On General Paralysis

1860 ◽  
Vol 6 (32) ◽  
pp. 198-205
Author(s):  
Harrington Tuke

In attempting to describe the rise and progress of the paralysis, that forms one of the most remarkable symptoms of ‘paralytic insanity,’ I propose to notice first the affection of the muscles of the tongue, which is generally that which most strongly arrests the attention of the physician, who sees a patient in the second or fully-developed stage of the malady. In the last stage, the power of articulation is entirely gone; in both of these the paralysis is so marked, and the evidence of mental disease so clear, that the nature of the case can hardly be mistaken. It is in the very onset of the malady, that the peculiar lisp or failure of utterance, indicating disorder of the nervous centres, at or near the orifice of the nerves supply in the tongue muscles, is of paramount importance in the diagnosis, because if this symptom be superadded to eccentricity of conduct, or distinct delusion, there can remain but little doubt as to the existence of this special and almost invariably fatal form of disease. The alienist physician accustomed to watch the progress of general paralysis, and to recognize its slightest indication, cannot mistake the faulty pronounciation in question, for that of any other form of malady; but inasmuch as there are several affections of the speech that might mislead an unskilled practitioner, it may be useful to describe some of these derangements of the apparatus of articulation, and specify their points of difference. In the first place, an affection of the speech, very much resembling the embarassed articulation of incipient paralytic insanity, may be the result of temporary local congestion at the base of the brain, or may be produced by sudden fright, or by the action of poisons, particularly aconite; the indistinct utterance attending intoxication, is a familiar instance of poisoning of this kind: and all these are easily distinguished by the suddenness of their occurrence, and by their history, from the stutter of general paralysis. The articulation of the habitual stammerer is sometimes not unlike that which is the result of serious organic mischief; and still more striking in its resemblance, is the hesitation of speech, that may be observed in some cases of poisoning by lead. The ordinary signs of saturnine poisoning, the blue gum-line, the dropping of the wrist, &c., will mark this latter malady—the history of the case will prevent any mistake in the former. I may mention here, that I believe it to be an exceptional occurrence to find a person of unsound mind who stammers; such a case at least must be very uncommon, a fact which I can only account for on the supposition that the greater disease prevents any manifestation of the minor nervous derangement.

1860 ◽  
Vol 6 (32) ◽  
pp. 198-205
Author(s):  
Harrington Tuke

In attempting to describe the rise and progress of the paralysis, that forms one of the most remarkable symptoms of ‘paralytic insanity,’ I propose to notice first the affection of the muscles of the tongue, which is generally that which most strongly arrests the attention of the physician, who sees a patient in the second or fully-developed stage of the malady. In the last stage, the power of articulation is entirely gone; in both of these the paralysis is so marked, and the evidence of mental disease so clear, that the nature of the case can hardly be mistaken. It is in the very onset of the malady, that the peculiar lisp or failure of utterance, indicating disorder of the nervous centres, at or near the orifice of the nerves supply in the tongue muscles, is of paramount importance in the diagnosis, because if this symptom be superadded to eccentricity of conduct, or distinct delusion, there can remain but little doubt as to the existence of this special and almost invariably fatal form of disease. The alienist physician accustomed to watch the progress of general paralysis, and to recognize its slightest indication, cannot mistake the faulty pronounciation in question, for that of any other form of malady; but inasmuch as there are several affections of the speech that might mislead an unskilled practitioner, it may be useful to describe some of these derangements of the apparatus of articulation, and specify their points of difference. In the first place, an affection of the speech, very much resembling the embarassed articulation of incipient paralytic insanity, may be the result of temporary local congestion at the base of the brain, or may be produced by sudden fright, or by the action of poisons, particularly aconite; the indistinct utterance attending intoxication, is a familiar instance of poisoning of this kind: and all these are easily distinguished by the suddenness of their occurrence, and by their history, from the stutter of general paralysis. The articulation of the habitual stammerer is sometimes not unlike that which is the result of serious organic mischief; and still more striking in its resemblance, is the hesitation of speech, that may be observed in some cases of poisoning by lead. The ordinary signs of saturnine poisoning, the blue gum-line, the dropping of the wrist, &c., will mark this latter malady—the history of the case will prevent any mistake in the former. I may mention here, that I believe it to be an exceptional occurrence to find a person of unsound mind who stammers; such a case at least must be very uncommon, a fact which I can only account for on the supposition that the greater disease prevents any manifestation of the minor nervous derangement.


1866 ◽  
Vol 12 (59) ◽  
pp. 348-367
Author(s):  
Franz Meschede ◽  
G. F. Blandford

The disorder commonly called “general paralysis of the insane” presents so many points of interest to the pathologist and the physician, that as a necessary consequence it forms the commonest topic among the writings of those who specially study insanity. But after so much observation and so many treatises, it is disheartening to find that even now scarcely more than one fact with regard to it is laid down as settled and established beyond the possibility of doubt. One there is, the saddest that can be. It is, that for this malady we hitherto have found no cure; that to diagnose it is to pronounce the sentence, not only of incurable insanity, but also of speedy death. The marvel of the whole is, that although death occurs in every case at no very distant period, though postmortem examinations of general paralytics are made by hundreds every year in this and other countries, yet even at this day no two observers are agreed as to the pathology and morbid anatomy, as to the part in which it has its origin, or which constitutes its peculiar and proper seat. No wonder that the whole of the morbid anatomy of insane brain is vague and ill-defined, when this, the specially fatal form of mental disease, still hides itself from usâ still wraps itself in the mystery which envelopes all that relates to mind. I make no apology for drawing the attention of the readers of this Journal to a paper on the subject, published in the October and November numbers of ‘ Virchow's Archives/ 1865, and for giving a short and necessarily imperfect summary of its contents, it being too long for reproduction. But as every outline must needs be unsatisfactory, I trust my readers will go themselves to the original. In default of opportunity of examining many brains of paralytic patients, I present as a contribution to the English treatises on the subject these observations of another.


Genes ◽  
2021 ◽  
Vol 12 (5) ◽  
pp. 682
Author(s):  
Matthias Christen ◽  
Nils Janzen ◽  
Anne Fraser ◽  
Adrian C. Sewell ◽  
Vidhya Jagannathan ◽  
...  

A 7-month-old, spayed female, domestic longhair cat with L-2-hydroxyglutaric aciduria (L-2-HGA) was investigated. The aim of this study was to investigate the clinical signs, metabolic changes and underlying genetic defect. The owner of the cat reported a 4-month history of multiple paroxysmal seizure-like episodes, characterized by running around the house, often in circles, with abnormal behavior, bumping into obstacles, salivating and often urinating. The episodes were followed by a period of disorientation and inappetence. Neurological examination revealed an absent bilateral menace response. Routine blood work revealed mild microcytic anemia but biochemistry, ammonia, lactate and pre- and post-prandial bile acids were unremarkable. MRI of the brain identified multifocal, bilaterally symmetrical and T2-weighted hyperintensities within the prosencephalon, mesencephalon and metencephalon, primarily affecting the grey matter. Urinary organic acids identified highly increased levels of L-2-hydroxyglutaric acid. The cat was treated with the anticonvulsants levetiracetam and phenobarbitone and has been seizure-free for 16 months. We sequenced the genome of the affected cat and compared the data to 48 control genomes. L2HGDH, coding for L-2-hydroxyglutarate dehydrogenase, was investigated as the top functional candidate gene. This search revealed a single private protein-changing variant in the affected cat. The identified homozygous variant, XM_023255678.1:c.1301A>G, is predicted to result in an amino acid change in the L2HGDH protein, XP_023111446.1:p.His434Arg. The available clinical and biochemical data together with current knowledge about L2HGDH variants and their functional impact in humans and dogs allow us to classify the p.His434Arg variant as a causative variant for the observed neurological signs in this cat.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Nashaiman Pervaiz ◽  
Hongen Kang ◽  
Yiming Bao ◽  
Amir Ali Abbasi

Abstract Background There has been a rapid increase in the brain size relative to body size during mammalian evolutionary history. In particular, the enlarged and globular brain is the most distinctive anatomical feature of modern humans that set us apart from other extinct and extant primate species. Genetic basis of large brain size in modern humans has largely remained enigmatic. Genes associated with the pathological reduction of brain size (primary microcephaly-MCPH) have the characteristics and functions to be considered ideal candidates to unravel the genetic basis of evolutionary enlargement of human brain size. For instance, the brain size of microcephaly patients is similar to the brain size of Pan troglodyte and the very early hominids like the Sahelanthropus tchadensis and Australopithecus afarensis. Results The present study investigates the molecular evolutionary history of subset of autosomal recessive primary microcephaly (MCPH) genes; CEP135, ZNF335, PHC1, SASS6, CDK6, MFSD2A, CIT, and KIF14 across 48 mammalian species. Codon based substitutions site analysis indicated that ZNF335, SASS6, CIT, and KIF14 have experienced positive selection in eutherian evolutionary history. Estimation of divergent selection pressure revealed that almost all of the MCPH genes analyzed in the present study have maintained their functions throughout the history of placental mammals. Contrary to our expectations, human-specific adoptive evolution was not detected for any of the MCPH genes analyzed in the present study. Conclusion Based on these data it can be inferred that protein-coding sequence of MCPH genes might not be the sole determinant of increase in relative brain size during primate evolutionary history.


2021 ◽  
pp. 104063872110234
Author(s):  
Dah-Jiun Fu ◽  
Akhilesh Ramachandran ◽  
Craig Miller

A 3-y-old, female Quarter Horse with a history of acute neurologic signs was found dead and was submitted for postmortem examination. Areas of petechial and ecchymotic hemorrhage were present on cross-sections of the cerebrum, cerebellum, and brainstem. Histologic examination of the brain revealed severe, purulent meningoencephalitis and vasculitis with a myriad of intralesional gram-positive cocci. Streptococcus pluranimalium was identified from formalin-fixed, paraffin-embedded tissue obtained from sites with active lesions by PCR and nucleotide sequencing of bacterial 16S ribosomal RNA. S. pluranimalium should be considered as a cause of meningoencephalitis in a horse.


Children ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. 78
Author(s):  
Anne Bryden ◽  
Natalie Majors ◽  
Vinay Puri ◽  
Thomas Moriarty

This study examines an 11-year-old boy with a known history of a large previously asymptomatic arachnoid cyst (AC) presenting with acute onset of right facial droop, hemiplegia, and expressive aphasia. Shortly after arrival to the emergency department, the patient exhibited complete resolution of right-sided hemiplegia but developed headache and had persistent word-finding difficulties. Prior to symptom onset while in class at school, there was an absence of reported jerking movements, headache, photophobia, fever, or trauma. At the time of neurology consultation, the physical exam showed mildly delayed cognitive processing but was otherwise unremarkable. The patient underwent MRI scanning of the brain, which revealed left convexity subdural hematohygroma and perirolandic cortex edema resulting from ruptured left frontoparietal AC. He was evaluated by neurosurgery and managed expectantly. He recovered uneventfully and was discharged two days after presentation remaining asymptomatic on subsequent outpatient visits. The family express concerns regarding increased anxiety and mild memory loss since hospitalization.


1888 ◽  
Vol 34 (146) ◽  
pp. 167-176
Author(s):  
G. T. Revington

I think that the foregoing statistics, and those which follow, together with the large number of cases which I quote, and which connect general paralysis with almost every form of neurotic manifestation, will prove conclusively that neurotic inheritance is a striking feature in the causation of general paralysis. I question whether a distinction between “the cerebral and the insane element” in general paralysis can be maintained. If general paralysis is not a degeneration of the mind-tissue, then the pathology of insanity has no existence, and I would say that the subtle influence for evil, which is transmitted from parents, whose brains are deteriorated by neurotic outbursts, or soaked in alcohol, or wrecked by physiological immorality, tends strongly towards such degeneration. If insanity is, as Dr. Savage says, a perversion of the ego, then a general paralytic is the in-sanest of the insane. We know that the children of a melancholic parent, for example, may develop any form of neurosis—in other words, it is not that melancholia or general paralysis, or any other definite disease, is transmitted, but that a certain tendency to deviate from normal development is transmitted. This tendency to deviate is the neurotic diathesis, and the form of its development is determined by collateral circumstances, and a certain series of collateral circumstances determine the development of general paralysis. Perhaps neurotic inheritance may mean in some cases a limited capital of nervous energy, and if this is wasted recklessly the individual breaks down suddenly and pathologically, as we all do slowly and physiologically. I would also point out that considering the number of histories of insanity which owing to ignorance or reticence we, do not receive, and considering that we never receive information as to the existence of the slighter neuroses, it is marvellous that we get so high a percentage as 51. Of the 145 general paralytics with a reliable history, 38 had a family history of insanity, 28 a family history of drink, 8 of both, 43 had a personal history of drink, 8 of a previous attack too remote to be considered, at least, according to our present ideas, as part of the disease, and the vast majority had a history of some physiological irregularity which must be considered as conducive to the creation of an acquired neurosis. We may now pass to some further statistics.


2009 ◽  
Vol 60 (6) ◽  
pp. 495-504 ◽  
Author(s):  
Ioan Balintoni ◽  
Constantin Balica ◽  
Monica Cliveţi ◽  
Li-Qiu Li ◽  
Horst Hann ◽  
...  

The emplacement age of the Muntele Mare Variscan granite (Apuseni Mountains, Romania)Like the Alps and Western Carpathians, the Apuseni Mountains represent a fragment of the Variscan orogen involved in the Alpine crustal shortenings. Thus the more extensive Alpine tectonic unit in the Apuseni Mountains, the Bihor Autochthonous Unit is overlain by several nappe systems. During the Variscan orogeny, the Bihor Unit was a part of the Someş terrane involved as the upper plate in subduction, continental collision and finally in the orogen collapse and exhumation. The Variscan thermotectonic events were marked in the future Bihor Unit by the large Muntele Mare granitoid intrusion, an S-type anatectic body. Zircon U-Pb laser ablation inductively coupled plasma mass spectrometry (LA-ICP-MS) dating yielded a weighted mean age of 290.9 ± 3.0 Ma and a concordia age of 291.1 ± 1.1 Ma. U-Pb isotope dilution zircon analyses yielded a lower intercept crystallization age of 296.6 + 5.7/-6.2 Ma. These two ages coincide in the error limits. Thus, the Muntele Mare granitoid pluton is a sign of the last stage in the Variscan history of the Apuseni Mountains. Many zircon grains show inheritance and/or Pb loss, typical for anatectic granitoid, overprinted by later thermotectonic events.


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