Balo's Concentric Sclerosis

Balo's Concentric Sclerosis is an uncommon demyelinating disease.
It is characterized by alternative bands of demyelination and myelin preservation, often in whorl-like configurations.

Here T2 and postcontrast T1W images showing a large lesion in the left hemisphere with alternating T2-hyperintense and isointense bands.
On the T1W images after gadolinium there is alternating linear enhancement.
There is a smaller, similar lesion on the right.

LETM MRI (longitudinally extensive transverse myelitis)

Detection of a LETM spinal cord lesion associated with acute myelitis is the most specific neuroimaging characteristic of NMOSD (>3 vertebral segment longitudinally extensive transverse myelitis lesions)

(LETM) in NMO

Such lesions typically involve
—the central gray matter—cord swelling, —central hypointensity on T1- weighted sequences, and —enhancement following IV gadolinium administration; —extension of a cervical lesion into the brainstem is characteristic.

LETM pattern is characteristic of NMOSD - —7%–14% of initial and 8% of subsequent myelitis attacks in AQP4-IgG-seropositive patients do not meet the LETM definition.

LETM MRI pattern may also occur in patients with
infectious,
granulomatous,
neoplastic, and
paraneoplastic diseases,
acute disseminated encephalomyelitis (ADEM),
spinal cord infarction,
and dural arteriovenous fistula

Adson's sign

ADSON'S SIGN

 

Adson's sign is the loss of the radial pulse in the arm by rotating head to the ipsilateral side with extended neck following deep inspiration. This is accompanied by compression of the Subclavian artery by a cervical rib or tightened anterior and middle scalene muscles. Thoracic outlet obstruction may be caused by a number of abnormalities, including degenerative or bony disorders, trauma to the cervical spine, fibromuscular bands, vascular abnormalities, and spasm of the anterior scalene muscle. Symptoms are due to compression of the brachial plexus and subclavian vasculature, and consist of complaints ranging from diffuse arm pain to a sensation of arm fatigue, frequently aggravated by carrying anything in the ipsilateral hand or doing overhead work such as window cleaning.

Performed as follows

1. Patient in upright position.
2. Passively extend, abduct and externally rotate affected arm while palpating the radial pulse.
3. Ask patient to take a deep breath and hold it in.
4. Ask patient to extend neck and rotate the head towards affected side.

The test is positive if there is a marked decrease, or disappearance, of the radial pulse. It is important to check the patient's radial pulse on the other arm to recognize the patient's normal pulse. It is/was sometimes used as a sign of thoracic outlet syndrome (TOS). Adson's sign is no longer used as a positive diagnosis of TOS since many people without TOS will show a positive Adson's.

There is minimal evidence of interexaminer reliability.

It is named after Alfred Washington Adson.

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.

P

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

Memory

Procedural memory is a part of the long-term memory that is responsible for knowing how to do things, also known as motor skills. As the name implies, procedural memory stores information on how to perform certain procedures, such as walking, talking and riding a bike.  Procedural memory is sometimes referred to as implicit memory, because previous experiences aid in the performance of a task without explicit and conscious awareness of these previous experiences, although it is more properly a subset of implicit memory.

Declarative memory (“knowing what”) is memory of facts and events, and refers to those memories that can be consciously recalled (or "declared"). It is sometimes called explicit memory, since it consists of information that is explicitly stored and retrieved, although it is more properly a subset of explicit memory. Declarative memory can be further sub-divided into episodic memory and semantic memory.

Episodic memory is the memory of autobiographical events (times, places, associated emotions, and other contextual who, what, when, where, why knowledge) that can be explicitly stated. It is the collection of past personal experiences that occurred at a particular time and place.

Neurological Laws

Alexander's law refers to the phenomenon in which the spontaneous nystagmus of a patient with a vestibular lesion is more intense when the patient looks in the quick-phase than in the slow-phase direction. 

Forearm ischemic exercise screening test

There is evidence that ischemic exercise test can be harmful in patients with myopathies. Therefore recently non-ischemic forearm exercise test has been suggested. However in view of the long tradition of ischemic testing and the lack of sensitivity in mitochondrial myopathy the protocol for ischemic testing is given.

While the patient is at rest, blood samples are taken for baseline levels of lactate and ammonia. A blood pressure cuff is then placed over the upper arm and inflated to a pressure higher than systolic, rendering the forearm ischemic. The patient begins immediately repetitive, rapid grip exercises. Normal individuals are able to tolerate ischemic exercise for as long as 180 seconds before pain and fatigue ensues. Patients with glycogen metabolism disorders seldom exercise more than 60 seconds.

When the patient fatigues, the blood pressure cuff is released and 1 minute later blood is drawn from the exercised arm. Similar samples are taken again at 2,4,6,10,and 14 minutes following the end of the exercise.

Normal subjects exhibit a 3 to 5 fold rise in lactate and ammonia levels within 5 minutes after the end of the exercise, with a full return to baseline level about 10-15 minutes after cessation of the test. Failed lactate production suggest a metabolic block (myophosphorylase deficiency (McArdle disease)). The venous level of ammonia also rises during the test. Failure of the lactate and ammonia to rise suggests an inadequate test. Normal lactate but impaired ammonia production suggests myoadenylate deaminase deficiency or a related disorder of purine nucleotide metabolism.

Charcot-Bouchard aneurysms

Charcot-Bouchard aneurysms are a common cause of stroke. If a Charcot-Bouchard aneurysm ruptures, it will lead to an intracerebral hemorrhage.

• French physicians Jean-Martin Charcot and Charles-Joseph Bouchard described it. Bouchard discovered these aneurysms.
• Charcot-Bouchard aneurysms are associated with chronic hypertension.
• They are usually occur due to involvement of small penetrating (<300 µm) lenticulostriate branch of middle cerebral artery in the basal ganglia, brainstem and midbrain.

They are the reason why hypertensive bleed is most common in Basal Ganglia

Stiff Person Syndrome- Antibodies

The most common pathologic correlate is anti–glutamic acid decarboxylase (GAD) antibodies (It has been associated with a wide range of neurologic diseases). 

Additional possible pathophysiologic etiologies in patients negative for GAD antibody include postsynaptic elements such as synaptophysin, amphiphysin, gephyrin, and GABA-transaminase. 

It is also associated with a number of non-neurologic diseases, including diabetes mellitus and thyroiditis.

NOS

A dissociate disorder NOS (Not Otherwise Specified) is a disorder that includes a dissociative symptom (i.e., a disruption in the usually integrated functions of consciousness, memory, identity, or perception of the environment) that does not meet the criteria for any specific Dissociative Disorder. This disorder is no longer recognized in the 2013 DSM-5 and exists now for informational/historical purposes.

Examples-

• Presentations in which a) there are not two or more distinct personality states, or b) amnesia for important personal information does not occur.
• Derealization unaccompanied by depersonalization in adults.
• States of dissociation that occur in individuals who have been subjected to periods of prolonged and intense coercive persuasion (e.g., brainwashing, thought reform, or indoctrination while captive).
• Dissociative trance disorder: single or episodic disturbances in the state of consciousness, identity, or memory that are indigenous to particular locations and cultures. Dissociative trance involves narrowing of awareness of immediate surroundings or stereotyped behaviors or movements that are experienced as being beyond one’s control. Possession trance involves replacement of the customary sense of personal identity by a new identity, attributed to the influence of a spirit, power, deity, or other person, and associated with stereotyped “involuntary” movements or amnesia. Examples include amok (Indonesia), bebainan (Indonesia), latah (Malaysia), pibloktoq (Arctic), ataque de nervios (Latin America), and possession (India). The dissociative or trance disorder is not a normal part of a broadly accepted collective cultural or religious practice. 
• Loss of consciousness, stupor, or coma not attributable to a general medical condition.
• Ganser syndrome: the giving of approximate answers to questions (e.g., “2 plus 2 equals 5″) when not associated with Dissociative Amnesia or Dissociative Fugue.

WFNS system of grading for SAH

Hunt and Hess scale for SAH

Hunt and Hess scale is used to classify the severity of non-traumatic subarachnoid hemorrhage.
1	Asymptomatic, mild headache, slight nuchal rigidity.
2	Moderate to severe headache, nuchal rigidity, no neurologic deficit other than cranial nerve palsy.
3	Drowsiness / confusion, mild focal neurologic deficit.
4	Stupor, moderate-severe hemiparesis.
5	Coma, decerebrate posturing.

Epiconus Syndrome

Anatomically, the epiconus comprises the cord segment between L4 and S1, corresponding to the T12 and L1 vertebrae. The conus medullaris consists of the cord segment between S2 and S5 as well as coccygeal segments. The lumbosacral nerve roots are collectively termed the cauda equina wihch runs laterally and distally to the epiconus as well as the conus medullaris.

A lesion of the epiconus (L4 to S1 spinal cord segments) can produce, as weakness develops, a flaccid type paralysis with signs of lower motor neuron involvement. When the lesion involves the spinal cord above the epiconus, spastic paraesis as a sign of upper motor neuron dysfunction may also occur. Similarly, when the lesion involves the cord distal to the epiconus, bladder dysfunction, as a sign of conus medullaris compromise, and the cauda equina syndrome is usually evident on clinical presentation.

The epiconus syndrome presents with the following clinical features.

(1) A sensory disturbance in the leg (transverse, saddle, radicular, or socks type).

(2) Motor deficit as a sign of lower motor neuron involvement (foot drop, fasciculation, muscle atrophy).

(3) Diminished deep tendon reflexes.

(4) Occasional coexistence of positive pathological reflexes (Babinski's and Chaddock's signs).

(5) Diminished vibration sensation, and

(6) Bladder and bowel dysfunction.

In the presence of a dysfunctional conus medullaris, sensory disturbance in the perineal area (usually saddle type) and diminished vesicourethral as well as anorectal reflexes are significant findings. On the other hand, no significant abnormalities of voluntary leg movement and in deep tendon reflex activity are observed.

The cauda equina syndrome is a well recognised clinical entity with a flaccid type of paresis and/or intermittent neurogenic symptoms, and infrequently, parasympathetic disorders with unusual symptoms of penile erection as well as urinary incontinence.

The differential diagnosis should include old poliomyelitis, tethered cord syndrome as well as spinal dysraphism, amyotrophic lateral sclerosis, hereditary spastic paraplegia, hereditary sensorimotor neuropathy (Charcot-Marie-Tooth; Dejerine-Sottas), and spinal progressive muscular atrophy (Kennedy-Alter-Sung).

Rabbit Syndrome

Rabbit syndrome 
An antipsychotic-induced rhythmic motion of the mouth/lips, resembling the chewing movements of a rabbit. The movement consists of a vertical-only motion, at about 5Hz, with no involvement of the tongue. Usually, the involuntary movements associated with rabbit syndrome appear after a long period (in most cases months or years) of antipsychotic treatment; however, a few patients with the syndrome have had treatment histories with no antipsychotic involvement. 

The reported prevalence of rabbit syndrome ranges from 2.3 to 4.4% of patients treated with typical antipsychotics. There have been isolated reports of rabbit syndrome in patients treated with the atypical agents risperidone and clozapine. 

The most striking aspect of this syndrome is its specificity. Rabbit syndrome affects only the buccal region, and within this area it involves a highly stereotyped involuntary movement. This immediately focuses attention on the basal ganglia, in particular the substantia nigra pars reticulata, which is also implicated in oral dyskinesia. Continuing neurophysiological and pharmacological research of the basal ganglia holds the key to better understanding and treatment of this syndrome in the coming years.

Patients with rabbit syndrome are most often misdiagnosed as having oral tardive dyskinesia. In such cases the key for correct diagnosis is the involvement of tardive tongue movements, which does not occur in rabbit syndrome. 

The treatment of rabbit syndrome is empirical, reflecting poor understanding of its neuropathology. The first step is to reduce the amount of antipsychotic treatment as much as possible. However, since, in most cases, full withdrawal of antipsychotic treatment is impossible, the syndrome cannot be completely abolished without additional measures. The next stage of treatment involves specific drugs that aim to control the syndrome. Anticholinergic drugs are the best known treatment. Rabbit syndrome does not respond to treatment with levodopa or dopamine agonists. 

Stiff Person Syndrome

Stiff Person Syndrome

Occurs due to hyperexcitability of Anterior Horn Cells. Caused by interference of GABA mediated spinal cord inhibitory mechanisms. Symptoms are progressive, painful rigidity punctuated by intense muscle spasm. Affect the axial and paraspinal muscles. Symptoms limited to one limb.

The most common pathologic correlate is anti–glutamic acid decarboxylase (GAD) antibodies (It has been associated with a wide range of neurologic diseases). 
Additional possible pathophysiologic etiologies in patients negative for GAD antibody include postsynaptic elements such as synaptophysin, amphiphysin, gephyrin, and GABA-transaminase. 
It is also associated with a number of non-neurologic diseases, including diabetes mellitus and thyroiditis.

Additional Names- Synonyms

Stiff Person Syndrome- Stiff Man Syndrome,  Moersch Woltman syndrome

Double Crush Syndrome

Double Crush Syndrome

Once a nerve is damaged, it becomes more susceptible to injury elsewhere. The concept remains controversial e.g.
a. a pinched nerve in the neck from a herniated C6 disc increases likelihood of Carpal Tunnel syndrome in hand.
b. a patient with diabeticneuropathy have increased incidence of nerve entrapments in extremities.

If there are 2 points of nerve compression, then decompression of both the regions may be needed to optimize recovery.

While the exact pathophysiology is not known, it is possibly due to disturbance in axonal flow kinetics and the disruption of neurofilament architecture.

Milkmaid Grip

Milkmaid Grip

Milk maids grip is appreciated as an alternating squeezing and releasing of the finger like a milking motion, when asked to maintain a constant, firm grip of examiner's fingers, probably caused by combination of chorea and motor impersistence.

Inability to apply steady pressure during handshake leading to a characteristic squeeze and release of grip has been termed the milkmaid's grip. 

Similarly, patients have difficulty maintaining forced eyelid closure or sustained tongue protrusion. 

Huntington's disease is a progressive neurodegenerative trinucleotide repeat (CAG) disorder characterized by chorea, psychiatric disturbances, oculomotor dysfunction and cognitive decline. 

Motor exam often shows difficulty with fine motor skills and motor impersistence - the inability to maintain sustained voluntary contraction of a muscle group at a constant level. Motor impersistence occurs independently of chorea and has been shown to be linearly progressive over the course of disease suggesting a potential role as a surrogate marker of disease progression.

Also a finding typical of Sydenham’schorea, one of the Jones’ major criteria for diagnosing rheumatic fever

Introduction

This blog is meant for neurology tips and answers to questions asked during my DNB Neurology training.

Its personal, and not meant for teaching purposes. Accuracy and updates to existing information is not necessary.