Primary spontaneous mesencephalic hemorrhage
see also Thalamo-mesencephalic hemorrhage.
Primary tectal mesencephalic hemorrhage.
Non-traumatic, spontaneous primary mesencephalic hemorrhage is extremely rare. Of the few reported cases to date, most were associated with blood dyscrasias, vascular malformations or hypertension 1) 2) 3) 4) 5) 6).
Midbrain hemorrhage has also been seen in a small group of normotensive patients with negative angiograms and normal laboratory studies 7).
In addition, a more clinically benign form of non-aneurysmal subarachnoid hemorrhage with a perimesencephalic focus has been described 8).
primary mesencephalic hemorrhage is a distinct clinicopathological entity with a wide clinical and etiological spectrum. It differs from primary thalamic hemorrhage, pontine hemorrhage and associated intraparenchymal hemorrhages by a number of phenomenological, prognostic and neuroimaging features 9).
The diagnosis should be considered in the premonitory or secondary phase of any neurovascular illness in which mesencephalic syndromes and even subtle evidence of raised intracranial pressure are present. When such cases are confirmed by screening neuroimaging studies, an immediate inquiry into potentially remediable, occult prediposing factors should be undertaken.
Turkes M. Primary spontaneous mesencephalic hemorrhage. Acta Neurol Belg. 2017 Mar;117(1):311-312. doi: 10.1007/s13760-016-0702-6. Epub 2016 Sep 30. PubMed PMID: 27696213.
Martínez-Simón J, Sáez-Zea C, Katati MJ, Escamilla-Sevilla F, Mínguez-Castellanos A. Posthemorrhagic Hemiparkinsonism Treated by Unilateral Pallidal Stimulation. Mov Disord Clin Pract. 2014 May 19;1(2):139-141. doi: 10.1002/mdc3.12024. eCollection 2014 Jun. PubMed PMID: 30363849; PubMed Central PMCID: PMC6182986.
Case reports
A 62-year-old woman presented with gait instability and dysarthria. MRI and angiography revealed a diffuse vascular network involving the tectum and cerebellar vermis with intermingled brain parenchyma. This lesion had no dominant feeder, high-flow arteriovenous shunt, flow-related aneurysm or highly dilated veins on angiogram. These findings were consistent with a diagnosis of CPA. During follow-up, she developed progressive gait instability and eye movement abnormalities, but no remarkable change was detected on the repeated MRI and angiography. Nine years later, she died of mesencephalic hemorrhage originating from the CPA. To the best of our knowledge, this is the first description of a patient with CPA who died as a result of the initial hemorrhage. It is important to recognize that a part of CPAs is aggressive and can be more vulnerable to critical hemorrhage 10).
The case histories of two patients with clinically diverse forms of the very rare entity primary, spontaneous mesencephalic hemorrhage are presented. Each exhibited characteristic mesencephalic signs and neuroimaging profiles. However, both patients differed markedly in their predisposing factors, state of arousal, memory, temporal course and functional residua. One patient was unique in displaying a normal level of alertness throughout his clinical course and an isolated retrograde amnesia. The latter sign suggests a role for mesencephalic reticular activation in long-term retrieval and illustrates the differential effects of a subtle activation deficit on memory and arousal. These findings broaden the distinctive clinico-anatomical profile of primary, spontaneous mesencephalic hemorrhage 11).
5: Tarnutzer AA, Palla A, Marti S, Schuknecht B, Straumann D. Hypertrophy of the inferior olivary nucleus impacts perception of gravity. Front Neurol. 2012 May 11;3:79. doi: 10.3389/fneur.2012.00079. eCollection 2012. PubMed PMID: 22593754; PubMed Central PMCID: PMC3350027.
6: Goddeau RP Jr, Caplan LR, Alhazzani AA. Intraparenchymal hemorrhage in a patient with osteogenesis imperfecta and plasminogen activator inhibitor-1 deficiency. Arch Neurol. 2010 Feb;67(2):236-8. doi: 10.1001/archneurol.2009.319. PubMed PMID: 20142533.
8: Kimiskidis VK, Lalaki P, Papagiannopoulos S, Tsitouridis I, Tolika T, Serasli E, Kazis D, Tsara V, Tsalighopoulos MG, Kazis A. Sensorineural hearing loss and word deafness caused by a mesencephalic lesion: clinicoelectrophysiologic correlations. Otol Neurotol. 2004 Mar;25(2):178-82. PubMed PMID: 15021780.
9: Ishihara K, Furutani R, Shiota J, Kawamura M. [A case presenting with trochlear nerve palsy and segmental sensory disturbance due to circumscribed midbrain and upper pontine hemorrhage]. Rinsho Shinkeigaku. 2003 Jul;43(7):417-21. Review. Japanese. PubMed PMID: 14582368.
10: Işikay CT, Yücesan C, Yücemen N, Culcuoglu A, Mutluer N. Isolated nuclear oculomotor nerve syndrome due to mesencephalic hematoma. Acta Neurol Belg. 2000 Dec;100(4):248-51. PubMed PMID: 11233682.
11: Rodríguez-Gómez J, Colás J, Aragón A, Albo MI, Casado F. [Upward and downward gaze palsy with a unilateral mesencephalic hemorrhage]. Rev Neurol. 2000 Feb 16-29;30(4):324-6. Spanish. PubMed PMID: 10789140.
12: Lee AG, Brown DG, Diaz PJ. Dorsal midbrain syndrome due to mesencephalic hemorrhage. Case report with serial imaging. J Neuroophthalmol. 1996 Dec;16(4):281-5. PubMed PMID: 8956166.
13: Bolzani W, Rognone F, Savoldi F, Montalbetti L. [Colliculus hemorrhage]. Rev Neurol (Paris). 1996 Aug-Sep;152(8-9):548-51. Review. French. PubMed PMID: 8991177.
14: Hattori S, Utsunomiya K. [Isolated oculomotor nerve palsy caused by mesencephalic hemorrhage]. Rinsho Shinkeigaku. 1994 Oct;34(10):1021-5. Japanese. PubMed PMID: 7834946.
15: Roig C, Gironell A, Martí-Vilalta JL, Grau JM, Barraquer L. [Nuclear oculomotor nerve syndrome due to mesencephalic infarction or hemorrhage. Five cases and a review of literature]. Neurologia. 1994 Jun-Jul;9(6):224-32. Review. Spanish. PubMed PMID: 8086184.
16: Rinkel GJ, Wijdicks EF, van Gijn J. [Peri-mesencephalic hemorrhage: a benign subarachnoid hemorrhage]. Ned Tijdschr Geneeskd. 1992 Aug 8;136(32):1537-8. Dutch. PubMed PMID: 1528276.
17: Miyagi K, Imaizumi T, Nagura H, Yamanouchi H. [A case of transient parkinsonism due to mesencephalic hemorrhage]. Rinsho Shinkeigaku. 1992 Jul;32(7):743-6. Japanese. PubMed PMID: 1291168.
18: Ryu SJ, Chen KC, Lee CC. [Primary mesencephalic hemorrhage: report of four cases]. J Formos Med Assoc. 1991 Sep;90(9):904-7. Chinese. PubMed PMID: 1683395.
19: Iwanaga K, Kinoshita I, Ohe N. [Brainstem ptosis (midbrain ptosis) associated with mesencephalic hemorrhage]. Rinsho Shinkeigaku. 1990 Jul;30(7):793-5. Japanese. PubMed PMID: 2242638.
20: Gaymard B, Larmande P, de Toffol B, Autret A. Reversible nuclear oculomotor nerve paralysis. Caused by a primary mesencephalic hemorrhage. Eur Neurol. 1990;30(3):128-31. PubMed PMID: 2358005.
21: Tronci S, Congia S. Spontaneous mesencephalic hemorrhage: case report and physiopathological interpretation of its course. Ital J Neurol Sci. 1989 Apr;10(2):183-5. PubMed PMID: 2737864.
22: Han SH, Roh JK, Myung HJ. Mesencephalic hemorrhage–a report of 3 cases. J Korean Med Sci. 1989 Mar;4(1):1-5. PubMed PMID: 2789733; PubMed Central PMCID: PMC3053681.
23: Shuaib A, Israelian G, Lee MA. Mesencephalic hemorrhage and unilateral pupillary deficit. J Clin Neuroophthalmol. 1989 Mar;9(1):47-9. PubMed PMID: 2522947.
24: Mehler MF, Ragone PS. Primary spontaneous mesencephalic hemorrhage. Can J Neurol Sci. 1988 Nov;15(4):435-8. PubMed PMID: 3208231.
26: Shuaib A, Murphy W. Mesencephalic hemorrhage and third nerve palsy. J Comput Tomogr. 1987 Oct;11(4):385-8. PubMed PMID: 3443013.
27: Kamei T, Uchiyama F, Fukuyama J. [Primary tectal mesencephalic hemorrhage with isolated trochlear nerve palsy. A case report]. Rinsho Shinkeigaku. 1987 Sep;27(9):1167-9. Japanese. PubMed PMID: 3440362.
28: Stern LZ, Bernick C. Spontaneous, isolated, mesencephalic hemorrhage. Neurology. 1986 Dec;36(12):1627. PubMed PMID: 3785684.
The first case was reported in 1949.
The clinical diagnosis of a brainstem tumor was changed to hemorrhage after the surgery. Between 1978 and 1991, more than 20 additional patients were reported in the literature
Spontaneous (nontraumatic) midbrain hemorrhage (SMH) is an uncommon condition whose diagnosis is greatly assisted by the use of cranial computerized tomography. Of 18 cases described in the English language literature, only two were diagnosed without the aid of CT.
Fingerote et al. report five cases of SMH in five normotensive patients. Vertical gaze palsy were noted in four patients, headache in four, pupillary dysfunction in four, mild hemiplegia in two, unilateral ataxia in two, and unilateral asterixis in one. The diagnosis of SMH had not been considered before CT scanning in any of these patients. All patients had partial to complete recovery. Cerebral angiography in each case showed no abnormalities in the area of the hemorrhage 12).