Subarachnoid Hemorrhage

Seeking examples of subarachnoid hemorrhage in ancient texts, McHenry, in Garrison's History of Neurology, mentions possible instances of subarachnoid hemorrhage in the Bible-II Kings iv: 19-20-and in the Hippocratic aphorisms on apoplexy.[1] But neither are more than suggestive.

In 1764, Domenico Cotugno described subarachnoid cerebrospinal fluid and showed that ventricular and spinal fluids are connected. Two years later, in 1766, Albrecht von Haller described the cerebrospinal fluid. Morgagni, in 1761, reported the occurrence of meningeal apoplexy and implied a causal cerebral aneurysm.[1] But the fact that aneurysms could burst and bleed was not clinically evident for another 50 years. McHenry cites “A case of aneurysm of the carotid arteries,” [a paper I have not seen] of Gilbert Blane (1800) that provided “the best clinical picture and autopsy findings … John Hunter performed the autopsy.”

1812 saw the publication of Cases of Apoplexy and Lethargy [2] by John Cheyne (1777-1836) of Dublin, pupil of Charles Bell, which published five plates of apoplexy engraved from drawings in Charles Bell's museum. One plate is the first illustration of subarachnoid bleeding and shows a ruptured aneurysm at the junction of the left internal carotid with anterior cerebral arteries. Cheyne believed that cerebral anemia might be the cause of apoplexy and described pathological cases of cerebral infarction and of cerebral hemorrhage.

In 1813, John Blackall[3] furnished a dramatic case history of a woman:

… attacked suddenly, and without any apparent cause, by a most violent vomiting and diarrhoea, with headach of the most excruciating kind, a sensation as if the scalp were lifted by an internal force, some indistinctness of vision, intolerance of light, & c. She continued many days in most excessive agony, the pupils neither fixed nor at all dilated, and the understanding suffering now and then only a momentary eclipse … About twenty-four hours before death she fell into an apoplectic stupor. On examining the head we found the veins of the pia mater turgid; in each of the lateral ventricles about half an ounce of blood loosely and recently coagulated, with some serum; the third and fourth ventricles filled with a similar substance; and the brain itself in the immediate neighbourhood of these last considerably injured in its texture. The haemorrhage was traced to the basilar artery which nearly at its bifurcation was dilated into an aneurismal sac of the size of a hornbean, and appeared to have opened into the cavities of the brain at the communication between the third and fourth ventricles.

Wepfer had clearly portrayed in his Historiae apoplecticorum published in 1658,[4] [5] a detailed description of four cases of intracerebral hemorrhage, but no case of subarachnoid hemorrhage is apparent.

John Abercrombie (1780-1844) is renowned for one of the earliest texts on neuropathology[6] in 1828. He divided patients into three clinical groups. The first is typically primary cerebral hemorrhage, the third corresponds to infarction or transient ischemic attacks; the second group is strongly reminiscent of subarachnoid hemorrhage:

The disease begins with sudden pain in the head. The patient becomes pale and faint, vomits and often falls. He may or may not be paralysed, but is usually not. Recovery generally occurs, although coma and death may supervene. (Garrison p. 380.)

It was Sir William Withey Gull who first recognized that it was common, yet easily overlooked. The opening paragraph of his paper[7] (reproduced by Sir Charles Symonds[8]) in Guy's Hospital Reports of 1859 states:

There are several reasons why intracranial aneurism is likely to be overlooked. First of all, as here hinted at, it has not been looked for, and it is notorious that the eye can see only that it brings with it the aptitude to see. Again, when death occurs from rupture of the sac, recent coagula may so imbed and conceal it that unless strictly looked for it will not be found, for the sac is often small, and thin and transparent, except at the point of rupture. Further also, when death has taken place from changes around the aneurism, as by pressure or softening, the sac itself may present such appearances that unless a minute dissection be made of it its true nature may not be discovered. Whenever young persons die with symptoms of ingravescent apoplexy, and after death large effusion of blood is found, especially if the effusion be over the surface of the brain in the meshes of the pia mater, the presence of an aneurism is probable.

Symonds in 1924 relates[9] how Gintrac in his book Maladies de l'appareil nerveux, published in 1869, had a chapter on meningeal hemorrhage which included 34 reported instances (two of his own) of subarachnoid hemorrhage with notes on etiology, pathology, and symptomatology. Samuel Wilks (1824-1911) described the pathological anatomy in four cases,[10] and Wichern, Lowy, and James Collier (1870-1935)[11] provided excellent descriptions of symptoms and signs, which yielded a diagnostic picture of subarachnoid hemorrhage.

The 20th century has seen great advances in diagnosis, starting with the Georges Froin's thesis[12] of 1904; using lumbar puncture, he studied the blood stained cerebrospinal fluid and the diagnostic xanthochromia and marked coagulation that he attributed to meningeal irritation. Raven of Max Nonne's clinic showed Froin's syndrome also occurred in spinal block due to tumor. Symonds' superb piece of clinical writing highlights the salient features and describes five cases where aneurysm was diagnosed before death (see Cushing[13] and Symonds[8] [9]).

The last 50 years have demonstrated that excluding head injury, aneurysms of the vessels comprising the arterial circle of Willis account for ~85% of patients with subarachnoid hemorrhage.[14] The incidence is about 6 cases per 100,000 patient years. The “berry” aneurysms are saccular dilations sited at branches of the arterial tree. They occur at places where the media and elastica of the vessel were believed to be congenitally defective, but this is now doubted.[15] Aneurysms, varying from 2 mm to 3 cm or more in diameter occur in an estimated 1 to 2% of the population. The larger the aneurysm, the more likely it is to rupture. The incidence increases during the first three decades of life, and are often multiple. Approximately two thirds rupture and, including pre-hospital deaths (~10%), fatality is 40 to 50% overall; one third of survivors remain disabled. Neurosurgical clipping or insertion of endovascular coils are employed to reduce the risk of recurrent bleeding.

REFERENCES

• 1 McHenry L C. Garrison's History of Neurology. Springfield, IL; Charles C Thomas 1969: 11, 24
• 2 Cheyne J. Cases of Apoplexy and Lethargy: With Observations Upon the Comatose Diseases. London: Thomas Underwood; 1812
• 3 Blackall J. Observations on the Nature and Cure of Dropsies. London; Longman, Hurst Rees, Orne and Brown 1813 132-133 History cited by Bederson JB Subarachnoid Hemorrhage, Patholophysiology and Management. New York; American Association of Neurological Surgeons 1997 11
• 4 Wepfer J J. Historiae apoplecticorum. 1658. English translation from Bagvili's Practice of Physik, London 1704: cited in Major RH. Classic Descriptions of Disease. 3rd ed. Springfield, IL; Charles C Thomas 1945: 474-477
• 5 Pearce JMS. Johann Jakob Wepfer (1620-1695) and cerebral haemorrhage.  J Neurol Neurosurg Psychiatry. 1997;  62 387
• 6 Abercrombie J. Pathological and Practical Researches on Diseases of the Brain and Spinal Cord. Edinburgh; Waugh and Innes 1828
• 7 Gull W W. Guy's Hospital Reports, Third Series, Vol. 281. 1859
• 8 Symonds C P. Contributions to the clinical study of intracranial aneurysms.  Guys Hosp Rep. 1923;  73 139-158
• 9 Symonds C P. Spontaneous subarachnoid haemorrhage.  Q J Med. 1924;  18 93-122
• 10 Wilks S. Lectures on Diseases of the Nervous System. Delivered at Guy's Hospital. London; J&A Churchill 1878
• 11 Collier J. In: Price FW Textbook of the Practice of Medicine. London; Oxford Medical Publications 1922 pp. 1351 et seq
• 12 Froin G. Les Hémorragies sous-arachnoïdennes et le mécanisme de l'hématologie en general [thesis]. Paris; 1904
• 13 Cushing H. Contributions to the clinical study of cerebral aneurysms.  Guys Hosp Rep. 1923;  73 159-163
• 14 van Gijn J, Rinkel GJE. Subarachnoid haemorrhage: diagnosis, causes and management.  Brain. 2001;  124 249-278
• 15 Stehbens W E. Etiology of intracranial berry aneurysms.  J Neurosurg. 1989;  70 823-831