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Biomedical subjects

J C Jacob

Publications and source records attributed to J C Jacob.

At least 19 recordsLinked to original sources
PubMed1996

Historical vignette: cerebral cortical stimulation and surgery for epilepsy.

In 1909, in an isolated community hospital, on the northern tip of the Province of Newfoundland and Labrador, Canada, Dr. John Mason Little, Jr. performed electrical stimulation of the cerebral cortex, prior to cortical excision, as treatment of recurrent cerebral seizures in three patients. Extracts from Dr. Little's written records of the clinical features, the neurosurgical procedures and cerebral cortical stimulation are summarised. A brief review of the contemporaneous history of neurosurgical procedures for epilepsy provides a prospective of Dr. Little's remarkable surgical virtuosity.

Canada↗
PubMed1995

Effect of carbamazepine and valproate on bone mineral density.

OBJECTIVE: To examine the effect of carbamazepine and valproate monotherapy on bone mineral density in children. METHODS: Axial (second, third, and fourth lumbar vertebrae) and appendicular (distal third of radius) bone mineral density was measured by dual-energy x-ray absorptiometry in 27 healthy children and 26 children with uncomplicated idiopathic epilepsy treated with either carbamazepine (n = 13) or valproate (n = 13) for more than 18 months. Control subjects and patients were similar with respect to age, race (all white), and geographic area, and had no dietary restrictions, neurologic impairment, or physical handicaps. RESULTS: Subjects were seizure-free for more than 6 months on a regimen of carbamazepine or valproate therapy, and had mean serum trough levels of 6.88 +/- 2 micrograms/ml and 72.04 +/- 45.6 micrograms/ml, respectively. Dietary calcium intake was similar in control and treated groups. After correction for gender and age, children treated with valproate had a 14% (p = 0.003) and 10% (p = 0.005) reduction in bone mineral density at the axial and appendicular sites, respectively. The reduction in bone mineral density increased with the duration of valproate therapy. Carbamazepine did not significantly reduce bone mineral density. CONCLUSION: Valproate montherapy, but not carbamazepine therapy, significantly reduces axial and appendicular bone mineral density in children with idiopathic epilepsy and may increase their risk of osteoporotic fractures.

Absorptiometry, Photon↗
PubMed1993

Cervical myelopathy secondary to ossification of the posterior longitudinal ligament in a Caucasian patient.

Cervical myelopathy consequent on ossification of posterior longitudinal ligament (OPLL) is very rare in Caucasians. A 65-year-old Anglo-Saxon woman developed progressive gait disturbance, paresthesia in both legs and urinary urge incontinence. Radiological examination showed OPLL from fifth to seventh cervical vertebral level; the dense OPLL was graphically displayed by three-dimensional computerized tomography. Medial corpectomy, C5 to C7, and removal of OPLL, with subsequent fusion C4 to T1 using a free fibula graft resulted in clinical improvement. Three dimensional computerized tomographic imaging is a valuable diagnostic procedure in OPLL.

Aged↗
PubMed1990

Hemifacial spasm and craniovertebral anomaly.

Two patients with congenital anomaly of the craniovertebral junction causing disabling hemifacial spasm (HFS) are presented. In one patient, complete cessation of the HFS occurred for a period of two years following simple bony decompression of the craniovertebral junction raising unanswered questions as to the exact pathogenesis of HFS. Eventually both patients required microvascular decompression at the root entry zone of the facial nerve.

Adult↗
PubMed1988

The Newfoundland aggregate of neuronal ceroid-lipofuscinosis.

We have found a group of individuals with the late infantile, the early juvenile variant, and juvenile neuronal ceroid-lipofuscinosis (NCL) in Newfoundland, an island with a population of 500,000. In the past 25 yr, we have ascertained 44 cases of NCL in 32 sibships: 32 cases of late infantile NCL (LINCL) in 24 sibships, 11 cases of the early juvenile variant in 7 sibships, and one patient with the juvenile form (JNCL). The clinical presentation of the LINCL patients is very characteristic, with onset of seizures at age 2 1/2 to 3 1/2 yr, frequently with drop attacks and myoclonic jerks, followed by mental deterioration, ataxia, visual loss, and death by the end of the first decade. Typical curvilinear profiles are seen on electron microscopy (EM). The second group of patients mainly have the early juvenile variant with onset of seizures at age 5 to 6 yr and fingerprint profiles with occasional curvilinear profiles on EM. However, a child with the juvenile form presenting with blindness was also encountered. In both of these types, death occurs in the second decade of life. There is no overlap of these three clinical forms within sibships, although both late infantile and early juvenile variant types may occur in the same small fishing village. All three forms appear to be inherited as autosomal recessive traits. Although the early juvenile variant has been postulated to represent a double heterozygote between LINCL and JNCL, this cannot be confirmed on the basis of the present study.(ABSTRACT TRUNCATED AT 250 WORDS)

Child, Preschool↗
PubMed1986

Urinary sediment dolichols in the diagnosis of neuronal ceroid-lipofuscinosis.

Long-chain polyisoprenol alcohol (dolichols) levels are significantly increased in the urinary sediment of patients with infantile, late-infantile, and juvenile forms of neuronal ceroid-lipofuscinosis (NCL). The values in obligate heterozygotes for these diseases are similar to those in patients with other neurological diseases and in healthy controls. Antioxidant treatment of patients with juvenile NCL has no effect on dolichol values. The rate of false-negative results is 13.9% in infantile, 7.5% in late-infantile, and 15.0% in juvenile NCL. False-positive results were found in 8.2 to 14.3% of patients with other neurological diseases and in 15.4% of healthy controls. The test is of considerable value in the diagnosis of NCL and in decisions on whether to perform a biopsy. It is not useful in the screening of random samples, however.

Adolescent↗
PubMed1986

Primary malignant tumours of the trigeminal nerve.

We report a case of primary malignant schwannoma of the trigeminal nerve, and review the 5 previously reported cases. Clinical presentation, along with radiological and pathological features are discussed.

Combined Modality Therapy↗
PubMed1985

Friedreich's ataxia presenting as cardiac disease.

Three male children with Friedreich's ataxia, from a single family, are described. The first patient presented as a cardiologic problem with anginalike chest pain. He was found to have echocardiographic evidence of concentric left ventricular hypertrophy (LVH). He later developed ataxia. The younger brother also had LVH but was asymptomatic and later became ataxic. The elder brother was already ataxic at the time of diagnosis. To our knowledge this is the first report of echocardiographic concentric left ventricular hypertrophy preceding the neurologic syndrome of Friedreich's ataxia.

Cardiomyopathy, Hypertrophic↗
PubMed1984

Familial intracranial gliomas.

The pedigree of two interrelated families with 10 affected members suffering from malignant supratentorial gliomas is reported. In addition, three other unrelated families with two members each who were treated for different types of brain tumors are described. Genetic implications are discussed.

Adolescent↗
PubMed1982

Adult diastematomyelia: a complex dysraphic state.

It is rare for patients with diastematomyelia to manifest initial symptoms in adult life. Clinical, radiological, and surgical features of a patient with symptomatic adult diastematomyelia are presented. Pathogenetic mechanisms are considered to include a variety of congenital spinal anomalies, distinct from the spur, as well as mechanical factors.

Adult↗
PubMed1981

Spinal subdural hematoma in association with anticoagulant therapy.

A case of spinal subdural hematoma occurring in association with anticoagulant therapy is reported. Seven similar cases from the literature are reviewed the emphasis on the clinical features, investigation, and the results of treatment. The prognosis for recovery is good, only if the condition is diagnosed and the clot evacuated before severe spinal cord compression and subsequent ischemic necrosis has occurred.

Aged↗
PubMed1977

The ultrastructural characteristics of the abnormal cytosomes in Batten-Kufs' disease.

Patients with Batten-Kufs' disease may be divided into three groups by electronmicroscopy of their storage deposits. In the first group, those characterized by curvilinear profiles, there is a strong correlation with a particular clinical syndrome, the late infantile form of the disease. In the second group, characterized by finger-print profiles, there is great diversity as to age and type of presentation. This is paralleled by diversity in the deposits. To the third group belongs the infantile form of the disease, as well as rare patients with later onset. Pathological diagnosis can be reliably, conveniently and consistently made from biopsy of skin by electronmicroscopy, and usually from biopsy of skeletal muscle as well.

Age Factors↗