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PTOMAINE POISONING OR PERFORATION ?
396 of degeneration which is absent in The facial type of myopathy somewhat resembles the appearance in myasthenia gravis. But myopathy is progressive or stationary and not remittent, the tongue, pharynx, and larynx, and, almost invariably, the ocular muscles with the exception of the orbiculares escape. In superior nuclear palsy (polio-encephalitis) the ocular and bulbar muscles may be paralysed. But here, again, we do not meet with the remissions of symptoms which characterise ’i myasthenia gravis. Also superior nuclear palsy is frequently If associated with syphilis, tabes, or general paralysis of the insane, but this association has not been found in myasthenia
electrical
reaction
myasthenia gravis.
I,
gravis. 11’TOna
diphtheritic paralysis.-Myastbenia gravis is perhaps likely to be mistaken for diphtheritic paralysis than for any other disease. A nasal voice, regurgitation of fluids through the nose when swallowing is attempted, are wellknown symptoms of paralysis of the soft palate, and they occur almost without exception in cases of diphtheritic paralysis. In this disease, moreover, paralysis of the extrinsic and intrinsic ocular muscles, of the pharynx and glottis, and of the intercostals and diaphragm are extremely common. General muscular prostration, easily induced fatigue, are observed in diphtheritic paralysis and in myasthenia gravis alike. In both diseases we see sudden onsets of dangerous cardio-pulmonary crises, which may subside, but which often prove fatal. In myasthenia gravis. however, respiratory failure is most common and in diphtheritic paralysis cardiac failure. A most important point of distinction is that the knee-jerks in diphtheritic paralysis are, as a standing rule, absent, although for a brief period they may be exaggerated. In myasthenia gravis they are usually active. In a few cases they have been sluggish and in some they have been exhaustible on repetition. In no case have they been reported entirely absent. The myasthenic reaction is absent in diphtheritic paralysis, but faradaic excitability is diminished, and in some cases the ordinary reactions of degeneration are present. The history of a previous sore-throat is, of course, all important. Yet many cases diagnosed as diphtheritic paralysis have no such history, and possibly some of them may have actually been cases of myasthenia gravis. The following is probably an example. A boy, aged 11 years, was an in-patient at the Hospital for Epilepsy and Paralysis at Regent’s Park Hospital from April 14th till Oct. 8th, 1893, under the care of Dr. A. Hughes Bennett. In the latter’s absence I frequently had charge of the case, and regarded it as one of diphtheritic paralysis without evidence of preceding diphtheria. But Dr. Bennett’s more cautious diagnosis was" dysphasia and nervous debility." The boy was discharged "much improved." In April, 1894, however, he was re-admitted to hospital by Dr. W. Aldren Turner who noted "general feeble action of facial muscles, paresis of both orbiculares and frontales muscles, pupils dilated, no cycloplegia, cannot whistle or pout lips ; tongue not protruded as much as it should be ; nasal voice, but palate acts normally, weakness of upper and lower limbs general, especially of trapezii and deltoids, kneejerks elicited with difficulty ; feeble action of facial muscles to faradaism." On April 25th attacks of dyspnoea commenced and continued at intervals until the patient died in one of them on May 24th, 1894. Dr. Turner made a postmortem examination and found the cerebro-spinal system absolutely healthy in appearance. At the time neither he nor I were acquainted with myasthenia gravis and could only regard the symptoms as due to an extremely unusual form of diphtheritic paralysis ; unusual in its long duration (more than a year) and in the occurrence of a fatal relapse after a period of remission lasting several more
months.
The average duration of myasthenia gravis (in fatal cases) is one and a half yearp, but diphtheritic paralysis rarely lasts beyond ,a few weeks, during which the symptoms are fairly constant and (with the exception of the dangerous respiratory and cardiac crises which come and go) gradually subside. The difficulty in making the diagnosis in early cases of myasthenia gravis is obvious ; but when symptoms of supposed diphtheritic paralysis have lasted unusually long and relapses have occurred after apparent recovery the disease is probably myasthenia gravis and not diphtheritic paralysis. Sensory affections, such as numbness, tingling, and partial acassthesia. may be of value in the case of adults as evidence of diphtheritic paralysis. These symptoms are usually absent in myasthenia gravis, but in a case recorded
by Guastoni and Lombisnbjective sensations of heat occurred with formication. From
Landry’sparalysis.-Landry’s paralysis usually runa
It seldom course than myasthenia gravis. than a few days ; the symptoms are steadily progressive and not remittent. Electrical reactions are said tobe normal, myotatic irritability and superficial reflexes disappear, and sensibility is impaired. -From multiple neuritis.-Tbe history of alcoholic, arsenical, and other toxsemic influences, also the presence of pain, hyperassthesia or anaesthesia, tenderness in the course of nerves, local cedema, and glossiness or pigmentation of the skin, rapid wasting of the muscles, loss of tendon reflexes, absence of myasthenic reaction, distinguish multiple neuritis from myasthenia gravis. T’romz post-in.fluenzal neuroses.-Some of the cases of myasthenia gravis in which the onset has been sudden and associated with fever, headache, photophobia, and general pains suggest an influenzal origin. Moreover, in the early nineties, when influenza was a far more virulent affection than at the present day, sequelse in the shape of various forms of ophthalmoplegia, of paralysis of the soft palate, pharynx, and larynx, of various cranial nerves, and cardiopulmonary seizures were not infrequent. The complete muscular prostration and general neurasthenia following influenza also resemble the conditions in myasthenia gravis. But apart from psychical and hysterical affections the postinfluenzal paralyses in general are attributable to multiple neuritis or to definite myelitis affecting various segments of the cord, whilst in the case of myasthenia gravis the symptoms cannot be referred to such lesions. Treatment.-The symptoms of the disease are alwaj s rendered worse by bodily exercise and mental excitement. Hence, complete rest for mind and body should be the aim of treatment. The patients are from the very first in imminent danger of death, and their relatives should be warned of this, otherwise the deceptive lulls in the symptoms may encourage fatal indiscretions. Medical attendants should remember that frequent physical examinations may have disastrous results. Meals should be small, easily assimilated, and given at short intervals. The use of the stomach tube is dangerous, and should deglutition become impossible rectal feeding is the only course to pursue. Stimulant methods of treatment by cold douching -and faradaism are absolutely contra-indicated. Massage and galvanism have proved useless. None of the drugs hitherto employed appear really to influence the course of the disease. Strychnia has been used without benefit by Strumpell and Dr. Buzzard-my own patient improved under. strychnia at first, but subsequently relapsed whilst still taking it. Thyroid and suprarenal extract have proved also ineffectual. Tonics, such as arsenic and iron, have no specific action. Perhaps a more extended trial of suprarenal extract might be worth making. Its probably complex nature and variation in its constituents according to the condition of the glands at the time the extract is made may account for its failure hitherto as a remedy. Other organic extracts, such as cerebrin, myelin, didymin, and ovarian substance may also conceivably be of service in a tozoemia, which produces no visible changes in any part of the nervous system. Failing these medicaments, one can only hope that the toxin of myasthenia gravis may be traced to its source, isolated, and its antidote found in our chemical and physiological laboratories. For morbid anatomy has thrown no ligbt on the nature of the disease. Upper Berke’ey-street, W.
a
more
lasts
rapid
more
PTOMAINE POISONING OR PERFORATION ? BY E. KEMPSON
BROWN, M.R.C.S. ENG., L.R.C.P. LOND
ON Nov. 19th, 1900, I was called to see a woman who was said to have become suddenly unconscious. She was an albino, 27 years of age, of nervous temperament, and generally enjoyed good health. I found her lying on her back with her legs extended ; her face was pale, her skin was hot to the touch, and her pulse was almost imperceptible. Her eyes were closed. When spoken to she made no reply, but opened her eyes, which were then seen to be expressionless 7 II Policlinico,
Sept. 1st, 1900.
397
pupils : were dilated and her eyeballs might conceivably have commenced over the cardiac portion the stomach and so have progressively extended and irregularly. Her respirations were loud and 20 per more general. minute ; her temperature in the axilla was 103° F. There were twitchings of the muscles of her neck and face, her Andover, Hants. abdomen was slightly tympanitic, and signs, of pain were elicited on pressure. Physical examination of the lungs, A CASE OF heart, and liver showed nothing abnormal. After the lapse bf an hour the patient was more conscious; her pulse was CEREBELLAR HÆMORRHAGE PRESENT140 at the wrist, of very low tension and irregular, and her ING WELL-MARKED EARLY CERVICAL temperature in the axilla was 105°. Subsequently she had OPISTHOTONOS AND KERNIG’S rigors, her temperature fell progressively and was 99° five hours before death. Her extremities became cold, the surface SIGN. of her body became clammy, and she had hallucinations BY WILLIAM THYNE, M.A., M.D. EDIN. with general nervous prostration. Diarrhoea and vomiting were frequent, the stools being offensive and dark-coloured. In three days she passed about a pint of dark-coloured urine A MAN, aged 20 years, with an epileptic history, was in containing phosphates and mucus but no albumin. A his usual health on Christmas Day, 1900. After partaking of papular erythematous rash developed on the backs of her several hearty meals he was seized with vomiting, which hands and wrists and a few spots on her abdomen and knees on Nov. 21st. Death occurred early on the morning of continued through the night. About six hours after the Nov. 22nd, about 86 hours from the onset of the first sym- onset it was noticed that the head was retracted (see Fig. 1) =and vacant; her moved
ptoms. I ascertained the following facts from her friends. She had been poorly the previous week from the effects of mentruation and cold. On Nov. 17th she felt well and ate two beef sausages. Three other persons had also partaken of sausages the same night. Two of them suffered from diarrhoea and vomiting. The third ate very little and was not affected ; she gave the rest of her sausage to her dog, which was violently sick 12 hours afterwards. The deceased was seized with severe pain in her abdomen and vomiting 12 hours after eating the two sausages ; diarrhoea set in about 20 hours afterwards. At the postmortem examination the abdominal cavity was found to contain about a pint of turbid fluid. There was exudation of lymph and there were recent adhesions between the omentum and intestines. The parietal peritoneum was smooth and there were The peritoneum no signs of intiammation. over the small intestines appeared to be intensely inflamed, especially in the region of the ileum ; the upper part of the small The intestine was markedly distended. spleen and the posterior surface of the stomach were bathed in curdy lymph. The mucous membrane of the stomach was acutely inflamed over the cardiac the and lesser curvature of greater portion with numerous ecchymoses, and the external surface in these regions was likewise inIn the upper part of flamed. the duodenum the mucous membrane showed petechim in the submucous tissue ; the duodenum was dilated. The inner surface of the rest of the intestine was pale with a few pin-point nodules at the lower six inches of the ileum. The lumen of the appendix was occluded; there were no adhesions externally and no signs of recent inflammation in this region. In connexion with the left ovary there was a multilocular tumour of the s;ze of a hen’s egg containing clear fluid ; there were no signs of inflammation around it. The right ovary shoved commencing cystic formation The Fallopian tubes and uterus were healthy; the bladder was normal ; the kidneys, liver, spleen, and lungs were congested ; the heart was normal; the brain
was
of become
FIG. 1.
Showing retraction of head. FIG. 2.
anasmic.
- B6MK)M’.—The points of interest about
this case, I think, are the following:e (1) the sudden occurrence of the semiunconscious state; (2) the high temperature ; (3) the absence of marked mus-
Showing Kernig’s sign.
weakness; ana (.’1-) tine very intiense innammation and the patient complained of severe frontal headache. in the peritoneum covering the intestine without There were no loss of consciousness, no motor paralysis, and any apparent inflammation inside from. which the no convulsions or twitchings, and the organic reflexes were The peritonitis The tendon reflexes were increased, Kernig’s toxic irritant presumably extended, umar
unimpaired.
electrical
reaction
myasthenia gravis.
I,
gravis. 11’TOna
diphtheritic paralysis.-Myastbenia gravis is perhaps likely to be mistaken for diphtheritic paralysis than for any other disease. A nasal voice, regurgitation of fluids through the nose when swallowing is attempted, are wellknown symptoms of paralysis of the soft palate, and they occur almost without exception in cases of diphtheritic paralysis. In this disease, moreover, paralysis of the extrinsic and intrinsic ocular muscles, of the pharynx and glottis, and of the intercostals and diaphragm are extremely common. General muscular prostration, easily induced fatigue, are observed in diphtheritic paralysis and in myasthenia gravis alike. In both diseases we see sudden onsets of dangerous cardio-pulmonary crises, which may subside, but which often prove fatal. In myasthenia gravis. however, respiratory failure is most common and in diphtheritic paralysis cardiac failure. A most important point of distinction is that the knee-jerks in diphtheritic paralysis are, as a standing rule, absent, although for a brief period they may be exaggerated. In myasthenia gravis they are usually active. In a few cases they have been sluggish and in some they have been exhaustible on repetition. In no case have they been reported entirely absent. The myasthenic reaction is absent in diphtheritic paralysis, but faradaic excitability is diminished, and in some cases the ordinary reactions of degeneration are present. The history of a previous sore-throat is, of course, all important. Yet many cases diagnosed as diphtheritic paralysis have no such history, and possibly some of them may have actually been cases of myasthenia gravis. The following is probably an example. A boy, aged 11 years, was an in-patient at the Hospital for Epilepsy and Paralysis at Regent’s Park Hospital from April 14th till Oct. 8th, 1893, under the care of Dr. A. Hughes Bennett. In the latter’s absence I frequently had charge of the case, and regarded it as one of diphtheritic paralysis without evidence of preceding diphtheria. But Dr. Bennett’s more cautious diagnosis was" dysphasia and nervous debility." The boy was discharged "much improved." In April, 1894, however, he was re-admitted to hospital by Dr. W. Aldren Turner who noted "general feeble action of facial muscles, paresis of both orbiculares and frontales muscles, pupils dilated, no cycloplegia, cannot whistle or pout lips ; tongue not protruded as much as it should be ; nasal voice, but palate acts normally, weakness of upper and lower limbs general, especially of trapezii and deltoids, kneejerks elicited with difficulty ; feeble action of facial muscles to faradaism." On April 25th attacks of dyspnoea commenced and continued at intervals until the patient died in one of them on May 24th, 1894. Dr. Turner made a postmortem examination and found the cerebro-spinal system absolutely healthy in appearance. At the time neither he nor I were acquainted with myasthenia gravis and could only regard the symptoms as due to an extremely unusual form of diphtheritic paralysis ; unusual in its long duration (more than a year) and in the occurrence of a fatal relapse after a period of remission lasting several more
months.
The average duration of myasthenia gravis (in fatal cases) is one and a half yearp, but diphtheritic paralysis rarely lasts beyond ,a few weeks, during which the symptoms are fairly constant and (with the exception of the dangerous respiratory and cardiac crises which come and go) gradually subside. The difficulty in making the diagnosis in early cases of myasthenia gravis is obvious ; but when symptoms of supposed diphtheritic paralysis have lasted unusually long and relapses have occurred after apparent recovery the disease is probably myasthenia gravis and not diphtheritic paralysis. Sensory affections, such as numbness, tingling, and partial acassthesia. may be of value in the case of adults as evidence of diphtheritic paralysis. These symptoms are usually absent in myasthenia gravis, but in a case recorded
by Guastoni and Lombisnbjective sensations of heat occurred with formication. From
Landry’sparalysis.-Landry’s paralysis usually runa
It seldom course than myasthenia gravis. than a few days ; the symptoms are steadily progressive and not remittent. Electrical reactions are said tobe normal, myotatic irritability and superficial reflexes disappear, and sensibility is impaired. -From multiple neuritis.-Tbe history of alcoholic, arsenical, and other toxsemic influences, also the presence of pain, hyperassthesia or anaesthesia, tenderness in the course of nerves, local cedema, and glossiness or pigmentation of the skin, rapid wasting of the muscles, loss of tendon reflexes, absence of myasthenic reaction, distinguish multiple neuritis from myasthenia gravis. T’romz post-in.fluenzal neuroses.-Some of the cases of myasthenia gravis in which the onset has been sudden and associated with fever, headache, photophobia, and general pains suggest an influenzal origin. Moreover, in the early nineties, when influenza was a far more virulent affection than at the present day, sequelse in the shape of various forms of ophthalmoplegia, of paralysis of the soft palate, pharynx, and larynx, of various cranial nerves, and cardiopulmonary seizures were not infrequent. The complete muscular prostration and general neurasthenia following influenza also resemble the conditions in myasthenia gravis. But apart from psychical and hysterical affections the postinfluenzal paralyses in general are attributable to multiple neuritis or to definite myelitis affecting various segments of the cord, whilst in the case of myasthenia gravis the symptoms cannot be referred to such lesions. Treatment.-The symptoms of the disease are alwaj s rendered worse by bodily exercise and mental excitement. Hence, complete rest for mind and body should be the aim of treatment. The patients are from the very first in imminent danger of death, and their relatives should be warned of this, otherwise the deceptive lulls in the symptoms may encourage fatal indiscretions. Medical attendants should remember that frequent physical examinations may have disastrous results. Meals should be small, easily assimilated, and given at short intervals. The use of the stomach tube is dangerous, and should deglutition become impossible rectal feeding is the only course to pursue. Stimulant methods of treatment by cold douching -and faradaism are absolutely contra-indicated. Massage and galvanism have proved useless. None of the drugs hitherto employed appear really to influence the course of the disease. Strychnia has been used without benefit by Strumpell and Dr. Buzzard-my own patient improved under. strychnia at first, but subsequently relapsed whilst still taking it. Thyroid and suprarenal extract have proved also ineffectual. Tonics, such as arsenic and iron, have no specific action. Perhaps a more extended trial of suprarenal extract might be worth making. Its probably complex nature and variation in its constituents according to the condition of the glands at the time the extract is made may account for its failure hitherto as a remedy. Other organic extracts, such as cerebrin, myelin, didymin, and ovarian substance may also conceivably be of service in a tozoemia, which produces no visible changes in any part of the nervous system. Failing these medicaments, one can only hope that the toxin of myasthenia gravis may be traced to its source, isolated, and its antidote found in our chemical and physiological laboratories. For morbid anatomy has thrown no ligbt on the nature of the disease. Upper Berke’ey-street, W.
a
more
lasts
rapid
more
PTOMAINE POISONING OR PERFORATION ? BY E. KEMPSON
BROWN, M.R.C.S. ENG., L.R.C.P. LOND
ON Nov. 19th, 1900, I was called to see a woman who was said to have become suddenly unconscious. She was an albino, 27 years of age, of nervous temperament, and generally enjoyed good health. I found her lying on her back with her legs extended ; her face was pale, her skin was hot to the touch, and her pulse was almost imperceptible. Her eyes were closed. When spoken to she made no reply, but opened her eyes, which were then seen to be expressionless 7 II Policlinico,
Sept. 1st, 1900.
397
pupils : were dilated and her eyeballs might conceivably have commenced over the cardiac portion the stomach and so have progressively extended and irregularly. Her respirations were loud and 20 per more general. minute ; her temperature in the axilla was 103° F. There were twitchings of the muscles of her neck and face, her Andover, Hants. abdomen was slightly tympanitic, and signs, of pain were elicited on pressure. Physical examination of the lungs, A CASE OF heart, and liver showed nothing abnormal. After the lapse bf an hour the patient was more conscious; her pulse was CEREBELLAR HÆMORRHAGE PRESENT140 at the wrist, of very low tension and irregular, and her ING WELL-MARKED EARLY CERVICAL temperature in the axilla was 105°. Subsequently she had OPISTHOTONOS AND KERNIG’S rigors, her temperature fell progressively and was 99° five hours before death. Her extremities became cold, the surface SIGN. of her body became clammy, and she had hallucinations BY WILLIAM THYNE, M.A., M.D. EDIN. with general nervous prostration. Diarrhoea and vomiting were frequent, the stools being offensive and dark-coloured. In three days she passed about a pint of dark-coloured urine A MAN, aged 20 years, with an epileptic history, was in containing phosphates and mucus but no albumin. A his usual health on Christmas Day, 1900. After partaking of papular erythematous rash developed on the backs of her several hearty meals he was seized with vomiting, which hands and wrists and a few spots on her abdomen and knees on Nov. 21st. Death occurred early on the morning of continued through the night. About six hours after the Nov. 22nd, about 86 hours from the onset of the first sym- onset it was noticed that the head was retracted (see Fig. 1) =and vacant; her moved
ptoms. I ascertained the following facts from her friends. She had been poorly the previous week from the effects of mentruation and cold. On Nov. 17th she felt well and ate two beef sausages. Three other persons had also partaken of sausages the same night. Two of them suffered from diarrhoea and vomiting. The third ate very little and was not affected ; she gave the rest of her sausage to her dog, which was violently sick 12 hours afterwards. The deceased was seized with severe pain in her abdomen and vomiting 12 hours after eating the two sausages ; diarrhoea set in about 20 hours afterwards. At the postmortem examination the abdominal cavity was found to contain about a pint of turbid fluid. There was exudation of lymph and there were recent adhesions between the omentum and intestines. The parietal peritoneum was smooth and there were The peritoneum no signs of intiammation. over the small intestines appeared to be intensely inflamed, especially in the region of the ileum ; the upper part of the small The intestine was markedly distended. spleen and the posterior surface of the stomach were bathed in curdy lymph. The mucous membrane of the stomach was acutely inflamed over the cardiac the and lesser curvature of greater portion with numerous ecchymoses, and the external surface in these regions was likewise inIn the upper part of flamed. the duodenum the mucous membrane showed petechim in the submucous tissue ; the duodenum was dilated. The inner surface of the rest of the intestine was pale with a few pin-point nodules at the lower six inches of the ileum. The lumen of the appendix was occluded; there were no adhesions externally and no signs of recent inflammation in this region. In connexion with the left ovary there was a multilocular tumour of the s;ze of a hen’s egg containing clear fluid ; there were no signs of inflammation around it. The right ovary shoved commencing cystic formation The Fallopian tubes and uterus were healthy; the bladder was normal ; the kidneys, liver, spleen, and lungs were congested ; the heart was normal; the brain
was
of become
FIG. 1.
Showing retraction of head. FIG. 2.
anasmic.
- B6MK)M’.—The points of interest about
this case, I think, are the following:e (1) the sudden occurrence of the semiunconscious state; (2) the high temperature ; (3) the absence of marked mus-
Showing Kernig’s sign.
weakness; ana (.’1-) tine very intiense innammation and the patient complained of severe frontal headache. in the peritoneum covering the intestine without There were no loss of consciousness, no motor paralysis, and any apparent inflammation inside from. which the no convulsions or twitchings, and the organic reflexes were The peritonitis The tendon reflexes were increased, Kernig’s toxic irritant presumably extended, umar
unimpaired.