William T. McLean

Recurrent thrombotic deterioration in the Sturge-Weber syndrome.

Hemiparesis, homonymous hemianopia and intellectual impairment are common features of the Sturge-Weber syndrome. While cerebrovascular thromboses have been noted with the syndrome, the pattern of recurrent thrombotic episodes producing an apparently gradual loss of function is not widely appreciated. The most important implication of this concept is the possibility of developing new methods of treatment, such as antiplatelet agents.

Regional cerebral blood flow characteristics of the Sturge-Weber syndrome.

Four patients with the Sturge-Weber syndrome were studied using the non-invasive Xenon-133 inhalation technique. All four patients had decreased regional cerebral blood flow in the area of their lesion, and in two patients who were subsequently tested with 5% carbon dioxide inhalation, impaired vasomotor reactivity was documented. Diminished regional cerebral blood flow is consistent with previously described nuclide flow studies which demonstrated a delay in the initial perfusion blush in the region of the abnormal vasculature. The focal decrease in blood flow was greatest in the most severely affected patient, but was also prominent in the two younger patients, both of whom have excellent neurologic function. These studies suggest that localized decrease in blood flow and vasomotor dysfunction in Sturge-Weber syndrome can precede the occurrence of severe neurologic impairment and extensive cerebral atrophy and possibly be a major contributing factor in progressive dysfunction. A secondary observation was that the blood flow in the unaffected hemisphere was significantly greater in two children compared to the two adults and was similar to the age-related differences reported for normal children and adults.

Wilson's disease: evoked potentials and computed tomography

SummaryMulti-modality evoked potentials and computed cranial tomography (CT) were performed in ten patients with Wilsons disease to determine if any of these studies would correlate reliably with neurologic status. While all four patients with CT abnormality had neurologic signs, two additional patients with neurologic findings had normal scans. Evoked responses were normal in nine patients. The remaining patient displayed abnormal visual, brainstem, and somatosensory evoked potentials, and follow-up studies after clinical deterioration revealed worsening of the brainstem and visual evoked potentials. This patient died unexpectedly from a subdural hematoma, and postmortem examination confirmed the radiographic findings of cortical atrophy of the cerebrum and cerebellum and bilateral cystic degeneration of the basal ganglia. However, localized demyelination in the visual, auditory, and sensory pathways was not present. We conclude that the clinical neurologic status of patients with Wilsons disease cannot be reliably predicted by either CT or multi-modality evoked potentials.

Cerebellar hemorrhage complicating isovaleric acidemia A case report

Isovaleric acidemia (IVA) is an inborn error of leucine metabolism, resulting in an accumulation of isovaleric acid in the body fluids. The neuropathologic findings in an 11-day-old infant with JYA consisted of diffuse cerebral edema, massive cerebellar hemorrhage, upward transtentorial herniation, and focal degeneration of clusters of glial cells in white and gray matter. Although abnormal mye-lination has been described in different aminoacidopathies, the above findings have not been reported previously in IVA.

A case for cerebral thromboangiitis obliterans.

The existence of cerebral thromboangiitis obliterans (CTAO) has been controrersial. The clinical, laboratory and angiographic features of a young woman with recurrent thrombophlebitis, digital gangrene and a bilateral anterior opercular syndrome (Foix-Charany-Marie) are reported. The cerebral angiognun demonstrated significant narrowing of fronto-opercular branches of both middle cerebral arteries. Histology of small digital muscular arteries rerealed segmental adventitial flbrosis, narrowing or occlusion of lumen and mild lyraphocitic infiltrates; occasional reins showed phlebitis. An etiologic relationship between cerebral occlusire disease and peripheral thromboangiitis obliterans (TAO) is suggested. Stroke, Vol 12, No 5, 1981

Early-onset myasthenia gravis

Signs of myasthenia gravis developed by age 3 years in 11 children. Six of these patients had persistent neonatal myasthenia gravis, a familial abnormality of neuromuscular transmission that is not immunologically mediated. Five patients had juvenile onset myasthenia gravis, an autoimmune disorder similar to myasthenia gravis in adults. Autoimmune myasthenia has rarely been recognized by age 3 years, but the presence of five cases in our series suggests that the disorder may be more common in young children than once believed. The development of anti-acetylcholine receptor antibody assays makes it easier to distinguish autoimmune myasthenia gravis from the congenital forms. This distinction is important, because the prognosis, treatment, and risk of recurrence in family members is different for each type of myasthenia.

Recovery from posttraumatic locked-in syndrome with basilar artery occlusion

Abstract A 43-year-old woman suffered mild trauma to the head and neck in an automobile accident and subsequently developed a locked-in syndrome. Arteriography revealed abnormal retrograde filling of the distal basilar artery. Dramatic improvement occurred over two months, and she eventually recovered neurologically. This case illustrates the need for early and appropriate medical management of patients with locked-in syndrome.

Intractable Hiccups as Presenting Symptom of Brainstem Tumor in Children

Hiccups are an unusual presenting symptom of brainstem tumors in children. An 8-year-old boy is described who presented with hiccups 2½ years prior to any major neurological manifestation. Central ner

Pupillary sparing oculomotor palsy from metastatic carcinoma

A 60-year-old male with adenocarcinoma of the rectum had the acute onset of complete oculomotor palsy with pupillary sparing. Computed tomography scan and sellar tomograms demonstrated an ipsilateral parasellar mass with bony destruction. This case illustrates the need for a thorough search for metastatic disease in patients with third nerve palsy and known carcinoma.

An Epilepsy Workshop for Professionals

Summary: This experimental study evaluated the impact of an epilepsy workshop format designed for community professionals who may be involved in the treatment plan and follow‐up of patients. The goals of the workshop were to increase the professionals knowledge and comfort in the presence of epilepsy and to effect better delivery of services to patients. To achieve this, a didactic approach to learning was modified to include patient contact and participant involvement in the learning process. A pre‐ and posttest experimental design was utilized. Those who attended the workshop improved significantly more than the two control groups in their knowledge of and comfort with epilepsy. Following the workshop, there was a marked increase in the referral of school children for possible neurological problems by teachers who had attended. There was a subsequent improvement in the delivery of health care services to such children in this county.