This patient, a 65-year-old male, presented with 6 months of difficulty walking and 4 months of forgetfulness and hallucinations. Examination found he was semiconscious with rigidity and myoclonic jerks. Imaging and tests were consistent with a prion disease like Creutzfeldt-Jakob disease (CJD). A lumbar puncture found elevated proteins. He was diagnosed with a rapidly progressive neurodegenerative condition likely CJD.
Case Presentation of a patient presented with polyradiculoneuropathy and bilateral bulbar palsy. Detailed evaluation finally pinpoints to Guillian barre syndrome.
Case Presentation of a patient presented with polyradiculoneuropathy and bilateral bulbar palsy. Detailed evaluation finally pinpoints to Guillian barre syndrome.
Part 1: Neurological history and physicaltschmitt2002
This is a power point presentation that a student completed for an independent study in his BSN program. It covers the basics of the nurse driven neurological history and physical.
SSPE, dr. amit vatkar, pediatric neurologistDr Amit Vatkar
Subacute sclerosing pan encephalitis (SSPE) also known as Dawson Disease, Dawson encephalitis, and measles encephalitis is a rare and chronic form of progressive brain inflammation caused by a persistent infection with measles virus.
In this presentaion i will a case a sspe and give u some information regarding daignosis and treatment
Dr. john millichap kcnq2 Cure summit professional track learn more at kcnq2cu...scottyandjim
Dr. John Millichap speaking at 2014 Denver KCNQ2 Cure summit professionals track at Children's Hospital of Colorado. More information at www.kcnq2cure.org
a presentation on autoimmune encephalitis, paraneoplastic syndrome. their types and various imaging and lab finding
their differential diagnosis
acute and long term management plans
Dr. sarah weckhuysen kcnq2 Cure summit professional track - Lean more at kcn...scottyandjim
Dr. Sarah Weckhuysen speaking at 2014 Denver KCNQ2 Cure summit professionals track at Children's Hospital of Colorado. More information at www.kcnq2summit.org
263778731218 Abortion Clinic /Pills In Harare ,sisternakatoto
263778731218 Abortion Clinic /Pills In Harare ,ABORTION WOMEN’S CLINIC +27730423979 IN women clinic we believe that every woman should be able to make choices in her pregnancy. Our job is to provide compassionate care, safety,affordable and confidential services. That’s why we have won the trust from all generations of women all over the world. we use non surgical method(Abortion pills) to terminate…Dr.LISA +27730423979women Clinic is committed to providing the highest quality of obstetrical and gynecological care to women of all ages. Our dedicated staff aim to treat each patient and her health concerns with compassion and respect.Our dedicated group ABORTION WOMEN’S CLINIC +27730423979 IN women clinic we believe that every woman should be able to make choices in her pregnancy. Our job is to provide compassionate care, safety,affordable and confidential services. That’s why we have won the trust from all generations of women all over the world. we use non surgical method(Abortion pills) to terminate…Dr.LISA +27730423979women Clinic is committed to providing the highest quality of obstetrical and gynecological care to women of all ages. Our dedicated staff aim to treat each patient and her health concerns with compassion and respect.Our dedicated group of receptionists, nurses, and physicians have worked together as a teamof receptionists, nurses, and physicians have worked together as a team wwww.lisywomensclinic.co.za/
Couples presenting to the infertility clinic- Do they really have infertility...Sujoy Dasgupta
Dr Sujoy Dasgupta presented the study on "Couples presenting to the infertility clinic- Do they really have infertility? – The unexplored stories of non-consummation" in the 13th Congress of the Asia Pacific Initiative on Reproduction (ASPIRE 2024) at Manila on 24 May, 2024.
Title: Sense of Taste
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the structure and function of taste buds.
Describe the relationship between the taste threshold and taste index of common substances.
Explain the chemical basis and signal transduction of taste perception for each type of primary taste sensation.
Recognize different abnormalities of taste perception and their causes.
Key Topics:
Significance of Taste Sensation:
Differentiation between pleasant and harmful food
Influence on behavior
Selection of food based on metabolic needs
Receptors of Taste:
Taste buds on the tongue
Influence of sense of smell, texture of food, and pain stimulation (e.g., by pepper)
Primary and Secondary Taste Sensations:
Primary taste sensations: Sweet, Sour, Salty, Bitter, Umami
Chemical basis and signal transduction mechanisms for each taste
Taste Threshold and Index:
Taste threshold values for Sweet (sucrose), Salty (NaCl), Sour (HCl), and Bitter (Quinine)
Taste index relationship: Inversely proportional to taste threshold
Taste Blindness:
Inability to taste certain substances, particularly thiourea compounds
Example: Phenylthiocarbamide
Structure and Function of Taste Buds:
Composition: Epithelial cells, Sustentacular/Supporting cells, Taste cells, Basal cells
Features: Taste pores, Taste hairs/microvilli, and Taste nerve fibers
Location of Taste Buds:
Found in papillae of the tongue (Fungiform, Circumvallate, Foliate)
Also present on the palate, tonsillar pillars, epiglottis, and proximal esophagus
Mechanism of Taste Stimulation:
Interaction of taste substances with receptors on microvilli
Signal transduction pathways for Umami, Sweet, Bitter, Sour, and Salty tastes
Taste Sensitivity and Adaptation:
Decrease in sensitivity with age
Rapid adaptation of taste sensation
Role of Saliva in Taste:
Dissolution of tastants to reach receptors
Washing away the stimulus
Taste Preferences and Aversions:
Mechanisms behind taste preference and aversion
Influence of receptors and neural pathways
Impact of Sensory Nerve Damage:
Degeneration of taste buds if the sensory nerve fiber is cut
Abnormalities of Taste Detection:
Conditions: Ageusia, Hypogeusia, Dysgeusia (parageusia)
Causes: Nerve damage, neurological disorders, infections, poor oral hygiene, adverse drug effects, deficiencies, aging, tobacco use, altered neurotransmitter levels
Neurotransmitters and Taste Threshold:
Effects of serotonin (5-HT) and norepinephrine (NE) on taste sensitivity
Supertasters:
25% of the population with heightened sensitivity to taste, especially bitterness
Increased number of fungiform papillae
Report Back from SGO 2024: What’s the Latest in Cervical Cancer?bkling
Are you curious about what’s new in cervical cancer research or unsure what the findings mean? Join Dr. Emily Ko, a gynecologic oncologist at Penn Medicine, to learn about the latest updates from the Society of Gynecologic Oncology (SGO) 2024 Annual Meeting on Women’s Cancer. Dr. Ko will discuss what the research presented at the conference means for you and answer your questions about the new developments.
Prix Galien International 2024 Forum ProgramLevi Shapiro
June 20, 2024, Prix Galien International and Jerusalem Ethics Forum in ROME. Detailed agenda including panels:
- ADVANCES IN CARDIOLOGY: A NEW PARADIGM IS COMING
- WOMEN’S HEALTH: FERTILITY PRESERVATION
- WHAT’S NEW IN THE TREATMENT OF INFECTIOUS,
ONCOLOGICAL AND INFLAMMATORY SKIN DISEASES?
- ARTIFICIAL INTELLIGENCE AND ETHICS
- GENE THERAPY
- BEYOND BORDERS: GLOBAL INITIATIVES FOR DEMOCRATIZING LIFE SCIENCE TECHNOLOGIES AND PROMOTING ACCESS TO HEALTHCARE
- ETHICAL CHALLENGES IN LIFE SCIENCES
- Prix Galien International Awards Ceremony
NVBDCP.pptx Nation vector borne disease control programSapna Thakur
NVBDCP was launched in 2003-2004 . Vector-Borne Disease: Disease that results from an infection transmitted to humans and other animals by blood-feeding arthropods, such as mosquitoes, ticks, and fleas. Examples of vector-borne diseases include Dengue fever, West Nile Virus, Lyme disease, and malaria.
Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
New Directions in Targeted Therapeutic Approaches for Older Adults With Mantl...i3 Health
i3 Health is pleased to make the speaker slides from this activity available for use as a non-accredited self-study or teaching resource.
This slide deck presented by Dr. Kami Maddocks, Professor-Clinical in the Division of Hematology and
Associate Division Director for Ambulatory Operations
The Ohio State University Comprehensive Cancer Center, will provide insight into new directions in targeted therapeutic approaches for older adults with mantle cell lymphoma.
STATEMENT OF NEED
Mantle cell lymphoma (MCL) is a rare, aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for 5% to 7% of all lymphomas. Its prognosis ranges from indolent disease that does not require treatment for years to very aggressive disease, which is associated with poor survival (Silkenstedt et al, 2021). Typically, MCL is diagnosed at advanced stage and in older patients who cannot tolerate intensive therapy (NCCN, 2022). Although recent advances have slightly increased remission rates, recurrence and relapse remain very common, leading to a median overall survival between 3 and 6 years (LLS, 2021). Though there are several effective options, progress is still needed towards establishing an accepted frontline approach for MCL (Castellino et al, 2022). Treatment selection and management of MCL are complicated by the heterogeneity of prognosis, advanced age and comorbidities of patients, and lack of an established standard approach for treatment, making it vital that clinicians be familiar with the latest research and advances in this area. In this activity chaired by Michael Wang, MD, Professor in the Department of Lymphoma & Myeloma at MD Anderson Cancer Center, expert faculty will discuss prognostic factors informing treatment, the promising results of recent trials in new therapeutic approaches, and the implications of treatment resistance in therapeutic selection for MCL.
Target Audience
Hematology/oncology fellows, attending faculty, and other health care professionals involved in the treatment of patients with mantle cell lymphoma (MCL).
Learning Objectives
1.) Identify clinical and biological prognostic factors that can guide treatment decision making for older adults with MCL
2.) Evaluate emerging data on targeted therapeutic approaches for treatment-naive and relapsed/refractory MCL and their applicability to older adults
3.) Assess mechanisms of resistance to targeted therapies for MCL and their implications for treatment selection
Flu Vaccine Alert in Bangalore Karnatakaaddon Scans
As flu season approaches, health officials in Bangalore, Karnataka, are urging residents to get their flu vaccinations. The seasonal flu, while common, can lead to severe health complications, particularly for vulnerable populations such as young children, the elderly, and those with underlying health conditions.
Dr. Vidisha Kumari, a leading epidemiologist in Bangalore, emphasizes the importance of getting vaccinated. "The flu vaccine is our best defense against the influenza virus. It not only protects individuals but also helps prevent the spread of the virus in our communities," he says.
This year, the flu season is expected to coincide with a potential increase in other respiratory illnesses. The Karnataka Health Department has launched an awareness campaign highlighting the significance of flu vaccinations. They have set up multiple vaccination centers across Bangalore, making it convenient for residents to receive their shots.
To encourage widespread vaccination, the government is also collaborating with local schools, workplaces, and community centers to facilitate vaccination drives. Special attention is being given to ensuring that the vaccine is accessible to all, including marginalized communities who may have limited access to healthcare.
Residents are reminded that the flu vaccine is safe and effective. Common side effects are mild and may include soreness at the injection site, mild fever, or muscle aches. These side effects are generally short-lived and far less severe than the flu itself.
Healthcare providers are also stressing the importance of continuing COVID-19 precautions. Wearing masks, practicing good hand hygiene, and maintaining social distancing are still crucial, especially in crowded places.
Protect yourself and your loved ones by getting vaccinated. Together, we can help keep Bangalore healthy and safe this flu season. For more information on vaccination centers and schedules, residents can visit the Karnataka Health Department’s official website or follow their social media pages.
Stay informed, stay safe, and get your flu shot today!
ARTIFICIAL INTELLIGENCE IN HEALTHCARE.pdfAnujkumaranit
Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, especially computer systems. It encompasses tasks such as learning, reasoning, problem-solving, perception, and language understanding. AI technologies are revolutionizing various fields, from healthcare to finance, by enabling machines to perform tasks that typically require human intelligence.
Recomendações da OMS sobre cuidados maternos e neonatais para uma experiência pós-natal positiva.
Em consonância com os ODS – Objetivos do Desenvolvimento Sustentável e a Estratégia Global para a Saúde das Mulheres, Crianças e Adolescentes, e aplicando uma abordagem baseada nos direitos humanos, os esforços de cuidados pós-natais devem expandir-se para além da cobertura e da simples sobrevivência, de modo a incluir cuidados de qualidade.
Estas diretrizes visam melhorar a qualidade dos cuidados pós-natais essenciais e de rotina prestados às mulheres e aos recém-nascidos, com o objetivo final de melhorar a saúde e o bem-estar materno e neonatal.
Uma “experiência pós-natal positiva” é um resultado importante para todas as mulheres que dão à luz e para os seus recém-nascidos, estabelecendo as bases para a melhoria da saúde e do bem-estar a curto e longo prazo. Uma experiência pós-natal positiva é definida como aquela em que as mulheres, pessoas que gestam, os recém-nascidos, os casais, os pais, os cuidadores e as famílias recebem informação consistente, garantia e apoio de profissionais de saúde motivados; e onde um sistema de saúde flexível e com recursos reconheça as necessidades das mulheres e dos bebês e respeite o seu contexto cultural.
Estas diretrizes consolidadas apresentam algumas recomendações novas e já bem fundamentadas sobre cuidados pós-natais de rotina para mulheres e neonatos que recebem cuidados no pós-parto em unidades de saúde ou na comunidade, independentemente dos recursos disponíveis.
É fornecido um conjunto abrangente de recomendações para cuidados durante o período puerperal, com ênfase nos cuidados essenciais que todas as mulheres e recém-nascidos devem receber, e com a devida atenção à qualidade dos cuidados; isto é, a entrega e a experiência do cuidado recebido. Estas diretrizes atualizam e ampliam as recomendações da OMS de 2014 sobre cuidados pós-natais da mãe e do recém-nascido e complementam as atuais diretrizes da OMS sobre a gestão de complicações pós-natais.
O estabelecimento da amamentação e o manejo das principais intercorrências é contemplada.
Recomendamos muito.
Vamos discutir essas recomendações no nosso curso de pós-graduação em Aleitamento no Instituto Ciclos.
Esta publicação só está disponível em inglês até o momento.
Prof. Marcus Renato de Carvalho
www.agostodourado.com
2. HISTORY OF PRESENT ILLNESS
• According to family members this 65 y/o male patient developed difficulty in walking for the
past 6 months.
• Patient was apparently normal 6 months back when he started developing gradual difficulty
in walking, in form of in coordination so that he used to walk holding on to walls or other
support available .
3. HISTORY OF PRESENT ILLNESS
• They were also complaining forgetfulness, hallucinations for past 4 months.
• Gradually family Members noticed that patient was becoming forgetful over time, and he used
to forget familiar faces, names, topic he discussed half an hour back
4. HISTORY OF PRESENT ILLNESS
• They gave h/o altered sensorium with mycoclonic jerks for 2 months.
• He also used to develop sudden , brief loss of contact with the environment.
5. HISTORY OF PRESENT ILLNESS
• His sensorium became gradually impaired .
• He was admitted at a hospital in nepal 2 months back, diagnosed as a case of Neurosyphilis,
was given benzathine penicillin but did not improved.
6. HISTORY OF PRESENT ILLNESS
• He became irritable in the hospital, for which he was given serenace (Haloperidol)and
thereafter he became unconscious, rigid.
• Despite withdrawing Serenace , his sensorium did not improved and since then he gradually
slipped to unconscious state. He was referred to BLK with these complaints.
7. PAST HISTORY
• He was diagnosed with hypertension 3 years back.
• No h/o DM
8. ON EXAMINATION
• He is semiconscious
• Vitals at the time of admission
BP:138/100 mm hg
PR: 98/minute
RR: 30/minute
Temp: 101* f
CVS :S1 S2 + No abnormal sounds
Chest: Bilateral air entry positive , No crepitations
Abdomen: Soft , No Swellings.
9. NEUROLOGICAL EXAMINATION
• Patient was opening his eyes spontaneously
• Not following commands
• Moving his limbs(flexion) in response to painful stimuli.
• Pupils: Normal in size , reacting to light
• No apparent cranial nerve abnormality
10. NEUROLOGICAL EXAMINATION
• Motor exam: Rigidity felt in all 4 limbs which is more in upperlimbs
• Flexion of both his upperlimbs in response to deep painful stimuli
• DTR-
• Plantar response: flexor response in both feet
• Myloclonic jerks – present in all 4 limbs.
11. INVESTIGATIONS
• CSF FLUID EXAMINATION FOR CELL COUNT
Clear ,colourless
Microscopic examination:
Total leucocyte count 5 cells/cu mm
(Adults- 0-5 cells/cu mm Neonate-0-30 cells/cu mm)
Differential leucocyte count
neutrophils 6/10 cells counted
lymphocytes 4/10 cells counted
12. INVESTIGATIONS
• TORCH PANEL- negative
• TB PCR
M.Tuberculosis complex : not detected
nontuberculosis mycobacteria: not detected
• CMV PCR- Negative.
• TPHA CSF- Negative
• Cryptococcal antigen CSF-Negative.
• CSF Indian ink staining -Encapsulated yeast cells resembling
Cryptococcus species not seen.
13. INVESTIGATIONS
• CSF Cerebrospinal fluid
Gram stain :
No cells seen.
No organisms seen.
Culture &sensitivity:
The culture is sterile after 5 days of aerobic incubation at 35
degree centigrade.
14. INVESTIGATIONS
CT SCAN OF THE HEAD – PLAIN
• The cerebral parenchyma shows normal attenuation values.
• The ventricles, cisterns and sulci are generally prominent.
• Bilateral basal ganglia calcification is seen.
• There is no midline shift.
• The cerebellar hemispheres and brain stem are normal.
• The 4th ventricle is normal in size and midline in position.
CONCLUSION:
The findings are consistent with diffuse cerebral atrophy consistent with the age of the
patient.
15. INVESTIGATIONS
• MRI findings are suggestive of marked diffuse cerebral atrophy with symmetric
diffuse signal alteration and restricted diffusion involving bilateral basal ganglia.
The thalami (pulvinar) or neocortex do not reveal any restricted diffusion. In view of short history,
possibility of rapidly progressive neurodegeneration - ? Creutzfeldt Jakob disease (sCJD) may be
considered. However, differentials of infective etiology with basal ganglia ischemia cannot be
entirely excluded. Clinical correlation & further work up with CSF analysis and EEG is suggested.
• Also noted are chronic white matter ischemic changes & mild cerebellar atrophy. No brainstem
atrophy is seen.
26. SLOW VIRUS INFECTIONS
• Slow virus infections are also known as prion diseases, after the presumed infectious agent,
as well as transmissible spongiform encephalopathies (TSEs), after the histopathologic
changes associated with these infections.
27. SLOW VIRUS INFECTIONS
• Prions are proteinaceous infectious particles (PrPs).
• The brain pathology of prion diseases consists of a vacuolar (spongiform) degeneration of the
neuropil, cortical neurons, and subcortical gray matter with neuronal loss and gliosis.
28. SLOW VIRUS INFECTIONS
• Early diagnosis is difficult, in part because prions do not have nucleic acids, making
conventional nucleic acid–based viral detection systems ineffective.
• PrPs also elude detection by not producing a humoral immune response.
30. TYPES OF SLOW VIRUS INFECTIONS
• Prion diseases are categorized into three groups: sporadic, (85%), hereditary &acquired(15%)
• 15% consist of hereditary forms (hereditary Creutzfeldt-Jakob disease [CJD], Gerstmann-
Straussler-Scheinker disease, fatal familial insomnia) and acquired forms.
• Acquired forms may be transmitted iatrogenically (through human growth hormone therapy, dura
mater grafts, or other neurosurgical procedures) or through cannibalism (kuru) , variant CJD
(vCJD) in humans, scrapie in sheep, and bovine spongiform encephalopathy (BSE) in cattle.
31. WORLD HEALTH ORGANIZATION DIAGNOSTIC CRITERIA FOR
CREUTZFELDT-JAKOB DISEASE
• 1. Creutzfeldt-Jakob Disease (CJD) clinical diagnosis:
Criteria for probable sporadic CJD:
• The clinical diagnosis of CJD is currently based on the combination of
progressive dementia, myoclonus, and multifocal neurological
dysfunction, associated with a characteristic periodic
electroencephalogram (EEG).
• However, new variant CJD, most growth hormone-related iatrogenic
cases, and up to 40% of sporadic cases are not noted to have the
characteristic EEG appearance. This
hampers clinical diagnosis, and hence surveillance, and illustrates
the need for additional diagnostic tests.
32. WORLD HEALTH ORGANIZATION DIAGNOSTIC
CRITERIA FOR
CREUTZFELDT-JAKOB DISEASE
• Proposed criteria for probable sporadic CJD:
a. Progressive dementia.
and
b. At least two out of the following four clinical features:
i. Myoclonus.
ii. Visual or cerebellar disturbance.
iii. Pyramidal/extrapyramidal dysfunction.
iv. Akinetic mutism.
and
2. A typical EEG during an illness of any duration.
and/or
3. A positive 14-3-3 cerebral spinal fluid assay and a clinical duration to death less than 2 years.
4. Routine investigations should not suggest an alternative diagnosis.
33. NEW-VARIANT CREUTZFELDT-JAKOB DISEASE:
SUSPECT CASE DEFINITION
• New-variant Creutzfeldt-Jakob disease (nvCJD) cannot be diagnosed with certainty on clinical
criteria alone at present.
• However, based on the 23 neuropathologically confirmed cases, the diagnosis of nvCJD
should be considered as a possibility in a patient with a progressive neuropsychiatric
disorder, with at least five out of the following six List 1 clinical features. The suspicion of
nvCJD is strengthened by the following criteria in List 2.
34. NEW-VARIANT CREUTZFELDT-JAKOB DISEASE:
SUSPECT CASE DEFINITION
• A patient with a progressive neuropsychiatric disorder and five out of the six clinical features in List 1 and all of the
criteria in List 2 should be considered as a suspect case of nvCJD for surveillance purposes.
• List 1
1. Early psychiatric symptoms.
2. Early persistent parasthesias/dysesthesias.
3. Ataxia.
4. Chorea/dystonia or myoclonus.
5. Dementia.
6. Akinetic mutism.
• List 2
1. The absence of a history of potential iatrogenic exposure.
2. Clinical duration more than 6 months.
3. Age at onset less than 50 years.
4. The absence of a PrP gene mutation.
5. The EEG does not show the typical periodic appearance.
35. DEFINITION OF CREUTZFELDT-JAKOB DISEASE
SUBTYPES
• Sporadic CJD
1. Definite Diagnosed by standard neuropathological techniques and/or immunocytochemically
and/or Western blot-confirmed protease-resistant PrP and/or presence of scrapie associated
fibrils.
2. Probable
a. Progressive dementia and at least two out of the following four clinical features:
• Myoclonus.
• Visual or cerebellar disturbance.
• Pyramidal/extrapyramidal dysfunction.
• Akinetic mutism.
and
b. A typical EEG during an illness of any duration
and/or
c. A positive 14-3-3 CSF assay and a clinical duration to death less than 2 years.
d. Routine investigations should not suggest an alternative diagnosis.
3. Possible Same clinical criteria as definite but no, or atypical, EEG and duration less than 2 years
• Iatrogenic CJD Progressive cerebellar syndrome in a recipient of human cadaveric-derived pituitary hormone;
Or sporadic CJD with a recognized exposure risk, e.g., antecedent neurosurgery with dura
mater graft
• Familial CJD Definite or probable CJD plus definite or probable CJD in a first-degree relative
36. NEUROPATHOLOGICAL CRITERIA FOR CREUTZFELDT-JAKOB
DISEASE AND OTHER HUMAN TRANSMISSIBLE
SPONGIFORM ENCEPHALOPATHIES
• 1. Creutzfeldt-Jakob disease (CJD)—sporadic, iatrogenic (recognized risk) or familial (same disease in first
degree relative or disease-associated PrP gene mutation): Spongiform encephalopathy in cerebral and/or
cerebellar cortex and/or subcortical gray matter; and/or encephalopathy with prion protein (PrP) immunoreactivity
(plaque and/or diffuse synaptic and/or patchy/perivacuolar types).
• 2. New-variant CJD—Spongiform encephalopathy with abundant PrP deposition; in particular, multiple fibrillary
PrP plaques surrounded by a halo of spongiform vacuoles (“florid” plaques, “daisy-like” plaques) and other PrP
plaques, and amorphous pericellular and perivascular PrP deposits; especially prominent in the cerebellar
molecular layer.
• 3. Gerstmann-Sträussler-Scheinker disease (in family with dominantly inherited progressive ataxia and/or
dementia and one of a variety of PrP gene mutations): Encephalo(myelo)pathy with multicentric PrP plaques.
• 4. Familial fatal insomnia (in member of a family with a PrP gene mutation at codon 178 in frame with methionine
at codon 129): Thalamic degeneration, variable spongiform change in cerebrum.
• 5. Kuru: Spongiform encephalopathy in the Fore population of Papua New Guinea.
37. CONDITIONS THAT MAY CAUSE A CREUTZFELDT-
JAKOB DISEASE-LIKE ELECTROENCEPHALOGRAM
• Alzheimer’s disease
• Hyperammonemia
• Lewy body disease
• Binswanger’s disease
• AIDS dementia
• Hyperparathyroidism
• Hypo- and hypernatremia
• Hypoglycemia
• Multiple cerebral abscesses
• MELAS syndrome
• Hepatic encephalopathy
• Baclofen, mianserin, metrizamide, and lithium toxicity
• Postanoxic encephalopathy
• MELAS ( mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes)
38. CONDITIONS OTHER THAN CREUTZFELDT-JAKOB
DISEASE THAT CAN HAVE A POSITIVE 14-3-3 RESULT
• Herpes simplex and other encephalitides
• Stroke (especially recent)
• Subarachnoid haemorrhage
• Hypoxic/Ischemic encephalopathy
• Barbiturate intoxication
• Glioblastoma
• Carcinomatous meningitis (especially small-cell lung carcinoma)
• Paraneoplastic encephalopathy
• Corticobasal degeneration