Brown syndrome

1. Brown syndrome is a rare form of strabismus characterized by limited elevation of the affected eye.  It is a mechanical problem in which the superior oblique muscle/tendon (on the outside of the eyeball) is unable to lengthen and therefore does not move freely. This makes looking up and in with the affected eye difficult. Often the higher eye is mistakenly presumed to be the abnormal eye, but it is actually the lower eye that is affected. Brown syndrome causes the affected eye to have trouble looking upward and inwards towards the nose. Essentially the affected eye is “tethered” or held down by the tight superior oblique tendon.The disorder may be congenital (existing at or before birth), or acquired.Acquired Brown syndrome is uncommon but may be seen following surgery, after trauma or in association with inflammatory diseases. Trauma can cause a Brown Syndrome if a blunt object hits the eye socket in the upper inside corner near the nose. Surgery for the eyelid, frontal sinus, eyeball (retinal detachment) and teeth (dental extraction) have been linked to acquired Brown syndrome. Inflammation of the tendon-trochlea complex (from adult and juvenile rheumatoid arthritis, systemic lupus erythematosus and sinusitis) can be associated with development of the problem. Sometimes the cause is never identified.Hereditary cases of Brown syndrome are rare. Most cases arise without a family history (sporadic).
2. Harold W. Brown characterized the syndrome in many ways such as:

   • Limited elevation in the eye when head is straight up
   • Eyes point out in a straight up gaze (divergence in up gaze)
   • Widening of the eyelids in the affected eye on adduction
   • Head tilts backwards (compensatory chin elevation to avoid double vision)
   • Near normal elevation in abduction.

   
   

   Brown syndrome can be classified according to severity. In mild cases there is a reduced ability to look up and in with the affected eye. In moderate cases, there is also a tendency for the eye to move downward as it moves inward. In severe cases there is a tendency for the affected eye to turn downward when the patient looks straight ahead.
3. In Brown's original series there was a 3:2 predominance of women to men. Ninety percent of patients have only one affected eye, more commonly the right. Treatment recommendations for Brown syndrome vary according to the cause and severity of the movement disorder. Close observation alone is usually sufficient in mild cases. Visual acuity and the ability to use both eyes at the same time (binocular vision) should be monitored closely in young children. Nonsurgical treatment is often advised for recently acquired, traumatic and variable cases. Systemic and locally injected corticosteroids have been used to treat inflammatory cases of acquired Brown syndrome. Non-steroidal anti-inflammatory agents (like ibuprofen) have also been used. Surgical treatment is usually recommended if any of the following are present: eye misalignment when looking straight ahead, significant double vision, compromised binocular vision or pronounced abnormal head position. More than one surgery may be needed for optimal management.The goal of surgery is to restore free ocular rotations. Various surgical techniques have been used:
• Harold Brown advocated that the superior oblique tendon be stripped. A procedure named sheathotomy. The results of such a procedure are frequently unsatisfactory because of reformation of scar tissue.
• Tenotomy of the superior oblique tendon (with or with out a tendon spacer) has also been advocated. This has the disadvantage that it frequently produces a superior oblique paresis.
• Weakening of the inferior oblique muscle of the affected eye may be needed to compensate for iatrogenic fourth nerve palsy. 

1. It's also known as Superior Oblique Tendon Sheath syndrome. Not to be confused with Brown-Vialetto-Van Laere syndrome.

Cerebral perfusion pressure

Cerebral perfusion pressure (CPP), the pressure of blood flowing to the brain, is normally fairly constant due to autoregulation, but for abnormal mean arterial pressure (MAP) or abnormal ICP the cerebral perfusion pressure is calculated by subtracting the intracranial pressure from the mean arterial pressure: CPP = MAP − ICP 

Monro-Kellie hypothesis

Monro-Kellie doctrine, or hypothesis, is that the sum of volumes of brain, CSF, and intracranial blood is constant. An increase in one should cause a decrease in one or both of the remaining two. This hypothesis has substantial theoretical implications in increased intracranial pressure and in decreased CSF volume. Many of the MRI abnormalities seen in intracranial hypotension or CSF volume depletion can be explained by the Monro-Kellie hypothesis. These abnormalities include meningeal enhancement, subdural fluid collections, engorgement of cerebral venous sinuses, prominence of the spinal epidural venous plexus, and enlargement of the pituitary gland.

Bell Magendie Law

Bell Magendie Law

In anatomy and neurophysiology, this is the finding that the anterior spinal nerve roots contain only motor fibers and posterior roots only sensory fibers and that nerve impulses are conducted in only one direction in each case.

Exception- The unmyelinated Group C nerve fibers that transmit pain and temperature from the pelvic viscera enter the spinal cord via ventral roots at L5-S3, thus violating the Bell–Magendie law.

Mc Donald Criteria 2010

Sites of Locked in syndrome (deefferantation)

Sites of Locked in syndrome (deefferantation)

1. Basilar artery occlusion
2. B/L ventral pontine lesion
3. Polyneuritis
4. Myasthenia gravis
5. Polio
6. GB Syndrome

Sites causing Ataxic Hemiparesis

Sites causing Ataxic Hemiparesis

1. C/L thalamocapsular
2. C/L posterior limb of internal capsule
3. C/L red nucleus
4. C/L basis pontis- juntion of upper third and lower two thirds
5. Superficial ACA supplying paracentral area

Sites of Dysarthria- Clumsy Hand Syndrome

Sites of Dysarthria- Clumsy Hand Syndrome

1. Genu of internal capsule
2. Small deep cerebellar hemorrhage
3. Basis pontis at junction of upper third and lower two third

Sites of Pure Motor Hemiparesis

Sites of Pure Motor Hemiparesis

A. Intracranial-
1. Posterior limb of Internal Capsule
2. Cerebral Peduncle
3. Medullary pyramid.
4. Basis pontis 

House-Brackmann scale

The House-Brackmann scale is a facial nerve grading system, at one end of the scale there is normal facial nerve function and at the other there is complete paralysis.

Grade	FunctionLevel	Symmetry at Rest	Eye(s)	Mouth	Forehead
I	Normal	Normal	Normal	Normal	Normal
II	Mild	Normal	Easy and complete closure	Slightly asymmetrical	Reasonable function
III	Moderate	Normal	With effort, complete closure	Slightly affected with effort	Slight to Moderate movement
IV	Moderately Severe	Normal	Incomplete closure	Asymmetrical with maximum effort	None
V	Severe	Asymmetry	Incomplete closure	Minimal Movement	None
VI	Total Paralysis	Total Paralysis

Complications of IvIg infusion

Nephrotic syndrome
Aseptic meningitis
Serum sickness
Thrombotic venous/arterial occlusion
Stroke
Hypotension

Wernicke's Encephalopathy- non alcoholic causes

prolonged intravenous feeding,

hyperemesis gravidarum, 

anorexia nervosa, 

refeeding after starvation, 

thyrotoxicosis, 

regional enteritis, 

malabsorption syndromes, 

hemodialysis, 

peritoneal dialysis,

uremia, 

HIV,

malignancy, and 
gastroplasty with postoperative vomiting

Hughes GBS Disability Scale

Guillain-Barré Syndrome Disability Scale (Hughes)

0	Healthy
1	Minor symptoms or signs of neuropathy but capable of manual work/capable of running
2	Able to walk without support of a stick (5m across an open space) but incapable of manual work/running
3	Able to walk with a stick, appliance or support (5m across an open space)
4	Confined to bed or chair bound
5	Requiring assisted ventilation (for any part of the day or night)
6	Death

Asbury Criteria for GBS

Diagnostic criteria
Assessment of current diagnostic criteria for Guillain-Barre syndrome

Required features

• Progressive weakness in both arms and legs
• Areflexia (or hyporeflexia).

Features supportive of diagnosis

• Progression of symptoms over days to 4 weeks
• Relative symmetry
• Mild sensory signs or symptoms
• Cranial nerve involvement, especially bilateral facial weakness
• Recovery beginning 2 to 4 weeks after progression ceases
• Autonomic dysfunction
• Absence of fever at onset
• Typical CSF (albuminocytologic dissociation)
• EMG/nerve conduction studies (characteristic signs of a demyelinating process in the peripheral nerves)

Features casting doubt on the diagnosis

• Asymmetrical weakness
• Persistent bladder and bowel dysfunction
• Bladder or bowel dysfunction at onset
• >50 mononuclear leukocytes/mm³ or presence of polymorphonuclear leukocytes in CSF
• Distinct sensory level.

Features that rule out the diagnosis

• Hexacarbon abuse
• Abnormal porphyrin metabolism
• Recent diphtheria infection
• Lead intoxication
• Other similar conditions: poliomyelitis, botulism, hysterical paralysis, toxic neuropathy.

Uses of phenytoin



Phenytoin is used as both an abortive and preventive medication in seizure management. Intravenous administration of phenytoin ceased seizure activity in 60%-80% of patients in status epilepticus within 20 minutes. Prophylactic indications include pregnancy-induced hypertension, postneurosurgery, cerebrovascular accidents, and traumatic brain injury. Of these, there is inconsistent evidence to support the routine use of phenytoin in patients who have undergone craniectomy.

Less-common uses of phenytoin include the treatment of neuropathic pain, motion sickness, muscular dystrophy, and arrhythmia.

Phenytoin levels



Phenytoin is used as both an abortive and preventive medication in seizure management.
The total phenytoin reference range varies by age, as follows:

• Children and adults: 10-20 µg/mL
• Neonates: 8-15 µg/mL

Toxic phenytoin levels are defined as greater than 30 µg/mL.
Lethal levels are defined as greater than 100 µg/mL.
The reference range of free phenytoin is 1-2.5 µg/mL.
In patients with renal failure associated with hypoalbuminemia, free phenytoin levels may be more accurate than total phenytoin levels.However, the Sheiner-Tozer formula (below) can be used to correct the phenytoin level.
Adjusted concentration = measured total concentration / [(0.2 x albumin) + 0.1].
Administration of phenytoin and interpretation of serum phenytoin levels vary depending on the clinical scenario. Loading doses to achieve rapid therapeutic levels should be checked 1 hour after an intravenous loading dose and 24 hours after an oral loading dose.
Patients who are on long-term phenytoin therapy generally do not need to be monitored at intervals less than 3-12 months after a steady state has been reached unless clinically indicated, for example in patients who may have intentionally or unintentionally taken a toxic dose.
Although the reference range is between 10 and 20 µg/mL, about half of patients’ seizures are controlled at values lower and higher than the therapeutic range.
Some adverse effects of phenytoin are related to specific serum levels. Nystagmus is frequently observed at levels greater than 20 µg/mL.At greater than 30 µg/mL, patients may exhibit slurring of speech, ataxia, and movement disorders such as tremor, choreoathetosis, and orofacial dyskinesia.At serum levels that exceed 40 µg/mL, patients are often lethargic, stuporous, and confused and may require aggressive supportive measures.

Anatomy of Speech

Language processes have a clear neuroanatomical basis. 

In simplest terms, the reception and processing of spoken language take place in the auditory system, beginning with the cochlea and proceeding through a series of way stations to the auditory cortex, the Heschl gyrus, in each superior temporal gyrus. Decoding sounds into linguistic information involves the posterior part of the left superior temporal gyrus, the Wernicke area or Brodmann area 22, which gives access to a network of cortical associations to assign word meanings. For both repetition and spontaneous speech, auditory information is transmitted to the Broca area in the posterior inferior frontal gyrus. This area of cortex “programs” the neurons infrom which descending axons travel to the brainstem cranial nerve nuclei. The inferior parietal lobule, especially the supramarginal gyrus, also may be involved in phoneme processing in language comprehension and in phoneme production for repetition and speech. 

Reading requires perception of visual language stimuli by theoccipital cortex, followed by processing into auditory language information via the heteromodal association cortex of the angular gyrus. Writing involves activation of motor neurons projecting to the arm and hand.

Lateral Ventricle- Parts

There are several areas in the lateral ventricle:
• the frontal horn, which is bounded by the caudate nucleus, Corpus callosum and septum pellucidum
• the body of the lateral ventricle is bounded by the caudate nucleus / thalamus, corpus callosum and fornix
• the atrium: this is the focal point of the occipital and temporal horns
• the occipital horn
• the temporal horn

The two lateral ventricles (right, left) communicate with each other via the third ventricle.

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Etiological Classification of seizures

The classification (database) of etiologies of the epilepsies is divided into four main categories.

Definitions

1. Idiopathic epilepsy—defined here as an epilepsy of predominately genetic or presumed genetic origin and in which there is no gross neuroanatomic or neuropathologic abnormality. Included here are epilepsies of presumed multigenic or complex inheritance, but for which currently the genetic basis has not been elucidated.

2. Symptomatic epilepsy—defined here as an epilepsy of an acquired or genetic cause, associated with gross anatomic or pathologic abnormalities, and/or clinical features, indicative of underlying disease or condition. We thus include in this category developmental and congenital disorders where these are associated with cerebral pathologic changes, whether genetic or acquired (or indeed cryptogenic) in origin. Also included are single gene and other genetic disorders in which epilepsy is

only one feature of a broader phenotype with other cerebral or systemic effects.

3. Provoked epilepsy—defined here as an epilepsy in which a specific systemic or environmental factor is the predominant cause of the seizures and in which there are no gross causative  neuroanatomic or neuropathologic changes. Some ‘‘provoked epilepsies’’ will have a genetic basis and some an acquired basis, but in many no inherent cause can be identified. The reflex epilepsies are

included in this category (which are usually genetic) as well as the epilepsies with a marked seizure precipitant.

4. Cryptogenic epilepsy—defined here as an epilepsy of presumed symptomatic nature in which the cause has not been identified. The number of such cases is diminishing, but currently this is still an important category, accounting for at least 40% of adult-onset cases of epilepsy.

ABCD2 SCALE- TIA