resonance imaging. Our patient illustrates uncommon neurologic manifestations of Henoch-Schönlein purpura. The nature and location of the lesions and the normalization of the patient's magnetic resonance imaging is consistent with a posterior predominant parieto-occipital encephalopathy and suggests that cerebral edema from blood-brain barrier breakdown may play a central role in the pathophysiology of the central nervous system symptomatology in some patients.

Appendectomy

Acquired brachial-plexus neuropathy in the neonate: a rare presentation of late-onset group-B streptococcal osteomyelitis.

Acquired brachial-plexus neuropathy outside the immediate neonatal period is uncommon. Pseudopalsy of a limb, associated with osteomyelitis, is well recognized. Acquired brachial-plexus neuropathy as the initial presentation of osteomyelitis of the humerus in the neonatal period is described. Three infants presented at 3, 15, and 21 days respectively, with acute monoplegia consistent with brachial-plexus neuropathy. The infants were afebrile and generally well. Initial radiographs of the humerus were normal and blood cultures grew group-B streptococcus in all infants. Nerve conduction studies were consistent with brachial-plexus neuropathy. Following intravenous antibiotics, there was complete recovery in all infants. Osteomyelitis of the humerus should be considered in infants in whom there are no overt signs of sepsis and who present with brachial-plexus neuropathy. Early diagnosis and appropriate treatment should result in a complete neurological recovery.

Brachial Plexus

Therapist interventions in early sessions of brief supportive-expressive psychotherapy for depression.

Although psychotherapy manuals provide treatment guidelines, detailed descriptions of therapist interventions in manual-guided therapies are lacking. The purpose of the present investigation was to evaluate the types of therapist interventions in Supportive-Expressive (SE) psychotherapy for depression by using a molecular method of assessment and then to compare the results with those attained with a molar method. Four percent of therapist statements per session early in treatment were interpretations, which most often focused on the patient's parents, significant others, and self in the present time frame. This molecular method for assessing therapist interventions did converge with the molar adherence/competence method.

Adult

Prospective, open-label, add-on study of lamotrigine in 56 children with intractable generalized epilepsy.

The role of lamotrigine (LTG) in childhood epilepsy is emerging. We evaluated the efficacy and adverse effects of LTG in an open, prospective study of 56 children with generalized epilepsies. Six (11%) children became seizure-free, and 24 (43%) had greater than 50% reduction in seizure frequency. LTG was effective against a broad range of generalized seizure types. Three of 15 patients with Lennox-Gastaut syndrome achieved complete seizure control and eight demonstrated 50 to 99% improvement in seizure control. Increase in seizures (7) and rash (5) were the most common side effects. After valproate was discontinued, LTG therapy was resumed, with no recurrence of rash in any patient. This study suggests that LTG may be a useful drug in the treatment of generalized epilepsies in children.

Adolescent

Varieties of transference patterns in psychotherapy.

This investigation explored the nature of transference of interpersonal patterns in patients' psychotherapy narratives. The relation between interpersonal patterns with significant others in a patient's life and the pattern with the therapist early in treatment was examined. Cluster analysis was used to categorize similar relationships for each of 35 patients. Many patients revealed multiple interpersonal themes in their relationship narratives. Furthermore, these interpersonal themes correlated significantly with the interpersonal pattern extracted from narratives told about the therapist for many of the patients who discussed the therapeutic relationship during therapy. However, the interpersonal pattern evident in the relationship with the therapist was not necessarily the most pervasive pattern exhibited in the narratives about significant others.

Humans

Seizures involving the supplementary sensorimotor area in children: a video-EEG analysis.

Most reports of supplementary sensorimotor seizures have been of adults with medically refractory epilepsy. Typically, supplementary sensorimotor seizures have onset in childhood. We describe the electroclinical features in 12 children. Cases were selected from an EEG laboratory population in whom video-EEG was performed routinely on all children. Supplementary sensorimotor seizures were diagnosed when there was bilateral tonic posturing of the upper or lower extremities, preserved consciousness, and lack of postictal confusion. Sensory auras were reported by 8 of 9 children aged > 3 years who had daytime seizures. Speech arrest occurred in all patients in whom it could be assessed, and abnormal vocalization was observed in 7 children. Interictal EEGs were often normal (49% of recordings), and ictal EEG changes could be subtle. An etiology was demonstrated in only 2 children, and brain imaging studies were normal in the other 10 patients. Seizures were controlled with antiepileptic drugs (AEDs) in 6 of the 12 children. The clinical manifestations of supplementary sensorimotor seizures in children are similar to those reported in adults; misdiagnosis is common at all ages.

Age Factors

Rapid cycling in severely multidisabled children: a form of bipolar affective disorder?

A debilitating, regularly recurring, biphasic disorder is described in 6 severely multidisabled children. It was characterized by several days of lethargy, withdrawal, loss of abilities, irritability, and hypersomnolence followed or preceded by a high-energy state for several days during which the children slept very little, at times were euphoric, had improved mental ability, and were hyperactive. These cyclic episodes had been present for years but unexpectedly disappeared in one child. The etiology is unknown, in spite of detailed neurologic, metabolic, and endocrine investigations. All patients had family histories positive for affective disorder. Melatonin treatment helped to regulate the coexisting chronic sleep disorders of 3 children but failed to eliminate the cycles. Antiepileptic drug treatment, lithium, sedatives, stimulants, tranquilizers, and light therapy were largely ineffective. The children's symptoms and signs fit the diagnostic criteria of a bipolar affective illness, as it was modified for patients with associated neurologic disability; therefore, the patients appeared to have a unique disorder that closely resembles or is a variant of rapid cycling affective disorder.

Adolescent

Magnetic resonance imaging in pseudotumor cerebri.

The pathophysiology of pseudotumor cerebri is unclear, but may relate to an abnormality in water transport in the brain. The authors performed MR imaging in seven children with pseudotumor cerebri; the signal intensity in the white matter was normal in all patients. These data suggest that periventricular brain water content is not increased markedly in children with pseudotumor cerebri. The authors speculate that this may relate to the establishment of an equilibrium between increased resistance to cerebrospinal fluid outflow and increased brain stiffness, occurring as a consequence of increased cerebral blood-volume and/or interstitial pressure.

Adolescent

Hepatic dysfunction in Alström disease.

Alström disease is a rare disorder; less than 20 cases have been reported. An 11-year-old girl is described with this condition. She has pigmentary retinopathy, sensory neural deafness, obesity, Type II diabetes mellitus, hyperlipidemia, and acanthosis nigricans. However, in addition she developed hepatic dysfunction, pathologically similar to chronic active hepatitis. This may be a further, previously undescribed systemic manifestation of Alström disease.

Acanthosis Nigricans

Rapid recovery from cortical visual impairment following correction of prolonged shunt malfunction in congenital hydrocephalus.

Three children with congenital hydrocephalus are described in whom increased intracranial pressure was associated with severe, long-lasting cortical visual impairment. Following shunt revisions or reconstructive craniotomies, visual improvement began within hours. It is speculated that increased intracranial pressure can occasionally result in chronic hypoperfusion of the parietal-occipital lobes, either due to posterior cerebral artery compromise secondary to transtentorial pressure gradient or to direct compression of cerebral tissues.

Cerebrospinal Fluid Shunts

Head shaking by visually impaired children: a voluntary neurovisual adaptation which can be confused with spasmus nutans.

Rapid, horizontal, pendular head oscillations were observed in 18 visually impaired children with nystagmus during intense visual fixation, and the characteristics of this behaviour were analysed. Head tilting and eye deviations also occurred in 14 of the children. Their symptoms and signs resembled spasmus nutans. Head shaking appeared to be a voluntary, learned, neurovisual adaptation to improve visual acuity. Accurate simultaneous recordings of eye and head movements are required to understand the pathophysiological significance of these head oscillations.

Adolescent

Clinical features for prediction of survival in neonatal muscle disease.

Review of 17 newborns with muscle disease demonstrated that clinical features associated with survival beyond 1 year of age included gestational age of at least 35 weeks and requirement of mechanical ventilation for less than 21 days. In contrast, poor outcome was associated with Apgar scores below 5 at 5 min, pulmonary complications, arthrogryposis, or other congenital anomalies. The incidence of decreased fetal movements, polyhydramnios, hypotonia, and assisted delivery was not statistically different between infants who died early and those who survived beyond 1 year of age. Clinical features of newborns with muscle disease may be useful for prediction of outcome, especially when muscle biopsy abnormalities are nonspecific.

Biopsy

Outpatient video-EEG monitoring in children.

Video-EEG monitoring enables correlation of behavioral activity with EEG activity, which is useful in recognition of pseudoepileptic seizures and in investigation of patients for epilepsy surgery. Because most patients are monitored for a prolonged time as in-patients, the cost of the procedure is high. We investigated the value of brief (2-3 h) outpatient video-EEG monitoring in 43 children with frequent seizures, most of whom had symptomatic generalized epilepsy. Indications for monitoring included differentiation of epileptic from nonepileptic behavior, seizure classification, and determination of seizure frequency. Clinical episodes were recorded in 36 of 43 children (83%). A definite diagnosis was established in 9 of the 17 patients investigated to determine the nature of the clinical behavior. Seizures were classified in 15 of the 25 patients investigated to determine seizure type, and classification was different from the original in 9 of the 15 children. A change in epilepsy syndrome classification was made in 9 children. The video-EEG allowed diagnosis in 25 of the 43 children (59.5%). Video-EEG appears to be an effective method for outpatient investigation of children with frequent seizures, particularly those with symptomatic generalized epilepsy.

Adolescent