Lewy body dementia when compared to Alzheimer's dementia

Which of the following is more commonly seen in patients with Lewy body dementia when compared to Alzheimer's dementia?

• A) Hallucinations
• B) Lip smacking
• C) Tremor
• D) Emotional lability
• E) Repetitive behavior

The answer is A.

Although difficult to know for sure, Lewy body dementia may be the second most common dementia after Alzheimer's disease.

Lewy bodies are hallmark lesions of degenerating neurons in Parkinson's disease "deposits of the protein alpha-synuclein inside nerve cells in the brain"  and occur in dementia with or without features of Parkinson's disease. In Lewy body dementia, Lewy bodies may predominate markedly or be intermixed with classic pathologic changes of Alzheimer's disease. Symptoms, signs, and course of Lewy body dementia resemble those of Alzheimer's disease, except hallucinations (mainly visual) are more common and patients appear to have an exquisite sensitivity to antipsychotic-induced extrapyramidal adverse effects.

How to distinguish Athetosis from Chorea ?

Sometimes is difficult to distinguish athetosis from chorea (hence the term choreoathetosis). Typically, however, athetoid movements are slower than choreiform movements.

Athetosis is an extrapyramidal sign characterized by slow, continuous, twisting, involuntary movements. Typically, these movements involve the face, neck, and distal extremities, such as the forearm, wrist and hand.
Facial grimaces, jaw and tongue movements, and occasional phonation are associated with neck movements. Athetosis worsens during stress and voluntary activity, may subside during relaxation, and disappears during sleep. It is commonly a lifelong affliction.

A: With athetosis, movements are typically slow, twisting, and writhing. They're associated with spasticity and most commonly involve the face, neck, and distal extremities. 

B: With chorea, movements are brief, rapid, jerky, and unpredictable. They can occur at rest or during normal movement. Typically, they involve the hands, lower arm, face, and head.

Positions of fingers in movements of athetosis.

Athetosis usually begins during childhood, resulting from hypoxia at birth, kernicterus, or a genetic disorder. In adults, athetosis usually results from a vascular or neoplastic lesion, a degenerative disease, drug toxicity, or hypoxia.

Grades of deep tendon reflexes

 Which of the following grades of deep tendon reflexes best describes a patient with transient
and intermittent clonus?

• a.Grade 0
• b.Grade 1+
• c.Grade 2+
• d.Grade 3+
• e.Grade 4+

The answer is (e).
Clonus is rapidly alternating involuntary contraction and relaxation of skeletal muscle. Using a reflex hammer deep tendon reflex (DTR) response is graded on a scale from 0 to 4+:

In a normal person, when a muscle tendon is tapped briskly, the muscle immediately contracts due to a two-neuron reflex arc involving the spinal or brainstem segment that innervates the muscle. The afferent neuron whose cell body lies in a dorsal root ganglion innervates the muscle or Golgi tendon organ associated with the muscles; the efferent neuron is an alpha motoneuron in the anterior horn of the cord.

Diagram shows pathway of the pupillary light reflex

This is a schematic diagram of the pupillary light reflex. The afferent limb originates in the retinal photoreceptors, which convert light energy to a neural signal. Pupillary information is conveyed from the eye to the brain by the melanopsin-expressing retinal ganglion cells, and their axons project to the dorsal midbrain, synapsing in the pretectal olivary nucleus. Each pretectal olivary nucleus distributes the afferent pupillary impulses to the ipsilateral and contralateral Edinger-Westphal subnucleus of the oculomotor nuclear complex.
The neurons of the Edinger-Westphal subnucleus initiate the efferent limb of the pupillary light reflex, that is, pupilloconstriction. Efferent pupillomotor impulses travel in the parasympathetic fibers of the oculomotor nerve, synapse in the ciliary ganglion of the orbit, and then pass via the short ciliary nerves to innervate the iris sphincter muscle.

Classic description of Normal-pressure hydrocephalus

A 75-year-old man is brought in to your office by his wife. She complains that he is not the same over the last 6 months. His memory is failing him, he has difficulty walking (especially when he initiates walking), and he is incontinent of urine. Which of the following is the most likely diagnosis based on his history?

• A) Alzheimer's disease
• B) Parkinson's disease
• C) Normal-pressure hydrocephalus
• D) Pick's disease
• E) Progressive supranuclear palsy

The answer is (C). 

Normal-pressure hydrocephalus (NPH) is a cause of dementia in the elderly. It may be caused by previous insult to the brain, usually as a result of a subarachnoid hemorrhage or diffuse meningitis that presumably results in scarring of the arachnoid villi over the brain convexities where cerebrospinal fluid (CSF) absorption usually occurs. However, elderly NPH patients seldom have a history of predisposing disease.
NPH classically consists of dementia, apraxia of gait, and incontinence, but many patients with these symptoms do not have NPH. Typically, motor weakness and staggering are absent, but initiation of gait is hesitant—described as a “slipping clutch” or “feet stuck to the floor” gait—and walking eventually occurs. NPH has also been associated with various psychiatric manifestations that are not categorical. NPH should be considered in the differential diagnosis of any new mental status changes in the elderly.

CT or MRI and a lumbar puncture are necessary for diagnosis. On CT or MRI, the ventricles are dilated. CSF pressure measured by a lumbar puncture is normal. A limited improvement after removing about 50 mL of CSF indicates a better prognosis with shunting. Radiographic or pressure measurements alone do not seem to predict response to shunting. Shunting CSF from the dilated ventricles sometimes results in clinical improvement, but the longer the disease has been present, the less likely shunting will be curative.

Picture of Cranial Nerve 12 (Hypoglossal Nerve XII) Dysfunction and Assessment

Hypoglossal nerve is a motor cranial nerve for the muscles of the tunge.So,If the right CN 12 is dysfunctional, the tongue will deviate to the right. This is because the normally functioning left half will dominate as it no longer has opposition from the right. Similarly, the tongue would have limited or absent ability to resist against pressure applied from outside the left cheek.

This photo shows Cranial Nerve 12 Dysfunction: Stroke has resulted in loss of function of left CN 12. When patient sticks tongue out, it therefore deviates to the left.

Assessment is performed as follows:

•Ask the patient to stick their tongue straight out of their mouth.
•If there is any suggestion of deviation to one side/weakness, direct them to push the tip of their
tongue into either cheek while you provide counter pressure from the outside.

About management of cauda equine syndrome

A 45-year-old man complains of decreased sensation in his buttocks and inability to achieve an erection. On examination he has decreased anal sphincter tone and decreased ankle reflexes bilaterally. the best next step in management?

• A.Bedrest and follow-up in 4 to 6 weeks
• B.Plain film X-ray of lumbosacral spine
• C.Sedimentation rate and complete blood count
• D.Immediate referral for surgical decompression

Answer is D. 

This individual has cauda equine syndrome, and requires immediate surgical decompression to avoid long-term nerve denervation and incontinence/lower extremity weakness. The decreased anal sphincter tone and decreased ankle reflexes indicate a peripheral neuropathy.

Bedrest with follow-up is indicated when no “red flag” symptoms and signs are present.

The plain film X-ray is often normal in patients with cauda equine syndrome.

Ramsay Hunt Syndrome(clinical)

CAUSES:
Classic Ramsay Hunt syndrome is ascribed to infection of the geniculate ganglion by herpesvirus 3 (varicella-zoster virus [VZV]).



HISTORY:
*Patients usually present with paroxysmal pain deep within the ear. The pain often radiates outward into the pinna of the ear and may be associated with a more constant, diffuse, and dull background pain.

*The onset of pain usually precedes the rash by several hours and even days.

*Classic Ramsay Hunt syndrome can be associated with the following:
-Vesicular rash of the ear or mouth (as many as 83% of cases),The rash might precede the onset of facial paresis/palsy.
-Ipsilateral lower motor neuron facial paresis/palsy (CN VII)
-Vertigo and ipsilateral hearing loss (CN VII)
-Tinnitus,Otalgia,Headaches,DysarthriaGait,ataxia.
-Fever,Cervical adenopathy.

*Facial weakness usually reaches maximum severity by one week after the onset of symptoms.

*Other cranial neuropathies might be present and may involve cranial nerves (CNs) VIII, IX, X, V, and VI.

*Ipsilateral hearing loss has been reported in as many as 50% of cases.

*Blisters of the skin of the ear canal, auricle, or both may become secondarily infected, causing cellulitis.


EXAMINATION:

.The primary physical findings in classic Ramsay Hunt syndrome include peripheral facial nerve paresis with associated rash or herpetic blisters in the distribution of the nervus intermedius.
.The location of the accompanying rash varies from patient to patient, as does the area innervated by the nervus intermedius. It may include the following:
1.Anterior two thirds of the tongue
2.Soft palate
3.External auditory canal
4.Pinna
.The patient may have associated ipsilateral hearing loss and balance problems.
.A thorough physical examination must be performed, including neuro-otologic and audiometric assessment.

Brain herniation Sites

Brain herniation refers to displacement of a portion of the brain from its normal position through openings in the inelastic dura secondary to focal or diffuse intracranial pressure. Recognition of the CT signs of brain herniation on the emergent head CT is critical to proper patient management. The types of brain herniations are schematically illustrated...":a) " Subfalcial (cingulate) herniation ; b) uncal herniation ; c) downward (central, transtentorial) herniation ; d) external herniation ; e) tonsillar herniation.Types a, b, & e are usually caused by focal, ipsilateral space occupying lesions, ie., tumor or axial or extra-axial hemorrhage."

"Schematic representation of various herniation pathways (1) subfalcine, (2) uncal (Transtentorial), (3) cerebellar, and (4) transcalvarium)

Cranial Nerve 9 Dysfunction Pic

Cranial Nerve 9 Dysfunction: Patient has suffered stroke, causing loss of function of left CN 9 (Glossopharyngeal nerve). As a result, uvula is pulled towards the normally functioning (ie right) side.

Researchers on a Completely fearless woman, Do you belive !!

It has been known since the 1930s that when a certain part of monkeys’ brains were removed, the animals became fearless. Now similar effects are being seen in humans according to a study in the journal Current Biology. Justin Feinstein, the study’s lead author and a clinical neuropsychologist at the University of Iowa said, “There’s not very many humans with this sort of brain damage… Luckily for us, we had access to a patient, SM, and we studied her different fear behaviours and we read her personal diaries.”

SM due to a rare condition called lipoid proteinosis does not have her amygdala. The amygdala is an almond-shaped structure that studies have shown plays a role in processing fear and other emotions, though experts say its exact role is unclear. Her condition is termed Urbach-Wiethe disease. This has made her fearless to all normally fear evoking stimuli like snakes, spiders etc. This has also made her put her life at risk a few times. Now the team is trying to coach the patient to behave in a more cautious manner.


Dr. Jon Shaw, professor of psychiatry at the University of Miami School of Medicine said, “It’s very striking that she has only a rational response, not a physiological one… The body is not prepared for a physiological response because the amygdala has been taken out of the loop.” Ruben Gur, professor in the Departments of Psychiatry, Radiology & Neurology at the University of Pennsylvania School of Medicine in Philadelphia was said, “If you look at past neuroimaging studies, there is clear evidence that the amygdala is extremely sensitive to fearful stimuli.”

Researchers believe that her condition may offer clues for therapy of people with excessive fear like war veterans. This condition is known as post-traumatic stress disorder or PTSD. Feinstein said, “We may be able to dampen the effects of the amygdale… We can do that through psychotherapy and possibly through medication.”

Not all are convinced. Elizabeth Phelps, professor of psychology and neural science at New York University said, “You have to interpret case studies with caution since there’s been contradictory work done.” She pointed out that there is one study that found an unusual emotional response in monkeys whose brains were experimentally damaged, but only during specific stages of development. She explained there was another patient HM who had part of his brain, including the amygdala, removed to treat severe epilepsy. His main problem, according to experts, was his memory loss.

The Confusion Assessment Model (CAM)

CLICK for enlarment

Diplopia in a Patient with HIV Infection

A 25-year-old man with human immunodeficiency virus (HIV) infection who was receiving highly active antiretroviral therapy presented with a 1-week history of diplopia and headache. The CD4 count was 218 cells per cubic millimeter, and the viral load was 50,000 copies per milliliter.
The neurologic examination revealed an inability to abduct the right eye with horizontal gaze, a finding that was consistent with an isolated right abducens nerve palsy (rightward gaze, Panel A; leftward gaze, Panel B). The examination of other cranial nerves was normal. The remainder of the motor and sensory examination was within normal limits.
The patient reported having had low back pain and constipation for the previous week. There was no history of bowel or bladder incontinence. A gadolinium-enhanced magnetic resonance image of the brain showed a minimally enhancing mass filling and expanding the right cavernous sinus (Panel C, arrow). Lumbar-spine imaging showed a mass with similar radiographic characteristics involving the ventral epidural compartment.
Biopsy of the spinal lesion revealed diffuse large-B-cell lymphoma. A chemotherapeutic regimen of cyclophosphamide, doxorubicin, vincristine, and prednisolone, along with the monoclonal antibody rituximab (R-CHOP), was started. Progressive leg weakness from spinal involvement developed, and the patient had a poor response to corticosteroids and radiation therapy and died 3 months later.

Diagnostic Algorithm for Evaluation of Acute Myelopathies and Myelitis.

Click on photo for enlargment

How is Neuroblastoma Clinically Presentating ?

Neuroblastoma is a childhood cancer that is diagnosed at a median age of about 17 months. Tumors can arise anywhere along the sympathetic nervous system, with the majority occurring in the adrenal medulla. Primary tumors in the neck or upper chest can cause Horner's syndrome (ptosis, miosis, and anhidrosis).
Tumors along the spinal column can expand through the intraforaminal spaces and cause cord compression, with resulting paralysis. Although many lower-stage neuroblastomas are encapsulated and can be surgically excised with little chance of complications, higher-stage tumors often infiltrate local organ structures, surround critical nerves and vessels such as the celiac axis, and are largely unresectable at the time of diagnosis.

Neuroblastomas typically metastasize to regional lymph nodes and to the bone marrow by means of the hematopoietic system. Tumor cells metastatic to marrow can infiltrate cortical bone. Neuroblastomas also can metastasize to the liver, most notably in patients with stage 4S tumors, in whom involvement can be extensive; however, transient and complete regression often occurs with no intervention other than supportive care.

Minimally Invasive Surgery Corrects Urinary Incontinence

A DMC patient with urinary stress incontinence regains control with less-invasive surgery at DMC Sinai-Grace, performed by DMC Ob/Gyn specialist Dr. Korial Atty. ~ Detroit Medical Center.

6th Cranial Nerve Palsy

Left 6th Cranial Nerve Palsy: Left eye unable to move laterally beyond midline. In this case, nerve
dysfunction was caused by diabetes-induced microvascular disease.


Sixth Left Cranial Nerve Palsy: Left eye cannot move laterally beyond midline. In this case, caused
by metastatic squamous cell cancer of the head and neck.

Blepharospasm

Blepharospasm is an abnormal tic, spasm, or twitch of the eyelid. It is sometimes referred to as Benign Essential Blepharospasm. Focal dystonia is another phrase for this condition, which involves a involuntary muscle contraction around the eyes. The cause can be fatigue, irritant or caffeine. The symptoms can last for a few days, but commonly disappear without treatment. Severe cases can be chronic.

Besides the tic, symptoms of blepharospasm are eye dryness, excessive blinking, twitching, sensitivity to the sun and bright lights. Blepharospasm occurs spontaneously with no indicators. Patients who experience blepharospasm usually have a history of dry eyes and photo sensitivity. Other syndromes such as Meige’s syndrome have been associated with blepharospasm. Drugs used to treat Parkinson’s disease have also been known to cause blepharospasm. Rarely, blepharospasms can be caused by concussions.

causes of coma, mnemonic

There are several medical causes of coma. One of the easiest ways to remember these causes is to use the mnemonic AEIOU TIPS:

* A - alcohol (overdose / withdraw / Wernicke's encephalopathy)
* E - epilepsy or exposure (heat stroke, hypothermia) or electrolytes
* I - insulin (diabetic emergency - high/low sugar)
* O - overdose or oxygen deficiency
* U - uremia (metabolic) or underdose
* T - trauma (head injury) or toxicology
* I - infection / inflammation
* P - psychosis or poisoning
* S - stroke / SAH / shock / syncope

Babinski Response

Babinski Response: Note upgoing great toe upon stimulation of lateral foot in patient with
upper motor neuron lesion.