Kalyan B Bhattacharyya

Acute myopia induced by topiramate: Report of a case and review of the literature

Topiramate, a new anticonvulsant, is also used for the prophylaxis of migraine and cluster headache. A serious but not often discussed side effect of the drug is the development of acute myopia and acute angle-closure glaucoma in the early stage of therapy that subsides rapidly with prompt discontinuation. One such case is reported here and the relevant literature in this regard is also reviewed.

Clinical profile and genetic correlation of patients with spinocerebellar ataxia: A study from a tertiary care centre in Eastern India

Background: Progressive cerebellar ataxia inherited by autosomal dominant transmission is known as Spino Cerebellar Ataxia (SCA). Aims and Objectives: To look for various clinical profile and molecular genetics of patients with SCAs and their phenotype-genotype correlation of patients with SCAs. Materials and Methods: This was a cross-sectional study conducted at Bangur Institute of Neurosciences, Kolkata from June 2010 to April 2013. We selected patients from the neurogenetic clinic of our institute and performed genetic test for SCA 1, 2, 3, 6 and 12. The diagnosis was based on suggestive clinical features and positive genetic study, done by polymerase chain reaction. Results: 83 patients were tested for trineucleotide repeats and turned out 45 positive for the mentioned SCAs. We found 13(28.9%) SCA-1, 18(40%) SCA-2, 7(15.6%) SCA-3, 6(13.3) SCA-6 and 1(2.2%) SCA-12 patients. Half of the remaining 38 patients had positive family history. The mean age of onset were 38.46 years in SCA-1, 29.55 years in SCA-2, 38.43 years in SCA-3, 47.33 years in SCA-6. Slow saccades were observed in 7(53.8%) SCA-1, 17(94.4%) SCA-2, 4(57.1%) SCA-3, 3(50%) SCA-6 patients. Hyporeflexia was noticed in 5(27.8%) SCA-2 patients. Pyramidal tract involvement was found in 8(61.5%) SCA-1, 4(22.2%) SCA-2, 4(57.1%) SCA-3 and 1(16.7%) SCA-6 patients. Conclusion: Our study showed SCA-2 is the most common variety of SCA and genotypic-phenotypic correlation was observed in SCA-1,2,6 and 12 patients.

The story of George Huntington and his disease

George Huntington described some families with choreiform movements in 1872 in the United States of America and since then many such families have been described in other parts of the world and works on the genetics of the disease have brought new vistas in the understanding of the disease. In 1958, Americo Negrette, a young Venezuelan physician observed similar subjects in the vicinity of Lake Maracaibo which was presented by his co-worker, Ramon Avilla Giron at New York in 1972 when United States of America had been commemorating the centenary year of Huntington′s disease. Nancy Wexler, a psychoanalyst, whose mother had been suffering from the disease attended the meeting and organized a research team to Venezuela and they systematically studied more than 18,000 individuals in order to work out a common pedigree. They identified the genetic locus of the disease in the short arm of chromosome 4 and observed that it was a trinucleotide repeat disorder.

Sexual Dysfunctions in Parkinson's Disease: An Underrated Problem in a Much Discussed Disorder

Sexual dysfunctions (SDs) are one of the most neglected nonmotor symptoms in Parkinsons disease (PD). A number of reasons including social and cultural factors might explain, at least partially, why SD is still one of the most underrecognized aspects of the condition after 200 years since the very first description by James Parkinson. SD has not been extensively investigated, however, a number of studies have shown a high prevalence of decreased libido, orgasmic dysfunction in both men and women with PD, and erectile dysfunction in male subjects. Moreover, SD in PD also comprises the increasingly recognized hypersexuality that is often associated with PD treatment. Taken together, SD in PD includes a remarkable range of symptoms and conditions that often require a multidisciplinary approach regarding assessment, investigation, and treatment.

Familial orthostatic tremor and essential tremor in two young brothers: A rare entity.

Orthostatic tremor (OT), is usually a disease of old age and is characterized by quivering movements of the legs during quiet standing or in the state of isometric contraction in the lower limbs. This is relieved on walking or on lying down. It is diagnosed by surface electromyography, particularly over the quadriceps femoris muscles which shows a distinctive frequency of 13 to 18 Hz on standing. Some investigators consider it as a variant of essential tremor (ET) and the two conditions often co-exist. The disease is usually non-familial. Two brothers presented with tremor in the lower limbs on standing and on the outstretched hands without any family history. Subsequently, they were proved to be suffering from OT and ET by clinical examination and surface EMG. Simultaneous occurrence of OT and ET in two young brothers without any family history in the previous generation has not been described before and they also appeared at a much earlier age than what is described in the literature.

Walter Edward Dandy (1886-1946): The epitome of adroitness and dexterity in Neurosurgery

Walter Edward Dandy did pioneering work in Neurosurgery in the early part of the 19th century. He worked all his life at the Johns Hopkins Hospital in USA and contributed extensively to research in hydrocephalus, the physiology of the cerebrospinal fluid and devised the technique of pneumoencephalography, apart from performing the first clipping operation in cerebral aneurysm and the division of the trigeminal nerve in neuralgic pain. This article summarizes his immense contributions in the field of Neurosurgery.

Edgar adrian and patrick merton: Names blurred with the passage of time

Edgar Douglas Adrian and Patrick Anthony Merton are two supreme neurophysiologists from England in the last century whose names are almost forgotten these days. Adrians work on all-or-none phenomenon in nerve and muscle excitability ushered in a new era and Mertons servo theory of muscular movement and muscle fatigue added a new dimension to the understanding of stretch reflex and deep tendon reflexes. Both of them trained and worked at Trinity College, Cambridge and both were elected as Fellow of the Royal Society and Adrian in addition, was awarded the Nobel Prize in 1932 along with Charles Scott Sherrington.

1892 and the tribulations of Joseph Babinski

In the early 1880s, Joseph Babinski was appointed as Chef de Clinique under Jean-Martin Charcot at Salpétrière, Paris, in France. He appeared for the post of Professeur Agrégé, the pinnacle of academic distinction in France in 1892. Charles Bouchard, the earliest pupil of Charcot, who described the Charcot-Bouchard aneurysm along with his master, was the Chief of the Board of Jury. Charcot and Bouchard did not see eye to eye in the later period, and when German Sée, an external examiner did not join the board of examiners following illness, Bouchard, instead of seeking the assistance of an alternate examiner, employed the special right of Vote of Absence. Babinski and all other pupils of Charcot were unsuccessful, while those of Bouchard, came out with flying colors. An embittered Babinski, along with some of the other unsuccessful candidates, appeared before the Ministry of Public Assistance after a protracted legal battle for nearly 2 years but lost the case. They were even ordered by the court to pay on behalf of the the successful candidates for the legal battle. Babinski never sat for the examination again and this is the story of one of the most ignominious episodes in an examination anywhere.

James wenceslaus papez, his circuit, and emotion

James Papez worked on the anatomical substrates of emotion and described a circuit, mainly composed of the hippocampus, thalamus and cingulum, and published his observations in 1937. However, such an idea existed before him, as evidenced by the rudimentary indications from Paul Broca, and Paul MacLean added some other structures like, septum, amygdala, and hypothalamus in its ambit and called it the limbic system. Paul Ivan Yakovlev, proposed a circuit which also referred to orbitofrontal, insular, anterior temporal lobe, and other nuclei of thalamus. Further works hinted at cerebellar projections into this system and the clinical picture of aggression, arousal and positive feeding responses with stimulation of cerebellar nuclei, attests its possible role. Finally, the work of Heinrich Klüver and Paul Bucy of the United States of America on ablating the temporal lobes and amygdala and the resultant behaviour of the animals, almost incontrovertibly adduced evidence for the operation of a neural circuitry in the genesis of emotion. Additionally, Papez circuit may also be concerned with memory and damage to its various components in Parkinsons disease, Alzheimers disease, Korsakoffs syndrome, semantic dementia, and global amnesia, where cognitive disturbance is almost universal, lends credence to its putative role.

Godfrey Newbold Hounsfield (1919-2004): The man who revolutionized neuroimaging

Godfrey Hounsfield, a biomedical engineer contributed enormously towards the diagnosis of neurological and other disorders by virtue of his invention of the computed axial tomography scan for which he was awarded the Nobel Prize in 1979. Working for the Electrical and Musical Industry (EMI) Limited and in collaboration with two radiologists, James Ambrose and Louis Kreel, he introduced the use of this machine in 1971 at the Atkinson Morley′s Hospital in Wimbledon. He continued to improve the quality of the devise and the human head was scanned for the first time in 1972. He continued his work on imaging of the human body was later concentrated on the next step in diagnostic radiology namely, magnetic resonance imaging.