NI FEATURE - COMMENTARY: TIMELESS REVERBERATIONS
|Year : 2017 | Volume
| Issue : 5 | Page : 944--947
Memorable clinical experiences, all in one year at Queen Square, London, United Kingdom (1968-1969): A tribute to Neurology legends
Past President, Neurological Society of India, Department of Neurology, Madurai Medical College, Madurai, Tamil Nadu, India
Emeritus Professor of Neurology, Madurai Medical College, 806/11-A, Bypass Road, Madurai - 625 016, Tamil Nadu
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Srinivasan K. Memorable clinical experiences, all in one year at Queen Square, London, United Kingdom (1968-1969): A tribute to Neurology legends.Neurol India 2017;65:944-947
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Srinivasan K. Memorable clinical experiences, all in one year at Queen Square, London, United Kingdom (1968-1969): A tribute to Neurology legends. Neurol India [serial online] 2017 [cited 2018 Dec 13 ];65:944-947
Available from: http://www.neurologyindia.com/text.asp?2017/65/5/944/214078
'Imitation is for the most part so unconscious that its effects are almost unheeded, but its influence is not the less permanent on that account. It is only when an impressive nature is placed in contact with an impressionable one that the alteration in the character becomes recognizable. Yet even the weakest natures exercise some influence upon those about them. The approximation of feeling, thought, and habit is constant, and the action of example unceasing.'
Samuel Smiles (23 December 1812 – 16 April 1904)
These anecdotes represent reminiscences of the time I spent at the National Hospital for Neurology and Neurosurgery, Queen Square, London, in 1968-1969. During my stay there, I was fortunate enough to gain first-hand experience in observing closely, the clinical methodology and functioning of several luminaries in the field of Neurology working there at that time. Interacting with these legendary doctors, while deciphering the complex diagnostic dilemmas encountered at that time, not only developed my clinical acumen and methodology but also provided me lessons for the future that have kept me in good stead throughout my years as a practising clinical neurologist. I share these lessons with you in the sincere hope that these lessons are of benefit to you in your quest for improving the lives of your patients suffering from neurological ailments.
Dr. John Marshall (an eminent Professor of Clinical Neurology at the National Hospital for Nervous Diseases, Queen Square, London and a founding member of the Cerebral Blood Flow Group), while examining young adults with stroke, always remarked, “Look for causes at both the sites- intracranial and infraclavicular.” A middle-aged patient presented with a history of transient ischemic attacks as well as reversible ischemic stroke in the carotid artery distribution. Dr. John Marshall said that the etiology was embolic since retinal emboli had been documented earlier and the angiogram had shown recanalization of vessels. Dr. Dereck Ernest Denny-Brown (a senior consultant at the National Hospital for Neurology and Neurosurgery, Queen Square, London who later on became the Professor of Neurology at Harvard University and the International Scholar at the National Institutes of Health, Bethesda, Maryland), however, forcibly argued that the etiopathogenesis was haemodynamic and was caused by a large focal plaque at an unusual angulation located at the carotid bifurcation. Dr. Brown, then went on to prove his assertion by reproducing the ischemic symptoms by tilting the patient head-down and then head-up over a tilting table. A pleasant controversy with lessons for future that in carotid or vertebrobasilar stenosis, the morphology of the plaque is as relevant as the degree of obstructionDr. Denny-Brown proposed the diagnosis of Wilson's Disease in a young patient with axial dystonia and athetosis along with dystonia of the limbs (also termed as tetanoid chorea). The patient, however, did not have any Kayser Fleisher ring. As soon as he discovered the absence of Kayser Fleisher ring in the patient, he stated, “In that case, this is not Wilson's disease!” He asked the patient to walk around a wooden stool and exposed the presence of axial dystonia that had been missed earlier! The final diagnosis in the patient turned out to be mercury poisoning, This boy had been working in an illicit, underground mercury thermometer factory, with the floor glistening with mercury spilt over during the construction of the thermometers. The patient had not mentioned his occupation earlier for due to its illegal nature. This story has an important lesson. Whenever a patient is encountered with an unusual clinical presentation or with vital missing signs, it is always better to go back to the patient to elicit details of his history and vital signs. This will often secure a clue to the diagnosis. This technique is much better than trying to fit the casually elicited clinical presentation into the most obvious clinical diagnosis that comes to mind, overruling the vital missing signsA young man was hospitalised for a rapidly evolving tetraparesis and cervical root pain that developed over only two days. As this was before the age when computed tomogram scanners were being used in routine clinical practice, a lumbar myodil myelogram was performed, which showed a smooth total high cervical block. Mr. Wylie McKissock (the senior most consultant neurosurgeon at the National Hospital for Neurology ad Neurosurgery, who also set up the neurosurgical unit at the Atkinson Morley Hospital, became the president of the Society of British Neurological Surgeons and became a recipient of the Order of British Empire), decided to explore the cervical spine by performing a laminectomy and decompression of the lesion in his patient, scheduling him for the first surgery of the day. Dr. James William Douglas Bull (the Dean and also the Professor of Neuroradiology at the National Hospital for Neurology and Neurosurgery, who later on became the President of the Royal College of Radiologists, the President of the Radiological and Neurological Sections of the Royal Society of Medicine, the President of the British Institute of Radiology, as well as founder President of the British and European Societies of Neuroradiology) asked me to bring the patient for his clinical examination before giving his report. He was visibly upset when as a resident, I told him, “The patient is on the operation table.” During surgery, while passing the dissection probe after performing the laminectomy, the spinal aneurysm present in the spinal canal in the patient (that was causing the spinal block) ruptured, and the ensuing bleeding could only be controlled due the considerable skill exhibited by the surgeon. The patient survived, albeit with tetraparesis and remained on ventilatory support for a considerable period of time. Prof. Bull said that he had diagnosed the lesion as an aneurysm on the radiological basis because of the very smooth margin and rounded space occupying appearance of the myelogram defect. He included this case for his presentation on “aneurysms mistaken as space occupying tumors!” Considering input from all possible sources prior to an intervention often helps in achieving good results and in avoiding morbidityDr. Douglas McAlpine (the founder of Multiple Sclerosis Society of Great Britain and writer of the book ‘Multiple Sclerosis') was an international authority on multiple sclerosis. His own sister was admitted to the Royal Homeopathic Hospital next door with the presentation of an ataxic gait. Although she progressively deteriorated, it took considerable effort to secure her transfer to the Neurology department. This was because the homeopathic experts were hopeful that mistletoe could melt away the cerebellar tumour that she was diagnosed to be having! Her midline cerebellar astrocytoma was excised successfully by Mr. Lawrence (Ticky) Walsh (a senior consultant neurosurgeon at the National Hospital of Neurology and Neurosurgery) but postoperatively she developed akinetic mutism. Prof. Dennis Hill (Emeritus Professor of Psychiatry, University of London) vehemently disputed this postoperative sequel and tried a low dose pentothal analysis on her. He, however, agreed with our diagnosis only when she did not improve following his treatment. She walked out weeks later showing a considerable improvement in her ataxic gait but was subsequently readmitted for gait apraxia and amnesic spells due to the development of normal pressure hydrocephalus. A cerebrospinal fluid diversion procedure was performed by Mr. Ticky Walsh and she remained well after that. One must never give up on the evolving clinical presentations in one's patients; often the variability in clinical spectrum may be due to different but related underlying pathologies that the patient may be developing. Involving colleagues with different expertise often helpsDr. Michael John Francis McArdle (a senior neurologist at National Hospital for Neurology and Neurosurgery) used to discuss diagnostic problems in regular weekly sessions for the benefit of international students. One young patient had bilateral wasting of small muscles of the hands without sensory loss. The cervical spine myelogram excluded the presence of any proximally placed lesion. Motor neuron disease (MND) was considered as a probable diagnosis. A simple recall of neuroanatomy, however, revealed that the deep branch of ulnar nerve at the palm supplies all the involved muscles. In the patient, a detailed clinical examination revealed that the abductor pollicis brevis was spared; thus, the diagnosis of MND was unlikely. An exploration of tissues of the hand confirmed a neuroma of the deep branch of ulnar nerve in both the hands. This had occurred due the an occupational hazard caused by the rimmed handgrips used by the patient, who was employed as a courier to deliver post, and performed his task using a motor cycle. Thus, before labelling a patient as having an irreversible disease, a concentrated effort must be made to rule out other likely possibilities. Often, what seems to be the most likely diagnosis may not always be the case. A simple explanation based upon the occupation and recurrent activities of the individual should also be sought forWe presented the history of a 30-year old female patient, who was having severe episodes of headaches, despite being normotensive. She also had bilateral papillodema but with no lateralising signs. Dr. McArdle made her bend forwards and made her perform the Valsalva maneuver. Immediately, her unilateral proptosis was both evident and palpable. A diagnosis of sphenoidal ridge meningioma, that had eroded through the greater wing of the sphenoid, and was causing recurrent temporal lobe herniation into the orbit, resulting in the reducible proptosis, could be established. The tumor was later on successfully excised after confirming the diagnosis with radiological investigations. The lesson learnt was that a central nervous system tumor may present with pure symptoms, pure signs or both. Thus, elicitation of physical signs should never be neglected to properly establish a clinical diagnosisSir Charles Symonds (the senior neurologist at the National hospital for Neurology and Neurosurgery, who also created a Hospital for Head Injuries at Oxford University and was the first to document cerebral aneurysms as the cause of subarachnoid hemorrhage) was discussing clinical problems during his regular Thursday afternoon sessions. Dr. Henry Miller (the consultant neurologist at Royal Victoria Infirmary in Newcastle-upon-Tyne who later on became the vice-chancellor of the University of Newcastle-upon-Tyne) telephoned from Newcastle about a lady patient with resistant episodes of migraine in whom all of the investigations including the angiogram and pneumoencephalography (PEG) were normal. Dr. Symonds advised Dr. Millar to re-examine the patient by asking the patient to perform an active Valsalva manoeuvre while he was bent forwards. This produced a transient, partial oculomotor nerve palsy with unilateral pupillary changes and a repeat PEG disclosed an anterior third ventricular tumour missed earlier because of its smaller size. It was causing symptomatic positional headache due to foramen of Monro obstruction, leading to a migraine-like picture. If there is an impasse regarding a particular diagnosis, the opinion from another knowledgeable colleague often helpsA young boy, aged 12 years, presented with opthalmoplegia and long tract signs. The clinical possibilities raised included a brain stem glioma or demyelination. We, as students, suggested steroid administration and a regular follow up for him. His cerebrospinal fluid globulin was positive with a paretic Lange curve, the conventional pointers to the presence of multiple sclerosis. Dr. Dennis John Williams (one of the most influential figures in neurology in Britain, founder trustee of the Brain Research Trust, and the editor of the journal ‘Brain'), however, insisted on the diagnosis being a glioma, as a response to steroids and spontaneous remission are possible with either of the diagnosis, and therefore, we could not decide between the two. He insisted that the cerebrospinal fluid specific protein that was detected could also have occurred due to the presence of a large neoplastic lesion. He was supported in this diagnosis by various Neurology textbooks including the one by DeJong. Thus, multiple, completely different disease entities may have overlapping manifestations; and, investigations and a correct interpretation of the overall sequence of clinical events has a great bearing on the establishment of the proper diagnosisDr. Wilder Penfield (the Founder Director of Montreal Neurological Institute, Canada, who was the first to map the functions of various regions of the brain such as the cortical homunculus) was awarded the Fellowship of the Royal Society (F.R.S) in London. He described a case that was later on published as a letter in the journal ‘Proceedings of the Royal Society of Medicine’ in which he showed the video of a 35-year old civil engineer, who was attempting to draw the details of a building. He suddenly received a phone call that he answered; subsequent to answering the call, he could not remember details of his work and crushed the paper and threw it into the waste paper basket! He continued his drawings from the beginning again but was distracted either by someone giving a cup of coffee to him or by a messenger. Every time he was distracted, he forgot what he was doing and disposed off his unfinished drawing into the waste paper basket. This was due to his impaired current memory and a disturbance in the sequence of his planning, whenever he was distracted! The waste paper basket became full of papers containing his unfinished work but due to constant interruptions, and the fact that after every interruption, he had to start all over again, his work could not be completed. This man had an unilateral temporal lobectomy performed on him for recurrent intractable seizures years earlier. Penfield diagnosed that his current memory impairment was due to further damage to his intact temporal lobe, as memory was a bilateral function. When this draftsman died, his post-mortem study validated the prediction made by Dr. Penfield and showed an atrophic anterior temporal lobe on the non-operated side. However, while addressing the large gathering of doctors in London, some of whom very already neuroscience legends, he stated, “We do not speculate at length as some senior neurologists do. We give acceptable proof.” Thus, despite the stature that he had achieved, which would perhaps have ensured that any conjectures made by him would have been acceptable to the doctor community at that time, he himself was not willing to accept his own scientific findings until he had gathered an irrefutable proof validating themDr. John Newsom-Davis (discoverer of the causes of, and treatments for, myasthenia gravis, and of other diseases of the nerve-muscle junction, including Lambert-Eaton myasthenic syndrome and acquired neuromyotonia; he later on became the Professor of Neurology at Oxford University) was the registrar and my senior colleague in the same unit in which I was working in London. A young lady with syringobulbia was hospitalised with respiratory distress. She had similar episodes earlier and had a posterior burrhole through which aspiration of the brain stem cyst was done, gave her an immediate relief in her symptoms. During this admission, Mr. McKissock, a very senior neurosurgeon, aspirated the cyst under local anaesthesia. Following this procedure, she had recurrent and persistent bouts of hiccups. During the discussion of this case, a controversy arose whether or not hiccups actually manifested as diaphragmatic myoclonus. Dr. Newsom Davis and myself were permitted to record the diaphragmatic response on either side with due aseptic precautions and were able to establish the role of sudden diaphragmatic movements in the causation of hiccups. Phrenic nerve conduction, however, could not be assessed. It was then established that the centre for causation of hiccups resides in the medullary area. It was also agreed upon that reflex hiccups can be produced by a highly spicy diet that is common in the tropics. Common clinical situations encountered on bedside rounds may often lead to significant discoveries, if pursued assiduouslyDr. Dennis Williams (a senior consultant, and at that time, editor of the journal ‘Brain') was visibly annoyed when a lady patient with status epilepticus could not be relieved of her seizures despite being administered polytherapy in his unit. He called me and firmly said, “If you use more than two drugs and the seizures are not controlled, then either the diagnosis is a disputed one or the neurologist should be replaced.” Later in that week, it was clear that the mental distress that the patient was undergoing on being told that that she could not marry another epileptic patient (who was aspiring to become a church priest) was the real cause responsible for her feigning her seizure episodes. Her ictal episodes responded dramatically to the same treatment when she got married to the person of her choice. A special permission had to be subsequently granted to her husband to take up the responsibilities of priesthood after their wedding; this was accompanied by medical advice to help him undergo effective treatment for his epilepsy. Decades later, at Berlin, Pope Francis said that the church may consider ordaining married men to work as priests. Often, non-responsiveness to medical treatment may be due to a yet unexplored psychogenic or social cause that needs to be identified for an effective outcomeA nun, aged 35 years, was hospitalised as she had developed left hemiparesis with an echocardiographic evidence of left atrial myxoma. She refused any treatment because of her religious beliefs. She permitted every investigation to be performed to diagnose her illness as this would help the doctors acquire knowledge regarding her ailments. She, however, declined treatment despite every persuasion adopted by the doctors. She said, “God will look after me hereafter as he has done so far.” She completely recovered from the hemiparesis in three weeks time. A repeat echocardiogram did not reveal any lesion! All the records were checked for any mistaken identity. The possibility that pictures sent by a computer might have been distorted or might have missed the real issues was also checked. Finally, it was concluded that she probably had a clot that had disappeared, and had never harboured a myxoma. This report is from a very highly reputed teaching institution in London. The intuitive feelings that patients have about themselves must be respected and never ignored.Dr. William Ian McDonald was an eminent neurologist, National Hospital for Neurology and Neurosurgery, and a physician at Moorfields Eye Hospital. He was also Professor of Neurology, Institute of Neurology and Institute of Ophthalmology, University of London and the Editor of Brain. He was an authority on multiple sclerosis. His Ph.D thesis was on the effect of experimental demyelination due to diptheria toxin on rats. He was also an expert in playing music on a piano. A minor embolic stroke of the right angular and supramarginal gyri robbed him of his musical skills. It also caused the appearance of other parietal lobe signs like prosopagnosia, visual hallucinations, failure to perceive velocity of moving objects, and topographical disorientation. Regular practice of the daily routine along with practicing of music ensured his recovery in one year. The process of recovery was much more with these activities than with medications. There was a reversible decline in his neurological status as soon as he stopped his music practice for a short duration after the development of stroke. An increased frequency of migraine also persisted for 18 months. He, however, soon recovered and was later back to practising his music and teaching students. A determination to overcome personal setbacks in personal and professional life, as well diverting one's mind towards activities that one loves the most during the period of adversity, often helps in overcoming the crisis.
Pursuing clinical neurology training at the National Hospital for Neurology and Neurosurgery was exciting and made a deep impact on my impressionable mind due to the presence of excellent teachers present there. This was during the time before the advent of mesmerising and glamorous modern gadgets with sophisticated technology that now aid in the diagnosis of neurological disorders; an internet-based education that is easily accessible to the patients and their doctors, was also not present!
A healthy and mature combination of both clinical skills and the ability to utilize the modern methods of diagnosis will benefit our patients as well as the treating neurologists.