Brain hemorrhage, Stroke or Cerebrovascular accident ( CVA) or Brain attack or Paralysis

The term "Heart attack" is very common used term and well understood by common people for a condition in which heart is affected and patient requires urgent medical treatmnt. Similarly in stroke, blood supply to the brain is affected and patient requires urgent medical attention.  

The brain is critically dependent on an uninterrupted supply of oxygenated blood. About 18% of the total blood volume in body circulates in the brain, which accounts for about 2% of body weight. Loss of consciousness occurs in less than 15 seconds after blood flow to the brain has stopped, and irreparable damage to the brain tissue occurs within 5 minutes.

Cerebrovascular accident or cerebrovascular disease or stroke occurs as a result of vascular compromise or hemorrhage and is one of the most frequent sources of neurologic disability.
Abrupt onset of Neurologic deficit is caused by inadequate perfusion of a region of brain.

Stroke is a common cause of neurological disability and death in elderly persons. Arterial thrombosis with occlusion of the cerebral arteries is the most common cause of stroke.

Most common modifiable risk factors are hypertension, cigarette smoking, obesity, increased blood lipids, heavy alcohol consumption, poor control of diabetes mellitus, stress, etc.

TIA ( Transient ischemic attack)
            Episode of focal neurological dysfunction as a result of ischemia which resolves completely within 24 hours.
            TIA are important determinant of stroke. around 30-50% of cases had previous transient ischemic attacks.

STROKE or CVA

About 85% of strokes are Ischemic and 15% Hemorrhagic.

HEMORRHAGIC STROKE
About 20% of strokes are hemorrhagic which is due to the spontaneous intracerebral hematoma (ICH). Hemorrhage most commonly results from rupture of the small penetrating arteries damaged by the degenerative effects of chronic hypertension.. In 1868, Charcot and Bouchard described the rupture of " microaneurysms" as the cause of ICH.

Common cause of spontaneous intracerbral hematoma in elderly is hypertensive bleed. As commonly seen in elderly that there is unnoticed hypertension in many elderly persons who are not aware about this condition or on irregular treatment of hypertension. Common site of hypertensive bleed is basal ganglia. 
So, the commonest cause of spontaneous intracerebral hematoma in adults is a hypertensive arteriosclerotic basal ganglionic bleed. The median age of spontaneous intracerebral hemorrhage is about 56 years.The common clinical features are sudden onset severe headache, vomiting, slurring of speech, depressed level of consciousness and weakness of face and limbs. 
Commonest cause is long standing hypertension, irregular antihypertensive medication, history of smoking and alcohol intake, diabetes and lack of physical exercise. 
CT scan of brain is the initial investigation.  

Basal ganglia ( Putamen, globus pallidus, caudate nucleus) is the commonest site of the hypertensive intracranial bleed.

Fibrous Dysplasia

 Fibrous dysplasia is usually a benign condition in which normal bone is replaced by fibrous connective tissue ( malignant transformation occurs in less than 1%). Most lesions occur in the ribs or craniofacial bones, especially maxilla.
Anatomical patterns may be Monostotic: most common,Polyostotic: 25%, & as part of McCune-Albright sdyndrome.
Clinically it may present as incidental finding, local pain, local swelling, pathologic fracture, cranial nerve compression
Fibrous dysplasia consists of proliferative connective tissue, causing thickening of bones.
There are 3 forms: Compact form, Lytic form and Pseudo pagetoid form.
1. Compact form is a dense thickening of bone , especially of the skull base, resulting in ground glass appearance. It may cause stenosis of the optic foramen , superior orbital fissure,  shallow orbits with proptosis, sellar and sphenoid involvement causing hypopituitarism and expansion of the temporal bone and greater wing of the sphenoid.
2. Lytic form takes the shape of a radiolucent area limited by a thin sclerotic line.
3. Pseudo pagetoid form is characterized by a combination of both sclerotic and  radiolucent lesions. The lesion stabilizes after the age of 25-30. There is a small risk of malignant transformation.


Investigation: Rraised serum alkaline phosphatase level,X Ray, CT scan & MRI.
Treatment of calvarial kesions: curettage and cranioplasty.

Sources: Manual of Neurosurgery,  Ramamurthy & Tandon, (jaypee publishers)
               Handbook of neurosurgery Greenberg, 7th ed ( Thieme) 

Coma and Glasgow Coma scale

Most of the non medical people use word coma to describe a patient who is unconscious. But for a medical professional word "coma" is very specific because the impairment of arousal can vary from drowsiness ( sleepiness) to non-responding to any stimulus like sound or pain. Coma is the severest impairment of arousal, and is defined as the inability to obey commands, speak, or open  the eyes to pain.

One should learn the GCS scale to better understand the different levels of impairment of conscious level and to avoid descrepencies in describing the daily condition of the patient by different medical professionals and nurses.
Teasdale and Jennet,  in year 1875,  proposed a scale known as GCS ( Glasgow Coma Scale). Three types of stimulus and response to the patient to these three stimuli is described.
First is EYE OPENING
If patient opens his eyes spontaneously , i.e., like a normal person without any problem , then 4 point is mentioned.
Next situation is that patient is drowsy or feeling sleepy and is having closed eyes. The sleepy patient if opens eyes on sound then 3 point is given.
If patient eyes are closed and he opens eyes only when painful stimulus is given the, only 2 points is given.
And patient does not open eyes even on a painful stimulus then only 1 point is given so the lowest score of eye opening is 1.

E  4   spontaneous eye opening
E  3   opening eyes to speech
E  2   opening eyes to pain
E  1   None


Then  patient's verbal response is examined ( V stands for verbal response)


 V   5         Person is oriented 
                 (aware about what is happening around, Person is oriented to place, person and time )
 
 V    4        Confused or disoriented  

 V    3       speaking inappropriate words 
                                      ( Not producing sentences )

 V    2       producing incomprehensible words 
                                       ( Not producing word i.e. only some sound is produced by the patient) 

 V    1      None ( No verbal output means patient is not speaking and even not producing any sound)


Patient's Motor response is assessed

M      6      Obeys
          (Best motor resonpse is  M6 when patients moves limbs themselves and obey the command to move hand and feet whenever asked to do so)

M     5    patient localizes pain 
           ( when patient is pinched he tries to remove your fingers) 

M    4    Withdraws to pain
            ( here when patient is pinched feels pain and tries to withdraw from the pain)

M    3    Flexion to pain ( decorticate)
             ( in medical terminology it is known as decorticate posture , i.e., posture seen in an animal when the central nervous system is cut just below the level of cerebral cortex. Like in an experient by Sherrington, father of modern neurophysiology, when the brain of a cat was cut just  above the midbrain or brain stem, animal,s upper limbs were flexed and lower limbs were extended. This abnormal posture is known as DECORTICATE POSTURE)

M    2   Extensor ( decerebrate )
               ( extensor response to a painful stimulus is a very bad neurological sign. When a patient is pinched his both upper and lower limbs are extended)

M    1  No response to the painful stimulus


The best responses of the patient are added . So, the maximum GCS score is 15 and minimum is 3.

 
Glasgow coma scale score of equal or less than 8 is a generally accepted operational definition of coma.
It can result from dysfunction of brain stem, diencephalon or lesions of both cerebral hemispheres.This may be due to neoplastic lesions, electrolyte imbalance, metablic or endocrine problems, vascular lesions, infections, trauma or nutritional reasons.

GCS is an important method of describing patient's neurological condition but blood pressure, pulse rate,  respiratory rate, response of the pupils of  eye to light, paralysis of the limbs are other important parts of the  complete neurological assessment.

Pilocytic Astrocytoma and Pilomyxoid Astrocytoma

Pilocytic astrocytoma  is a relatively circumscribed, slowly growing, and is  often cystic lesion.
It commonly occurs in in children and young adults.
It is histologically characterized by a biphasic pattern with varying proportions of compacted bipolar cells associated with Rosenthal fibers and loose -textured multipolar cells associated with microcysts and eosinophilic granular bodies.
Rosenthal fibers are intracytoplasmic  corkscrew-shaped, brightly eosinophilic, hyaline masses.
Pilocytic astrocytoma corresponds to WHO grade1. Pilocytic astrocytomas comprise approximately 5% of all gliomas. It is the most common glioma in children, in whom the majority ( 67%) arise in the cerebellum.
Preferred sites include the optic nerve, optic chiasma/ hypothalamus, thalamus and basal ganglia, cerebral hemispheres , cerebellum and brain stem.
Involvement of the subarachnoid space is a common finding in pilocytic astrocytoma. It is not indicative of aggressive or malignant behavior, nor does it pretend subarachnoid dissemination.
Pilocytic astrocytoma may also seed the neuraxis.
Pilocytic astrocytomas are remarkable in maintaining their WHO grade I status over years and even decades. Very rarely, especially when previously irradiated, a malignat transformation may occur.
As with other CNS lesions, the neurological sign and symptoms depend on the location and size of the tumor. similarly non localizing signs like macrocephaly, headache, endocrinopathy are also seen. Seizures are uncommon since the lesions infrequently involve the cerebral cortex. Visual loss, hypothalamic and pituitary dysfunction like obesity and diabetes insipidus may occur.
CT scan brain  with contrast or MRI brain with contrast with MR spectroscopy helps in preoperative provisional diagnosis of the lesion. The cysts may be either solitary and massive , the tumor being a mural nodule, or multiple, smaller and intratumoral.

Pilomyxoid Astrocytoma

A piloid neoplasm, closely related to pilocytic astrocytoma, that has a prominant mucoid matrix and angiocentric arrangement of monomorphous, bipolar tumor cells, without Rosenthal fibers  or eosinophilic granular bodies/ hyaline deposits. Pilomyxoid astrocytoma corresponds to
WHO Grade II neoplasm. It typically presents in very young age group , median 10 months.



 

Primary Central Nervous System Lymphoma ( PCNL)

Extranodal malignant lymphomas may arise in the CNS in the absence of lymphoma outside the nervous system at the time of diagnosis. it is different from secondary involovement of the nervous system in systemic lymphomas.
Primary CNS lymphomas ( PCNL) were first described by Bailey in 1929 as "perithelial sarcoma."
Primary CNS lymphoma constitutes about 3% of all brain tumors.
Most are large B-cell lymphomas , only 2% of T-cell origin.
PCNL affects all ages, with a peak incidence in immunocompetent subjects during the sixth and seventh decade of life.
In immunocompromised patients, the age at manifestation is lowest in individuals who have an inherited immunodeficiency ( 10 years ) , followed by transplant recipients ( 37 years ) and AIDS patients ( 39 years ).
Common location is the brain parenchyma surrounding the ventricular system, but any craniospinal structure can be involved. AIDS epidemic led to increase in the incidence of Lymphoma.  The  clinical manifestations are similar to other intracranial space occupying lesions of the brain and like other brain tumors CT scan and MRI with contrast and MR spectroscopy helps to diagnose these tumors.
Majority of patients present with focal neurological deficit. Some patients present with features of raised intracranial pressure like headache and vomiting. Few patients present with neuropsychiatric symptoms and seizures. Eye symptoms due to uveitis or vitreous lymphoma may occur in about 5 percent cases. Dementia may also be a symptom of cns lymphoma.
About 50% of transplantation associated primary cns lymphomas appear within a year after transplantation.
PCNSL occur as a single or multiple masses in in deep location in cerebral hemisphere or in the peri ventricular region. It may be diffuse or well demarcated. Diffuse infiltrative form may present as lymphomatous cerebri. Meningeal lymphoma may present as meningioma or meningitis.
On CT scan it is infiltrative lesion with indistinct margins and isodense or hyperdense with homogeneous enhancement with minimal surrounding edema or compressive effect.  Solitary lesion in majority of cases. Lesions are usually supratentorial and localized in the deep periventricular areas.
Clinical presentation is similar to any other space occupying lesion. Steroid , cerebral decongestants and antiepileptic medication are given and a tissue diagnosis is possible with open biopsy and stereotactic biopsy.

2016- World Health Organization (WHO) classification of CNS tumors has classified the Lymphomas of the central nervous system as follows:

Diffuse large B-cell lymphoma of the CNS

Immunodeficiency-associated CNS lymphoma

          AIDS-related diffuse large B-cell lymphoma

          EBV-positive diffuse large B-cell lymphoma, NOS

          Lymphomatoid granulomatosis

Intravascular large B-cell lymphoma

Low-grade B-cell lymphoma of the CNS

T-cell & NK/T cell-lymphoma of the CNS

Anaplastic large cell lymphoma , ALK-positive

Anaplastic large cell lymphoma , ALK-negative

MALT lymphoma of the dura

Source: WHO classification of CNS tumors, 4th Ed. 2007 and 2016 update

Gazi Yaşargil: Founder of Microneurosurgery

 

Mahmut Gazi Yaşargil is a Turkish medical scientist and neurosurgeon (born on July 6, 1925 in Lice, Diyarbakır, Turkey). He is the founder of microneurosurgery. Yaşargil treated epilepsy and brain tumors with instruments of his own design. From 1953 until his retirement in 1993 he was first resident, chief resident and then professor and chairman of the Department of Neurosurgery, University of Zurich and the Zurich University Hospital. In 1999 he was honored as "Neurosurgery’s Man of the Century 1950-1999" at the Congress of Neurological Surgeons Annual Meeting.

After attending Ankara Atatürk Lisesi and Ankara University in Ankara, Turkey between 1931 and 1943, he went to Germany to study medicine at the Friedrich Schiller University of Jena, Germany. His genius in developing microsurgical techniques for use in cerebrovascular neurosurgery transformed the outcomes of patients with conditions that were previously inoperable. In 1969 Yaşargil became associate professor and in 1973 professor and chairman of the Department of Neurosurgery, University of Zurich succeeding his mentor, Prof. Krayenbuhl. Over the next 20 years, he carried out laboratory work and clinical applications of micro techniques, performing 7500 intracranial operations in Zurich until his retirement in 1993. In 1994, Yaşargil accepted an appointment as Professor of Neurosurgery at the College of Medicine, University of Arkansas for Medical Sciences in Little Rock where he is still active in the practice of micro-neurosurgery, research, and teaching.

Together with Harvey Cushing, Yaşargil is hailed as one of the greatest neurosurgeons of the twentieth century. He has helped three generations of neurosurgeons, defining what is possible in neurosurgery, and then demonstrating how to achieve it. In the micro-neurosurgical anatomical laboratory in Zurich he trained around 3000 colleagues from all continents and representing all surgical specialties.

He is married to Dianne Bader-Gibson Yaşargil, who was the nurse in charge of the operating suite by his side since 1973, and is still assisting him in surgery.

Yaşargil published his surgical experiences in 330 papers and 13 monographs. The six-volume publication Microneurosurgery (1984–1996, Georg Thieme Verlag Stuttgart-New York) is the comprehensive review of his broad experiences and a major contribution to the neurosurgery literature.

Sources:

Wikipedia

http://www.neurochirurgie-karlsruhe-online.de

HUGO KRAYENBÜHL (1902-1985)

 

 

 

                                                     

HUGO KRAYENBÜHL was born in Zihlschlacht, Switzerland on December 3, 1902. He studied medicine in Geneva, Kiel, Paris, and Zürich. He became a Doctor of Medicine from University of Zurich in 1928

After postgraduate training Dr. Krayenbühl worked from 1934 to 1936 at the London Hospital, London, England, under Sir Hugh Cairns. Through Mr. Cairns he became familiar with the techniques of Harvey Cushing and the traditions of the Cushing school.

Upon his return to Zürich in 1936, Dr. Krayenbuhl worked at the Clinic of General Surgery of the University of Zurich. He began to build his own department of neurosurgery. In 1939, the Clinic was officially recognized as an independent unit, and in 1948, he was given the first chair of Neurosurgery in Switzerland.

 

Professor Hugo Krayenbühl, the founder of Swiss Neurosurgery, has not only been a brilliant surgeon and physician of highest moral standards but also an exceptional teacher. He trained not only young Swiss neurosurgeons but also a large number of foreign pupils who later returned to their countries of origin and are now leaders in their field. It was his strong belief that progress in every medical field is only possible with international cooperation. Therefore, as a president of the Société de Neurochirurgie de Langue Française he organized the first European Congress of Neurosurgery that took place from July 16 to 19, 1959 in Zürich. This led to the foundation of the European Association of Neurological Surgeons in 1971 in Prague whose primary object is “to promote the free interchange of neurosurgical knowledge and experience among the member Societies”.

Through his research and publications, he made contributions to many areas of neurosurgery. Since 1941, when his "Habilitationsschrift" on aneurysms of the brains was published, he was concerned with disturbances of cerebral venous, carotid, and vertebral thrombosis and spasms of the cerebral arteries. His endeavors in the field of diagnostic and therapeutic problems of aneurysms of the brain, culminated in the publication of The Cerebral Angiography, 1965, with M.G. Yasargil. This work has been translated into Italian (1967) and English (1968). His total number of published books and papers was more than 180.

In conjunction with Prof. Yasargil, he developed microsurgery for aneurysms. This highly developed technique brought patients from all over the world. Students came from across Europe to study at the Neurosurgical Clinic. This made Zürich a center for vascular surgery respected internationally.

Sources:

The society of Neurological Surgeons, USA
M. Gazi Yaşargil.  Hugo Krayenbühl — An Appreciation . Advances and Technical Standards in Neurosurgery Volume 11, 1984, pp 1-3