Neurology Breakthroughs

09/11/2022

25/10/2022

08/09/2022

MRI of a 45-year old man. Nonspecific small white matter lesions, most likely of
vascular origin
2. Multiple sclerosis
3. ADEM
4. The picture is characteristic for PC, but without symptoms
it is, at best, RIS
5. Leukoencephalomyelopathy
6. Toxoplasmosis
7. Alcoholic encephalopathy

28/07/2020

.

10/10/2017

Learn at a look .

17/05/2017

(DLB),

A ,
degenerative .
Makes10 to 20% cases of dementia.
Disease of late middle age and old age.
Slightly more common in men than in .

Symptoms and signs of DLB probably result, in part, from disruption of bidirectional information flow from the striatum to the , especially the frontal lobe. In DLB, nonpyramidal cells in layers V and VI of the neocortex may contain LBs. Their function in neocortical information processing and in relaying data to subcortical regions probably is impaired.

Single-photon emission computed tomography (SPECT) scanning studies in 145 DLB patients revealed the following.

Visual hallucinations - Were related to hypoperfusion
of the parietal and occipital association cortices
Misidentifications - Were related to hypoperfusion of
the limbic-paralimbic structures
Delusions - Were related to hyperperfusion of the
frontal cortices
The etiology of fluctuations in
cognitive function, which characterize DLB, is unknown.

The relationship of DLB and Parkinson disease is an area of considerable controversy, particularly because dementia frequently occurs in Parkinson disease and Parkinsonism in DLB. Parkinson disease and DLB have demonstrated LBs in the substantia nigra and possibly in the locus ceruleus, dorsal raphe, substantia innominata, and dorsal motor nucleus of cranial nerve X (CNX, the vagus nerve). LBs are found in the neocortex of many patients with idiopathic Parkinson disease and in all patients with DLB.

.

The following clinical features help to distinguish DLB from Alzheimer disease:

Fluctuations in cognitive
Varying levels of alertness and attention,
Daytime sleep problem,
Staring into space for long periods,

Episodes of disorganized speech

Visual hallucinations

Parkinsonian motor features

Extrapyramidal features may occur late in the course of Alzheimer disease, they appear relatively early in DLB.

In addition, whereas patients with Alzheimer disease virtually always have anterograde memory loss while less prominent in DLB.

Executive function deficits and visuospatial impairment may be more prominent in persons with DLB than in those with Alzheimer disease.

Delusions

Unexplained syncope

Rapid eye movement sleep disorder

Neuroleptic sensitivity



• Alzheimer Disease Imaging
• Cortical Basal Ganglionic Degeneration
• Frontotemporal Dementia
• Hydrocephalus
• Lacunar Syndrome
• Parkinson Disease
• Parkinson-Plus Syndromes
• Prion-Related Diseases
• Progressive Supranuclear Palsy



CBC, ELECTROLYTES, THYROID FUNCTION TEST,
VIT-B12, SYPHILLIS , HIV SCREENING, CSF USUALLY
NOT REQUIRED HOWEVER AMYLOID PROTEIN LEVEL
DECREASES IN BOTH DLB AND ALZHEIMER.

MRI FINDINGS

Features reported include.

Generalised decrease in cerebral volume most marked in
frontal lobes and parietotemporal regions

Enlargement of the lateral ventricles

Relatively focal atrophy of the
midbrain, hypothalamus and substantia innominata

Perhaps, more importantly, the hippocampi remain normal in size, helping to distinguish Lewy body disease from Alzheimer disease.

16/05/2017

(MBD)

is a rare condition characterized by demyelination of the corpus callosum. It is seen most often in patients with chronic alcoholism.

Most patients diagnosed with Marchiafava-Bignami disease (MBD) have a history of alcoholism and poor nutrition.


The disease is attributed to a deficiency of all eight types of vitamin B group and results in necrosis and demyelination of the corpus callosum.

.
The onset and the range of clinical symptoms vary, i.e. sudden onset (stupor or coma, and some present with seizures) acute, subacute, or chronic onset (dementia and/or gait problems, spasticity, hemiparesis, aphasia, and apraxia have been described)

.
The corpus callosum may also be affected in other diseases, such as ischemic stroke, contusion, lymphoma, and multiple sclerosis. However, Marchiafava-Bignami disease (MBD) is distinguished by callosal lesions that are usually and located in the of the corpus callosum.

Other conditions to consider in the differential diagnosis o MBD include the following:





;
Serum electrolyte, RBS, CBC), toxicology screening.
A spinal tap often is needed and usually is performed after findings on a brain CT scan have excluded an intracranial mass or hemorrhage.
No electroencephalographic findings are specific for or characteristic of MBD.


Marchiafava-Bignami disease typically begins in the body of the corpus callosum and later involves the genu and then splenium. It classically involves the central layers with relative sparing of the dorsal and ventral extremes (which may be seen as a sign on sagittal MRI imaging).
MRI
T1: may show hypointense foci in corpus callosum in the acute phase
T2: acute phase: hyperintensities in the corpus callosum in the correct clinical setting may aid in the diagnosis subacute phase: may show hypointense focal lesions.
CT
may typically show hypoattenuating regions in the corpus callosum

.
Administration of vitamin B complex results in improvement in many patients, although some do not recover and may die of the disease.

15/05/2017

A sporadic neurodegenerative tauopathy.
Both cortical (pyramidal and nonpyramidal) and subcortical abnormalities are seen.
Both neurons and glial cells are involved.
Cortical loss, predominantly affecting motor and premotor regions, may distinguish this disorderfrom progressive supranuclear palsy.
More common in women.
Typically, presents between the ages of 50 and 80 years.

CLINICAL PRESENTATION
Insidious onset and progressive course

Cortical dysfunction including at least 1 of the following:
Focal or asymmetric ideomotor apraxia,
("My hand/leg has a mind of its own.")
Cortical (parietal) sensory loss
Visual or tactile spatial neglect
Constructional apraxia
Focal or asymmetric myoclonus
Apraxia of speech or nonfluent aphasia

Extrapyramidal dysfunction,
including at least 1 of the following:
Focal or asymmetric appendicular rigidity
(unresponsive to levodopa)
Focal or asymmetric appendicular dystonia

Unusual presentations, for example, primary
progressive aphasia and progressive buccofacial apraxia,
The presence of prominent delusions or hallucinations
(not related to levodopa) suggests
the patient does not have CBGD;
they are more characteristic of diffuse Lewy body disease.

DIFFERENTIAL DIAGNOSIS.

Alzheimer Disease Imaging, Anterior Circulation Stroke,
Apraxia and Related Syndromes, CNS Whipple Disease,
Cardioembolic Stroke, CML, Creutzfeldt-Jakob Disease
Dementia With Lewy Bodies, Epilepsia Partialis Continua
Frontotemporal dementia, Glioblastoma Multiforme
Huntington Disease, Hydrocephalus,
Marchiafava-Bignami Disease, Multiple System Atrophy
Neuroacanthocytosis, Neuroacanthocytosis Syndromes
Neurosyphilis, Olivopontocerebellar Atrophy
Parkinson Disease, Pick Disease,
Progressive Supranuclear Palsy,
Striatonigral Degeneration, Subdural Hematoma
Vitamin B-12 Associated Neurological Diseases,
Vitamin E-Associated Neurological Diseases,
Wilson Disease,

WORK UP.

Ceruloplasmin, B-12 level, Rapid plasma reagin (RPR)
or (VDRL), TSH,
and consider thyroid autoantibody screening,
Electrolytes, CBC with differential and platelets
(ANA), (ESR), liver function tests, and ammonia level
Manual smear for acanthocytes
or genetic testing for Huntington disease
if patient has chorea.

Lumbar puncture (LP) may be done to examine
cerebrospinal fluid (CSF) for cells and elevated protein;
the polymerase chain reaction (PCR) test for the
organism Tropheryma whippleii should also be done

MRI OF BRAIN

This study is particularly helpful in evaluating the size
and appearance of the midbrain if any disturbance of
eye movements is noted
and progressive supranuclear palsy is being considered.
Midbrain size should be relatively normal in
cortical basal ganglionic degeneration (CBGD).

Cortical atrophy usually occurs, and this can be more
localized to the central sulci/supplementary motor area
(SMA) and superior frontal gyrus than to the
temporal/parietal cortex (the latter pattern is seen
in dementia of the Alzheimer type).

Abnormal signal in basal ganglia can occur with
metal deposition in Wilson disease or
Hallervorden-Spatz disease.

Functional brain imaging is not generally needed,
but it can be helpful in some patients to document that
cognitive changes are neurological and not psychological
in origin.

13/05/2017

Researchers believe they have figured out what it is about some static images that trigger seizures in those with photosensitive epilepsy.

13/05/2017

Researchers have identified a potential neuroimaging predictor for dementia that highlights brain structural changes that may occur years before people even notice memory problems.

10/05/2017

Aim of this page is to update and share knowledge.

09/05/2017

midbrain atrophy

midbrain to pons area ratio (figure 1): reduced area ratio on the midline sagittal plane to approximately 0.12 (normal ~ 0.24) 4. Most accurate imaging feature which also helps to distinguish it from MSA-P (shows pontine and midbrain atrophy)

(figure 2):
reduction of anteroposterior midline midbrain diameter, at the level of the superior colliculi on axial imaging (from interpeduncular fossa, to the intercollicular groove:

09/05/2017

(PSP)
A neurodegenerative disease
usually develops after the sixth decade of life. But can present after 40yrs.

Slow onset with
nonspecific symptoms like
fatigue, headache, arthralgias, dizziness, then personality changes, memory problems, un-explained falls and pseudobulber symptoms initially noted by family members.

of are..

First vertical gaze is affected



, , and disturbances that cause imbalance Frequent falls/impaired postural reflexes.

Slow and square wave jerks on ocular examination (early signs)
(classic gaze palsy in PSP)
typically involved before upgaze
Improvement in supranuclear vertical gaze limitation after extravolitional pathway activation with the vestibular ocular reflex (VOR) or the Bell phenomenon
Nearly continuous square wave jerks commonly observed with fixation
eye movements (may cause diplopia)
, eyelid opening or closing , , or lid lag
in advanced PSP

Poor postural reflexes, axial rigidity greater than appendicular rigidity, and dysarthria (monotone with slight hypophonic quality)
Absence of cogwheeling or tremor
Widely based and unstable gait
Bradykinesia with masked facies and a startled expression
may be present

Participants in a National Institute of Neurological Disorders and Stroke (NINDS)/Society for PSP conference have formulated and validated clinical research criteria for the diagnosis of PSP.

PSP are as follows:

with onset at age 40 years or older
Either palsy or both slowing of vertical saccades and prominent with falls in the first year of onset
of other diseases that can explain the clinical features

PSP

supranuclear palsy with prominent postural instability, falls in the first year of onset, and other features of possible PSP, as follows:
, especially retrocollis
Poor or absent response of parkinsonism to therapy
Early dysphagia and dysarthria
Early with at least 2 of the following: apathy, abstract thought impairment, decreased verbal fluency, imitation behavior, or frontal release signs

PSP are as follows:
History of probable or possible PSP
Histopathologic evidence that is typical of the disease

The workup in patients with suspected PSP is directed principally at eliminating other diagnoses (eg, PCR to eliminate possible Whipple disease).

27/04/2017

https://youtu.be/kXMydlXQYpY

Neurology - Topic 13 - Parkinson's disease female patient

27/04/2017

https://youtu.be/V6cxZa6gy6g

We have just tried to imitate a patient here.Your feedbacks are most welcome -- Dr.Raju Suresh Kumar (email:raju6712in@gmail.com)

27/04/2017

-Edson Zerati, MD -December, 11, 2009 http://edzerati.vilabol.uol.com.br

27/04/2017

-Edson Zerati, MD -December, 11, 2009 http://www.zerati.com

26/04/2017

A progressive, idiopathic, degenerative process beginning in adulthood.

Having..variable , signs, and dysfunction.

It includes..
(OPCA)
Predominant Cerebellar symptoms
(SND)
Predominant Parkinson's like symptoms

While the term shy drager syndrome is omitted because autonomic symptoms occurs in all types i.e. OPCA and SND.

The Movement Disorders Society Scientific Issues Committee Report (MDSSICR) revised the diagnostic criteria for MSA in 2003.
Divides MSA into two categories

-P predominant Parkinsonian symptoms. i.e. OPCA

-C predominant cerebellar symptoms. i.e. SND

The MDSSICR recognizes 4 clinical domains in MSA:






T2: hyperintensities typically present in the pontocerebellar tracts
pons: (MSA-C) middle cerebellar peduncles
cerebellum
putaminal findings in MSA-P 5:
reduced volume
reduced GRE and T2 signal relative to globus pallidus
reduced GRE and T2 signal relative to red nucleus
abnormally high T2 linear rim surrounding the putamen (" ")

MSA-C
disproportionate atrophy of the cerebellum and brainstem (especially olivary nuclei and middle cerebellar peduncle.


Median survival is 6.2 years, with a range of 0.5-24 years.
Patients with the cerebellar subtype of MSA survived longer.
Response to drug therapy is poor.
The ataxia is particularly resistant to therapy.
Levodopa replacement is the mainstay of therapy for the parkinsonian features Responses tend to be less clear-cut and may be transient.
Dopamine agonists are not effective and are more likely to cause hallucinations and psychosis. Blepharospasm and limb dystonia can be reduced with injections.

Autonomic dysfunction, especially orthostatic hypotension, is a prominent feature of the disease.
Initial treatment includes reduction of antihypertensive agents, increased salt and water intake, abdominal binders, and use of elastic stockings. and can be helpful when other measures fail. Other medications for treatment of orthostatic hypotension include and pyridostigmine.


Complications of multiple system atrophy include the following:
and glottic airway compromise requiring continuous positive airway pressure support or tracheostomy
secondary to dysphagia and vocal fold paresis
, often occurring at night and associated with sleep-disordered breathing

Patients with symptomatic postural hypotension should be educated with regard to the following:
or environments that produce excessive vagal stimulation or vasodilation (eg, extreme heat, overeating, alcohol, straining at stool) should be avoided
and carefully from seated or recumbent positions
or as soon as symptoms appear Consider using , elevating the head of the bed, and increasing sodium intake
Be_aware that there is a of fall with associated ; always seek medical attention for any but the most minor of falls.

First two images showing hot cross bun sign while in other images see putaminal ring sign.