45 male present with;
early congnitive symptoms, involuntary jerky movement (chorea), severe dementia and depression..

_Brain imaging:
Marked degeneration of caudate with lateral ventricle dilated.


_Which one of the following is the diagnosis of this case:

1-Huntington disease
2-parkinsonism
3-alzheimer


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#patho_in_clinic

#قسم_الباثو
#اللجنة_العلمية_للدفعة_الخامسة

🖍تشخيص الحالة:
。　✩ 。　　✩。　　✩
⋆。　＼　　｜　　／。　⋆
 Huntington disease(HD)
⋆。　／　　｜　　＼。　⋆
。　✩。 　　。　　✩。

وهو عبارة عن:
  autosomal dominant movement disorder.

📌ويحدث نتيجة:
Degeneration of the striatum (caudate & putamen) in basal ganglia
يحدث موت للخلايا بسبب زيادة exotoxins فتزيد الاشارات العصبية فبالتالي يزيد
↑Ca+2 influx → instability

◐◐◐◐◐◐◐◐◐◐◐◐◐◐◐

📌Pathogenesis:
abnormal protein form →leading to →abnormal movement & cognitive symptoms.

🔖الخلل يكون في الكروموسوم رقم ٤ في huntinton gene
يحدث تكرار للCAG sequence مما يؤدي لتكون بروتين abnormal

🔖التكرار الطبيعي للـCAGيكون من(11_34) أليل
أما في الـhuntinton patient فيكون 36 فأكثر

🔖one affected copy in gene cause the disease
وينتقل المرض من الأباء إلى الابناء لذلك فهو موجود في كل generation ذكورًا واناث بنسبة 50%

🔖 Triple repeated of CAG
يكون glutamine لذلك امراض الhuntinton يمتلكون منه ٣٦ أو أكثر
(polyglutamine  disease)

◐◐◐◐◐◐◐◐◐◐◐◐◐◐◐

💢 Anticipation:
Earlier symptoms onset with each generation. 

◐◐◐◐◐◐◐◐◐◐◐◐◐◐◐

📌Symptoms:

تظهر من عمر ٤٠ وهذا يعتمد على عدد تكرار CAG nucleotide ...

1_involantary  jerky movement
2_writhing movement of extremities
4_early congnitive ;
~forgetfulness
~severe dementia

5_behavioral changes:
~increase risk of suicide
6_chorea (dance movement)
7_depression

◐◐◐◐◐◐◐◐◐◐◐◐◐◐◐

📌Gross examination:
حجم الدماغ يكون صغير بسبب الatrophy لل caudate and putamen

وatrophy في frontal والparietal lobe

📌Microscopic examination:

Severe loss of neurons in affected striatum

◐◐◐◐◐◐◐◐◐◐◐◐◐◐◐

🔖يكون عندنا في هذا المرض:
#↓GABA
#↓Acrtylcholine
#↑Dopamine

◐◐◐◐◐◐◐◐◐◐◐◐◐◐◐

📌Treatment:


doesn't affect overall servival

❌Death❌
Occur 10 _20 years after diagnosis

◐◐◐◐◐◐◐◐◐◐◐◐◐◐◐

والسلام عليكـم..


#patho_in_clinic

#قسم_الباثو
#اللجنة_العلمية_للدفعة_الخامسة

63-years old female patient present with:
dementia, Hypokinesia, shuffling gait,sleep disturbance and  termors at rest but diminish with movement.

_Drug history:MPTP

_ON MICROSCOPIC examination: Lewy bodies

_Diagnosis of this case is...?!
① spinocerebellar ataxias.
② Parkinson's disease
③Huntington disease

_The cause of this disease is:
①↓GABA
②↑GABA
③↑Dopamine
④↓Dopamine



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#patho_in_clinic

#قسم_الباثو
#اللجنة_العلمية_للدفعة_الخامسة

🪄تشخيص الحالة:

。　✩ 。　　✩。　　✩
⋆。　＼　　｜　　／。　⋆
parkinson disease (PD)
⋆。　／　　｜　　＼。　⋆
。　✩。 　　。　　✩。

وهو بشكل عام عبارة عن ..
*Movment disorder*

◼️Is the most common  neurodegenerative disease.


◾️Caused by:
Loss of Dopaminergic neurons in substantia nigra in basal ganglia
والتي بدورها تتحكم بالـ movement
وتتصل بالـ cerebral cortex وتبدأ الحركة.

◾️Age:
Adult onset disease
1٪ of peopleover 60 (50_80)years old

◾️ يصيب الذكور والإناث

.ꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤ.

◯ Usually →no known cause (idiopathic)

◯ Sometimes→ there is genitic factor(mutation of encoding of a-synuclein, parkin, others)

~drugs as:MPTP
.ꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤ.

◾️CLINICAL FEATURES:

1)termors وهو مميز هنا انه (at rest)
ويقل مع الحركة عكسcerebellar disease 
2)rigidity of proximal muscle
3)bradykinesia ( slow)
5)hypokinesia (قلة الحركة)
6)instability.
7)shuffling gait (small steps)

وقد تصاحبها اعراض مشهورة لاحقاً مثل:
-dementia (خرف)
-depression
-sleep disturbances
-difficultysmelling



🔺- هل parkinsonism هو نفسه PD ؟!
لا..
The parkinsonism is clincal syndroms of PD
هو عبارة عن مجموعة اعراض تشبه اعراض pd وممكن تحدث في أمراض اخرى غير pd..
ولها عدة اسباب منها
١-الأدوية   ٢-toxins   ... وغيرها
وأشهرها الــ (idiopathic  pd)

يعني نقدر نقول أن pd جزء من parkinsonism

ꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤ

◾️GROSS:
pallor of the substantia nigra

◾️MICROSCPIC:
☆- loss of pigmented neurons and gliosis

☆-Lewy bodies →وهي علامة مميزة للمرض

ꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤ

◾️Treatment:
الإستراتيجيه الأساسية هنا هى زيادة الدوبامين داخل الدماغ..

وبما ان الدوبامين لا يستطيع العبور من BBB نجيب لهم Levodopa الذي يتحول داخل الbrain الى دوبامين

وممكن نعطيهم antiviral مثل Amantadine  يزيد من إنتاج الدوبامين

وهنا العلاج يساعد من تخفيف الأعراض لكنه لا يستطيع توقيف progressive  neurodegenerative..

ꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤꕤ

والسلام عليكم ..

#patho_in_clinic

#قسم_الباثو
#اللجنة_العلمية_للدفعة_الخامسة

▪️2-year-old boy with gross motor delays presented with wide-based, unsteady gait and headaches over 1 month. A brain MRI reveals a large posterior fossa mass (☝️🏼☝️🏼). 
He has mutations in the gene for β-catenin .
He had no evidence of familial predisposition syndrome.

What's your diagnosis?

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@Patho_5th_bot


#patho_in_clinic

#قسم_الباثو
#اللجنة_العلمية_للدفعة_الخامسة

▪️تشخيص الحالة ..
✨..(Medulloblastoma)..✨
حيث وتصيب الأطفال كما هو موضح بحالتنا ونلاحظ من خلال الأعراض قبل صورة ال MRI أن الطفل عنده مشكلة بالحركة والمشي والتوازن وهذا كله من وظائف المخيخ ال cerebellum فهذا الورم يصيب المخيخ والصورة المرفقة تؤكد مكان ال mass كما أن الصداع كان chronic وناتج بسبب ال pressure ..


#patho_in_clinic

#قسم_الباثو
#اللجنة_العلمية_للدفعة_الخامسة

▫️a 17-year-old young man unknown to have any medical illness transferred from other hospital as a case of hydrocephalus and intraventricular hemorrhage (IVH). He had a complain of headache for 3days that subsided with paracetamol.
(CT) was performed, and it showed a well-defined, lobulated large mass measuring expanding the frontal horn and body of right lateral ventricle .

□ What is your diagnosis?

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#patho_in_clinic

#قسم_الباثو
#اللجنة_العلمية_للدفعة_الخامسة

اضافات عين شمس من روبنز ومن فيديوهات علم ينتفع به👌🏻

#كتب_الله_أجر_مشاركه
#اللجنة_العلمية_للدفعة_الخامسة

▫️تشخيص الحالة ..
✨..(Central neurocytoma)..✨
يعتبر low grade neoplasm يوجد في ال ventricle وخاصة بال lateral ventricle وتسبب ضغط و dilatation للventricle وكأنها hydrocephalus ولكونه سرطان كان مُصاحب بنزيف بالمنطقة فأعتقدوا بالبداية أنه hydrocephalus ..
لكن ال ventricle لم تكن مملوءة بالسائل كانت تحوي كتلة صلبة ومحددة وهذا هو تشخيص الحالة ..

#patho_in_clinic

#قسم_الباثو
#اللجنة_العلمية_للدفعة_الخامسة

A 4-year old girl come to developed clumsiness and difficulty ambulatory over 6 months 
with chief complain involuntary eyes movement
And problem with balance and coordination physical examination, show difficult balance while walking, dysarthria,poor hand coordination absence deep tendon reflexes There was muscular weakness, she developed congestion heart failure and diabetes with occurs mitochondria dysfunction and oxidative damage

What's the diagnosis of cases?
What's the trinucloted disease?


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#patho_in_clinic

#قسم_الباثو
#اللجنة_العلمية_للدفعة_الخامسة

A prion is a misfolded protein that can cause other proteins to misfold in a chain reaction. This can lead to damage to the brain and other tissues. Prion diseases are rare and always fatal.

There are several types of prion diseases, including:

Creutzfeldt-Jakob disease (CJD): CJD is the most common prion disease in humans. It is characterized by a rapidly progressive dementia and other neurological symptoms.
Gerstmann-Sträussler-Scheinker syndrome (GSS): GSS is a rare prion disease that is characterized by a slowly progressive dementia and other neurological symptoms.
Fatal familial insomnia (FFI): FFI is a rare prion disease that is characterized by insomnia, difficulty walking, and other neurological symptoms.
Kuru: Kuru is a prion disease that is found in Papua New Guinea. It is caused by cannibalism.
Bovine spongiform encephalopathy (BSE): BSE, also known as mad cow disease, is a prion disease that affects cattle. It can be transmitted to humans who eat infected meat.
Prion diseases are caused by the misfolding of a protein called prion protein (PrP). PrP is found in all mammals, but it is only misfolded in people and animals with prion diseases. The misfolded PrP can then cause other PrP molecules to misfold, leading to a chain reaction that damages the brain and other tissues.

There is no cure for prion diseases. Treatment is supportive and aimed at relieving symptoms.

Prion diseases are rare, but they are always fatal. The incubation period for prion diseases can be long, ranging from months to years. The symptoms of prion diseases vary depending on the type of disease, but they can include:

Dementia
Memory loss
Difficulty walking
Seizures
Muscle rigidity
Coma

Prion diseases are transmitted through contact with infected tissue or fluids. This can happen through eating infected meat, through medical procedures, or through contact with blood or other bodily fluids.

There is no way to prevent prion diseases. However, there are steps that can be taken to reduce the risk of transmission, such as avoiding eating infected meat and practicing good hygiene.

If you are concerned about prion diseases, please talk to your doctor.

#مشاركة_مهدي_الزبيدي
#اللجنة_العلمية_للدفعة_الخامسة

#GROSS

#Diagnosis 👉🏻 Normal cerebral cortex


#The_black_arrows
Neurons
Large nuclei with prominent nucleoli

#The_red_arrows
Oligodendrocyte
Small hyperchrmatic nuclei with clear cytoplasm

#The_blue_arrows
Astrocyte
Start or elongated or triangle

#The_brown_area
Microglial cell
Nucleus elongated

#GROSS

#Diagnosis 👉🏻 Normal white matter


#The_black_arrows
Axon

#The_blue_arrows
Oligodendrocyte

#The_red_arrows
Microglia

#The_white_arrow
Astrocyte

CEREBRAL INFRACTION
Due to sever ischemia

𑁍 THE FEATURES
• In the first day
- Neurons cells shrunken (pyknotic) with eosinophilic cytoplasm ( red Neurons


• from first day to third day
- Neurons cells without nucleus
- Neutrophils infiltration in the parenchyma
- Neutrophils attack eosinophilic neurons
- Cuff of neutrophils around and within Blood vessels


• from sixth day to seventh day
- Foamy macrophages engulf the necrotic cells and debris

• After twelfth day
Occurs Gliosis with increase of Astrocytes
(non functional)

#GROSS

#Diagnosis 👉🏻 Cerebral infarct
After 2 weeks


#The_black_arrow
Liquefactive necrosis
Due to Ischemia

#The_White_arrow
Oil hole (cystic spaces) with hemorrhage

#MICROSCOPIC

#Diagnosis 👉🏻 Cerebral infarct (Acute hypoxic-ischemic injury)
In first day


#The_black_arrows
Glial cells

#The_blue_arrows
Neurons
- Eosinphilic cytoplasm
- Shrinkage of the nucleus (pyknosis)

#Type_of_stain
HE
In ischemia the cells cytoplasm ➪ eosinphilic due to protein denaturation

#MICROSCOPIC

#Diagnosis 👉🏻 Cerebral infarct
1-3 days


#The_black_arrows
Neutrophils attack red neurons

#The_white_arrows
Neutrophils infiltration

#The_red_arrows
clusters of erythrocytes

#The_blue_arrows
Neurons without nucleus

#The_black_star
Cuff of neutrophils around and within Blood vessels

#MICROSCOPIC

#Diagnosis 👉🏻  Cerebral infarct
6-7 days


#The_black_arrows
Foamy macrophages

#MICROSCOPIC

#Diagnosis 👉🏻  Cerebral infarct
After twelfth day