Biomedical subjects
Publications and source records attributed to A K Thacker.
The occurrence of motor neurone disease (MND) in a Libyan couple who lived together for 40 years and in whom the disease developed within a 15-month period is reported. This is believed to be the second documentation of conjugal MND in the English literature.
A 4-year-search for spinal muscular atrophies (hereditary motor neuropathies, HMN) in Benghazi, Libya, yielded a total of 24 patients, among whom 18 were index cases. This group comprised 6 acute infantile, 12 chronic childhood, and 3 each with adult-onset proximal, and distal forms of the disorder. Distal HMN constituted 12.5% of the total cases. The crude average annual incidence of acute infantile HMN was 0.3/100,000 total population and 1/12,500 births in Benghazi. The crude prevalence rates of chronic childhood, adult-onset proximal, and distal types of HMN were 2.3, 0.6, and 0.6/100,000 respectively. The segregation ratios, 0.26 for acute infantile HMN and 0.24 for chronic childhood HMN, suggested autosomal recessive inheritance. The consanguinity rates among parents of cases and the population did not differ significantly.
Proximal conduction studies by F-wave technique, with conventional distal motor and sensory conduction were performed along the ulnar nerves of 20 patients each with cervical spondylotic radiculopathy and/or myelopathy and with classical motor neurone disease (MND). Such F-wave parameters as shortest F-latency, F-conduction velocity, conduction time and F-ratio were calculated. Twenty-five age- and sex-matched healthy volunteers acted as controls. Proximal slowing associated with sensory conduction abnormalities and normal distal motor conduction favored cervical spondylosis (CS). Distal slowing with a normal proximal motor and sensory conduction was associated with motor neurone disease.
During a 4-year study period, January 1983 to December 1986, 24 patients (18 index cases) with spinal muscular atrophy (hereditary motor neuropathy, HMN), 9 with myasthenia gravis (MG), 6 with progressive supranuclear palsy (PSP), and 5 with subacute sclerosing panencephalitis (SSPE) were diagnosed in Benghazi. The HMN group comprised 6 acute infantile, 12 chronic childhood, and 3 each with adult-onset proximal, and distal forms of the disease. The crude average annual incidence of acute infantile HMN was 0.3/100,000 total population and 1/12,500 births in Benghazi. The crude prevalence rates of chronic childhood, adult-onset proximal, and distal types of HMN were 2.3, 0.6 and 0.6/100,000, respectively. The larger family size and the high rate of consanguineous marriages contribute to the high frequency of HMN in the study area. Distal HMN constituted 12.5% of the total cases. The adjusted average incidence of MG was 4.4/million/year, 2.1 for males and 6.8 for females. The female:male incidence ratio was 3.2:1. The crude average annual incidence rates/million inhabitants for PSP asnd SSPE were 3 and 2.4, respectively. The frequency of occurrence of SSPE among the subtropical Arab community under investigation is comparable with other surveys from the Middle East and Mediterranean region.
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In this report a family is described where cerebellar ataxia occurred along with total albinism. A possibility of syntenic condition of the two genes responsible for the traits could explain the occurrence of the two conditions. The presence of two traits singly in the sibs reflects a recombinant event and suggests that the linkage is not absolute. Chromosomal study did not show any structural or numerical anomalies except in 1/50 metaphase plates scored in the proband (with cerebellar ataxia) where in chromosome No. 14 proximal intense band (21) seemed to be shifted, inv. (14) (q13q23?). The affected sibs with ataxia and albinism or ataxia alone depicted relatively a lower sister chromatid exchange (SCE) rate than their parents and age matched controls.
Twenty-eight consecutive male patients with primary and secondary hypogonadism (14 each) were evaluated clinically and electrophysiologically for muscle dysfunction. Although generalised muscle weakness was initially reported by only 9 patients, on direct questioning, it was recorded in 19. Objective weakness was found in 13 patients and it involved both the proximal and distal limb muscles. Quantitative electromyography showed evidence of myopathy in the proximal muscle in 25 patients, i.e., reduced MUP duration and amplitude with increased polyphasia in the deltoid and the gluteus maximus. There were no denervation potentials. None of the patients showed clinical neuropathy or NCV abnormalities. Thus, the profile of muscle involvement in hypogonadism closely simulates limb-girdle muscular dystrophy and other endocrine myopathies. The incidence of muscle involvement was higher in secondary hypogonadism. Diminished androgens in primary hypogonadism and diminished growth hormone in the secondary hypogonadism are probably responsible for the myopathy.
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During the year 1994-1995, 20 of the 67 leprosy patients attending the dermatology department with any kind of nerve involvement were found to be having nerve abscess. These abscesses occurred in all types of leprosy (except the Indeterminate) and a variety of nerve trunks and cutaneous nerves. In none of the instances the abscess was associated with reaction. All the patients were surgically treated, without any steroid therapy. All cases showed significant improvement whenever there was nerve function deficit. Similarly, pain was relieved in all cases, when it was present.