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The 3-minute film reading column is updated every two weeks, and selected wonderful cases in the past two weeks for everyone to learn and discuss
.
Medical pulse nerve has a fast-paced breakfast case study five working days a week, 3 short and concise small cases every day, and 3 knowledge points every day
.
The collision of everyone's thoughts has produced countless sparks of wisdom, often wandering in the group, and getting new harvests every day
.
Case provided: Pei Zheng, Liu Xiang, He Zhongyan, Zhao Yaohui, Zhang Wensheng, Jiang Hongqi, Li Huiming, Liu Liquan, Liang Xinming, Shen Yongguo, Huang Botao Author: Zhao Wei, Zhao Lianhua Case 120 years old, male, 3 months after liver transplantation Acute irritability, convulsions, shortness of breath, dysphagia, and dystonia of the mouth and neck occur
.
The patient's condition deteriorated rapidly, including disturbance of consciousness and generalized tonic-clonic seizures, further progressing to a coma and loss of brainstem reflexes
.
Answer: Rabies encephalitis
.
Coronal and axial FLAIR images showed hyperintensity and mild swelling in the bilateral frontal cortex, hippocampus, and dorsal thalamus, and focal hyperintensity in the left caudate and lentiform nuclei
.
Axial T2-weighted image shows focal pons hyperintensity
.
Diffusion-weighted images and ADC maps showed corresponding restricted diffusion in bilateral fronto-parietal cortex, thalamus, and hippocampus
.
Human rabies has two clinical manifestations: the typical encephalitic type begins with nonspecific prodromal symptoms of autonomic dysfunction and progresses to hydrophobia, phonophobia, bloodlust, fluctuating levels of consciousness, and eventually coma
.
The paralytic type shows muscle paralysis, inhibition of consciousness and sensory systems
.
The main MRI findings included increased T2/FLAIR signal, preferentially involving the brainstem, hippocampus, thalamus, basal ganglia, frontal lobes, and parietal lobes
.
The encephalitic and paralytic forms have similar imaging findings, but the paralytic forms more commonly involve the medulla oblongata and spinal cord
.
The case was a 247-year-old male with bradykinesia and unsteady walking in his right hand for 8 months
.
Medopa, selegiline treatment effect is not good
.
Answer: Hereditary diffuse white matter lesions with spherical axons (HDLS)
.
CSF1R gene (the pathogenic gene of HDLS) detected c2381T>C(p.
lle794Thr)
.
HDLS is an autosomal dominant inherited progressive leukoencephalopathy with progressive cognitive impairment, mental and behavioral abnormalities, movement disorders, and stroke-like episodes as the main clinical manifestations.
Hormone therapy is ineffective
.
The characteristic pathological changes are diffuse white matter lesions with prominent axonal spheroid degeneration; pathological features include axonal spheroid formation, demyelination, axonal destruction, and gliosis
.
The imaging manifestations of HDLS include: bilateral frontal and fronto-parietal white matter T2/FLAIR hyperintensity, T1 hypointensity, early asymmetric manifestations of patchy or localized lesions, mostly non-enhancing, DWI signal may increase , some cases of CT scan showed spot-like calcifications in the lesion area - bilateral lateral ventricle body and central semiovale area DWI persistent high signal (relatively characteristic changes)
.
It mainly involves the deep white matter and the circumlateral ventricle area, but also the corpus callosum and corticospinal tract, but does not involve the "U"-shaped fibers.
Ventricular enlargement and secondary brain atrophy may occur, but generally there is no gray matter, brain stem, and cerebellum atrophy.
.
The 367-year-old female case was treated with oral hormone Metrol for pulmonary interstitial lesions in mid-April.
Tooth extraction was performed on May 12, and braces were placed on May 19.
On June 5, right limb weakness and fever developed
.
Lumbar puncture pressure 70, protein 2380, leukocyte 280 CSF lower limit of chloride, sugar 8, slightly higher (blood sugar 15), unconsciousness after admission, no mental symptoms, normal cardiac color Doppler ultrasound
.
Answer: Listeria encephalitis
.
Blood culture: positive for Listeria
.
Analysis: low-viral bacteria + susceptible heart disease = infective endocarditis
.
Toxic secondary infectious vasculitis secondary to infarction
.
The former is mainly embolism, with more cortex, and the latter is mainly small arteries, with more deep basal ganglia
.
Reasons for the high mortality of Lister: The first etiological basis is difficult to obtain, and the second conventional anti-infective combination (aciclovir + cephalosporin) (Vangu + Merlot) is difficult to cover Lister
.
When Listeria monocytogenes causes central nervous system infection, most patients present with meningitis, and a few can involve the brain parenchyma, causing encephalitis or brain abscess.
Brainstem encephalitis is a special and classic type
.
About 9% to 11%
.
The clinical manifestations of Listeria brainstem encephalitis are typically biphasic, with nonspecific intracranial infection symptoms such as fever, headache, nausea, and vomiting in the early stage
.
Cranial nerve palsy and brainstem involvement appear around 4 days after onset, with rapid onset, rapid progression, and prone to respiratory failure, with extremely high disability and mortality rates
.
Early brain MRI can clearly show the lesions of brainstem encephalitis.
The low brainstem (pons and medulla), cerebellum and upper cervical spinal cord are most commonly involved.
Diffusion
.
Patchy, nodular, or annular enhancement may be present, the latter suggesting abscess formation
.
Case 4, a 41-year-old male patient, was admitted to the hospital because of poor speech for 16 hours and headache and numbness of the left upper limb for 3 hours
.
Answer: Posterior Reversible Encephalopathy Syndrome (PRES)
.
Admission blood pressure: 220/150mmHg, family history of hypertension
.
After 2 weeks, the brainstem lesions disappeared
.
Clinical symptoms of PRES include seizures, encephalopathy, headache, visual disturbances, etc.
The common causes are malignant hypertension, renal failure, use of cytotoxic drugs, autoimmune diseases, preeclampsia or eclampsia
.
The mechanism is that the acute blood pressure fluctuation or the direct action of cytokines on the vascular wall causes endothelial damage, causing the destruction of the blood-brain barrier, and then causing reversible vasogenic edema
.
PRES is divided into two types: typical PRES and variant PRES
.
Typical PRES include the following three types: superior frontal groove, full watershed, and parieto-occipital (common)
.
Variant PRES typically only involves the brainstem, cerebellum, and basal ganglia, while the fronto-parietal-occipital cortex and subcortical white matter are not involved
.
Partial involvement of the spinal cord is found in variant PRES, so it is called the reversible posterior encephalopathy syndrome with spinal cord involvement
.
Case A 553-year-old male was admitted to the hospital on October 17, 2018 due to "sudden right limb hemiplegia for 1 day"
.
Head MRI in November 2015 was as follows: Answer: Cerebral autosomal dominant arteriopathy with subcortical infarction and leukoencephalopathy (CADASIL) with cerebral hemorrhage
.
The patient's medical history of the first stroke (47 years old) can be traced back to 2012, and the earliest imaging can be traced back to 2013.
According to imaging, there were 3 cerebral infarctions, all of which were lacunar cerebral infarction
.
The patient's mother died after a stroke at the age of 60, and her mother and brother also died of a stroke at a younger age
.
The characteristics of the case: 1.
Young people with repeated attacks; 2.
Family history; 3.
Subcortical infarction + leukoencephalopathy + lobar hemorrhage + multiple microbleeds; 4.
Arg607Cys hybridization mutation detected in NOTCH3 exon 11
.
Non-sclerotic, non-amyloidotic, and medial smooth muscle cell degeneration with GOM deposition in arterioles distinguish CADASIL from other forms of small arterial disease.
These vascular changes can occur in the brain, kidney, spleen, liver, muscle, and skin
.
Case 6 patient, male, 67 years old, was admitted to hospital due to "sudden left limb movement disadvantage for more than 8 hours"
.
Head CT: Symmetrical calcifications in bilateral cerebellum, basal ganglia, gray-white matter junction, and left occipital cortex
.
Answer: Fahr's disease with cerebral hemorrhage
.
The patient's genetic examination revealed a mutation in the SLC20A2 gene
.
Fahr disease is also known as familial idiopathic cerebrovascular ferrocalcinosis
.
Bilateral symmetrical calcification of the basal ganglia caused by a variety of causes is called Fahr syndrome
.
Fahr disease is mostly autosomal dominant or recessive
.
A total of 4 pathogenic genes have been found so far: SLC20A2, PDGFRB, PDGFB and XPR1
.
Mutations in genes lead to impairment of intracellular and extracellular inorganic phosphorus transport and disruption of the blood-brain barrier
.
Diagnostic criteria for Fahr disease: (1) bilateral basal ganglia calcification on imaging; (2) no hypoparathyroidism; (3) normal serum calcium and phosphorus; (4) normal tubular response to parathyroid hormone ; (5) No infection, poisoning, metabolism and other reasons
.
The main clinical manifestations are progressive mental disorders, mental retardation, seizures, language disorders, and severe growth disorders
.
Laboratory tests for parathyroid hormone, serum calcium, and phosphorus were normal
.
Symmetrical calcifications in the brain are the main imaging findings, calcifications in the basal ganglia can be characterized by a "bird's dialogue" sign, and cerebellar dentate nucleus calcifications are generally crescent-shaped
.
In addition, there are many white matter demyelination and brain atrophy, which can be combined with cerebral hemorrhage and cystic degeneration
.
The atrophy mainly affects the white matter areas, resulting in enlargement of the ventricles
.
Differentiate from hypoparathyroidism
.
Case 7 was a male, 34 years old
.
Admitted to hospital for "7 hours of unconsciousness after drinking alcohol"
.
The patient had a history of heavy drinking for 4 years, about 2 catties of liquor per day
.
Answer: Marchiafava-Bignami disease (MBD)
.
Clinical manifestations: There is a long history of chronic alcoholism, and it is more common in middle-aged and elderly men
.
MBD is divided into two subtypes, A and
B.
Type A clinical symptoms are mainly coma
.
Type B presents with normal or mild disturbance of consciousness
.
According to the severity of onset, MBD can be divided into three types: acute, subacute and chronic
.
Acute phase: sudden onset, disturbance of consciousness, psychiatric symptoms, epileptic seizures (frequent or persistent), acute onset of unsteady gait and severe neurological deficit, among which epileptic seizures and acute onset of unsteady gait may be the first onset Symptoms, often fatal in a short period of time; subacute phase: ataxia, dysarthria, hypertonia, rapidly progressive dementia that can persist to chronic forms; chronic phase: dissociative syndrome (dextrose due to hydrazine callopathies) Left hand apraxia, inability of one hemisphere to respond to visual or somatosensory stimuli projected to the other) and progressive dementia
.
More MBD patients died 4 to 6 years after the disease
.
There are also reports in the literature that many acute and subacute patients can survive for a long time
.
The imaging manifestations of acute MBD are diffuse swelling of the corpus callosum, body and/or knee, iso or slightly hypointense signal on T1W1, high signal on T2W1 and FLAIR, high signal on DWI, and low signal on ADC.
Appears as round or irregular patchy enhancement
.
Case 8, a 63-year-old female, was first admitted to hospital on September 20, 2021, with slurred speech for 3 days, aggravation and left limb weakness for 7 hours
.
2021.
09.
21 D-dimer (DD) 10.
75mg/L, 2021.
09.
23 D-dimer (DD) 12.
67mg/L, 2021.
09.
28 D-dimer (DD) 19.
30mg/L
.
The patient was readmitted to the hospital on October 4, 2021 due to "slurred speech and left limb weakness for half a month, aggravating for 1 day"
.
2021.
10.
5 D-dimer (DD) 24.
56mg/L
.
Answer: Trusseau Syndrome
.
The patient had multiple metastases from cervical cancer
.
For unexplained markedly elevated D-dimer levels, even in the absence of clinical manifestations, the possibility of VTE should be considered; after excluding thrombotic diseases and liver and kidney diseases, the possibility of malignancy should be highly suspected
.
Case 9 is a 79-year-old male who was admitted to the hospital due to "memory loss for more than 20 days"
.
Past history of lung cancer
.
Answer: Brain metastases
.
Studies have shown that brain metastases calcifications are more common in lung adenocarcinoma (56%), followed by breast ductal invasive carcinoma (20%) and small cell lung cancer (11.
8%).
Calcification is multiple
.
Calcification types can be divided into punctate, nodular, annular, clustered, and large granular (below)
.
Most of the calcification types are punctate calcifications, and there is no specific correlation between the type and the original tumor histology
.
Case 10 female, 1 year and 3 months old, had severe motor and cognitive developmental delay after birth, frequent focal epilepsy after 10 months, progressive aggravation, head circumference gradually increased, and bone suture cracking in the later stage
.
Brain MRI and CT are as follows: Answer: GM1 gangliosidosis
.
GM1 gangliosidosis is a rare autosomal recessive genetic disease.
The main clinical features of patients are psychomotor regression, abnormal muscle strength, convulsions and other manifestations of central nervous system damage
.
The gene encoding β-galactosidase, GLB1, is a disease-causing gene.
Mutation of the gene can reduce the activity of β-galactosidase, leading to the degradation of GM1 gangliosides and accumulation in the body, resulting in a series of clinical manifestations
.
According to the age of onset and the severity of the disease, GM1 gangliosidosis can be divided into infantile (type I), juvenile (type II) and adult (type III)
.
Infant type, often onset within 6 months after birth, manifested as severe central nervous system, bone and other damage, extremely serious disease, more than 2 years of age before death; juvenile type, more than 7 months to 3 years after birth Onset, the course of the disease progresses slowly, often manifested as progressive psychomotor retardation, convulsions, abnormal muscle strength, and skeletal developmental abnormalities, but less severe than infantile type; adult type, the onset time span is longer, average 3-30 years old It can be onset, and the clinical manifestations vary greatly.
It can be mild dystonia and language impairment, or severe extrapyramidal damage and intellectual disability
.
Imaging features: On FLAIR/T2-weighted sequences, there are bilaterally symmetrical high-intensity signals in the basal ganglia, mainly in the putamen
.
Significant neuronal loss in the head of the putamen and caudate nucleus with secondary gliosis is thought to be responsible for the MRI findings
.
Case 11 was a male, one month old, with refractory hypoglycemia in the neonatal period
.
Answer: Pituitary Stem Block Syndrome (PSIS)
.
Pituitary stalk blocking syndrome refers to the clinical syndrome caused by the absence or obvious thinning of the pituitary stalk, combined with posterior pituitary ectopic, and the hormone secreted by the hypothalamus cannot be transported to the pituitary through the pituitary stalk, resulting in hypoplasia of the anterior pituitary
.
Most children with PSIS are diagnosed with delayed growth and development in childhood, delayed sexual development, or lack of development beyond puberty
.
The main manifestations of PSIS in the neonatal period are hypoglycemia and delayed resolution of jaundice caused by cholestasis
.
Neonatal hypoglycemia is not uncommon, the more common hypoglycemia caused by insufficient intake and hyperinsulinemia
.
Hypoglycemia occurs in the neonatal period.
If it is associated with other neurological manifestations, endocrine diseases should be considered in addition to ingestion, absorption, and metabolic diseases.
In addition to insulin and C-peptide, thyroid function and adrenal function should also be checked.
, Once multiple pituitary hormone deficiencies are found, pituitary disease should be considered
.
.
Medical pulse nerve has a fast-paced breakfast case study five working days a week, 3 short and concise small cases every day, and 3 knowledge points every day
.
The collision of everyone's thoughts has produced countless sparks of wisdom, often wandering in the group, and getting new harvests every day
.
Case provided: Pei Zheng, Liu Xiang, He Zhongyan, Zhao Yaohui, Zhang Wensheng, Jiang Hongqi, Li Huiming, Liu Liquan, Liang Xinming, Shen Yongguo, Huang Botao Author: Zhao Wei, Zhao Lianhua Case 120 years old, male, 3 months after liver transplantation Acute irritability, convulsions, shortness of breath, dysphagia, and dystonia of the mouth and neck occur
.
The patient's condition deteriorated rapidly, including disturbance of consciousness and generalized tonic-clonic seizures, further progressing to a coma and loss of brainstem reflexes
.
Answer: Rabies encephalitis
.
Coronal and axial FLAIR images showed hyperintensity and mild swelling in the bilateral frontal cortex, hippocampus, and dorsal thalamus, and focal hyperintensity in the left caudate and lentiform nuclei
.
Axial T2-weighted image shows focal pons hyperintensity
.
Diffusion-weighted images and ADC maps showed corresponding restricted diffusion in bilateral fronto-parietal cortex, thalamus, and hippocampus
.
Human rabies has two clinical manifestations: the typical encephalitic type begins with nonspecific prodromal symptoms of autonomic dysfunction and progresses to hydrophobia, phonophobia, bloodlust, fluctuating levels of consciousness, and eventually coma
.
The paralytic type shows muscle paralysis, inhibition of consciousness and sensory systems
.
The main MRI findings included increased T2/FLAIR signal, preferentially involving the brainstem, hippocampus, thalamus, basal ganglia, frontal lobes, and parietal lobes
.
The encephalitic and paralytic forms have similar imaging findings, but the paralytic forms more commonly involve the medulla oblongata and spinal cord
.
The case was a 247-year-old male with bradykinesia and unsteady walking in his right hand for 8 months
.
Medopa, selegiline treatment effect is not good
.
Answer: Hereditary diffuse white matter lesions with spherical axons (HDLS)
.
CSF1R gene (the pathogenic gene of HDLS) detected c2381T>C(p.
lle794Thr)
.
HDLS is an autosomal dominant inherited progressive leukoencephalopathy with progressive cognitive impairment, mental and behavioral abnormalities, movement disorders, and stroke-like episodes as the main clinical manifestations.
Hormone therapy is ineffective
.
The characteristic pathological changes are diffuse white matter lesions with prominent axonal spheroid degeneration; pathological features include axonal spheroid formation, demyelination, axonal destruction, and gliosis
.
The imaging manifestations of HDLS include: bilateral frontal and fronto-parietal white matter T2/FLAIR hyperintensity, T1 hypointensity, early asymmetric manifestations of patchy or localized lesions, mostly non-enhancing, DWI signal may increase , some cases of CT scan showed spot-like calcifications in the lesion area - bilateral lateral ventricle body and central semiovale area DWI persistent high signal (relatively characteristic changes)
.
It mainly involves the deep white matter and the circumlateral ventricle area, but also the corpus callosum and corticospinal tract, but does not involve the "U"-shaped fibers.
Ventricular enlargement and secondary brain atrophy may occur, but generally there is no gray matter, brain stem, and cerebellum atrophy.
.
The 367-year-old female case was treated with oral hormone Metrol for pulmonary interstitial lesions in mid-April.
Tooth extraction was performed on May 12, and braces were placed on May 19.
On June 5, right limb weakness and fever developed
.
Lumbar puncture pressure 70, protein 2380, leukocyte 280 CSF lower limit of chloride, sugar 8, slightly higher (blood sugar 15), unconsciousness after admission, no mental symptoms, normal cardiac color Doppler ultrasound
.
Answer: Listeria encephalitis
.
Blood culture: positive for Listeria
.
Analysis: low-viral bacteria + susceptible heart disease = infective endocarditis
.
Toxic secondary infectious vasculitis secondary to infarction
.
The former is mainly embolism, with more cortex, and the latter is mainly small arteries, with more deep basal ganglia
.
Reasons for the high mortality of Lister: The first etiological basis is difficult to obtain, and the second conventional anti-infective combination (aciclovir + cephalosporin) (Vangu + Merlot) is difficult to cover Lister
.
When Listeria monocytogenes causes central nervous system infection, most patients present with meningitis, and a few can involve the brain parenchyma, causing encephalitis or brain abscess.
Brainstem encephalitis is a special and classic type
.
About 9% to 11%
.
The clinical manifestations of Listeria brainstem encephalitis are typically biphasic, with nonspecific intracranial infection symptoms such as fever, headache, nausea, and vomiting in the early stage
.
Cranial nerve palsy and brainstem involvement appear around 4 days after onset, with rapid onset, rapid progression, and prone to respiratory failure, with extremely high disability and mortality rates
.
Early brain MRI can clearly show the lesions of brainstem encephalitis.
The low brainstem (pons and medulla), cerebellum and upper cervical spinal cord are most commonly involved.
Diffusion
.
Patchy, nodular, or annular enhancement may be present, the latter suggesting abscess formation
.
Case 4, a 41-year-old male patient, was admitted to the hospital because of poor speech for 16 hours and headache and numbness of the left upper limb for 3 hours
.
Answer: Posterior Reversible Encephalopathy Syndrome (PRES)
.
Admission blood pressure: 220/150mmHg, family history of hypertension
.
After 2 weeks, the brainstem lesions disappeared
.
Clinical symptoms of PRES include seizures, encephalopathy, headache, visual disturbances, etc.
The common causes are malignant hypertension, renal failure, use of cytotoxic drugs, autoimmune diseases, preeclampsia or eclampsia
.
The mechanism is that the acute blood pressure fluctuation or the direct action of cytokines on the vascular wall causes endothelial damage, causing the destruction of the blood-brain barrier, and then causing reversible vasogenic edema
.
PRES is divided into two types: typical PRES and variant PRES
.
Typical PRES include the following three types: superior frontal groove, full watershed, and parieto-occipital (common)
.
Variant PRES typically only involves the brainstem, cerebellum, and basal ganglia, while the fronto-parietal-occipital cortex and subcortical white matter are not involved
.
Partial involvement of the spinal cord is found in variant PRES, so it is called the reversible posterior encephalopathy syndrome with spinal cord involvement
.
Case A 553-year-old male was admitted to the hospital on October 17, 2018 due to "sudden right limb hemiplegia for 1 day"
.
Head MRI in November 2015 was as follows: Answer: Cerebral autosomal dominant arteriopathy with subcortical infarction and leukoencephalopathy (CADASIL) with cerebral hemorrhage
.
The patient's medical history of the first stroke (47 years old) can be traced back to 2012, and the earliest imaging can be traced back to 2013.
According to imaging, there were 3 cerebral infarctions, all of which were lacunar cerebral infarction
.
The patient's mother died after a stroke at the age of 60, and her mother and brother also died of a stroke at a younger age
.
The characteristics of the case: 1.
Young people with repeated attacks; 2.
Family history; 3.
Subcortical infarction + leukoencephalopathy + lobar hemorrhage + multiple microbleeds; 4.
Arg607Cys hybridization mutation detected in NOTCH3 exon 11
.
Non-sclerotic, non-amyloidotic, and medial smooth muscle cell degeneration with GOM deposition in arterioles distinguish CADASIL from other forms of small arterial disease.
These vascular changes can occur in the brain, kidney, spleen, liver, muscle, and skin
.
Case 6 patient, male, 67 years old, was admitted to hospital due to "sudden left limb movement disadvantage for more than 8 hours"
.
Head CT: Symmetrical calcifications in bilateral cerebellum, basal ganglia, gray-white matter junction, and left occipital cortex
.
Answer: Fahr's disease with cerebral hemorrhage
.
The patient's genetic examination revealed a mutation in the SLC20A2 gene
.
Fahr disease is also known as familial idiopathic cerebrovascular ferrocalcinosis
.
Bilateral symmetrical calcification of the basal ganglia caused by a variety of causes is called Fahr syndrome
.
Fahr disease is mostly autosomal dominant or recessive
.
A total of 4 pathogenic genes have been found so far: SLC20A2, PDGFRB, PDGFB and XPR1
.
Mutations in genes lead to impairment of intracellular and extracellular inorganic phosphorus transport and disruption of the blood-brain barrier
.
Diagnostic criteria for Fahr disease: (1) bilateral basal ganglia calcification on imaging; (2) no hypoparathyroidism; (3) normal serum calcium and phosphorus; (4) normal tubular response to parathyroid hormone ; (5) No infection, poisoning, metabolism and other reasons
.
The main clinical manifestations are progressive mental disorders, mental retardation, seizures, language disorders, and severe growth disorders
.
Laboratory tests for parathyroid hormone, serum calcium, and phosphorus were normal
.
Symmetrical calcifications in the brain are the main imaging findings, calcifications in the basal ganglia can be characterized by a "bird's dialogue" sign, and cerebellar dentate nucleus calcifications are generally crescent-shaped
.
In addition, there are many white matter demyelination and brain atrophy, which can be combined with cerebral hemorrhage and cystic degeneration
.
The atrophy mainly affects the white matter areas, resulting in enlargement of the ventricles
.
Differentiate from hypoparathyroidism
.
Case 7 was a male, 34 years old
.
Admitted to hospital for "7 hours of unconsciousness after drinking alcohol"
.
The patient had a history of heavy drinking for 4 years, about 2 catties of liquor per day
.
Answer: Marchiafava-Bignami disease (MBD)
.
Clinical manifestations: There is a long history of chronic alcoholism, and it is more common in middle-aged and elderly men
.
MBD is divided into two subtypes, A and
B.
Type A clinical symptoms are mainly coma
.
Type B presents with normal or mild disturbance of consciousness
.
According to the severity of onset, MBD can be divided into three types: acute, subacute and chronic
.
Acute phase: sudden onset, disturbance of consciousness, psychiatric symptoms, epileptic seizures (frequent or persistent), acute onset of unsteady gait and severe neurological deficit, among which epileptic seizures and acute onset of unsteady gait may be the first onset Symptoms, often fatal in a short period of time; subacute phase: ataxia, dysarthria, hypertonia, rapidly progressive dementia that can persist to chronic forms; chronic phase: dissociative syndrome (dextrose due to hydrazine callopathies) Left hand apraxia, inability of one hemisphere to respond to visual or somatosensory stimuli projected to the other) and progressive dementia
.
More MBD patients died 4 to 6 years after the disease
.
There are also reports in the literature that many acute and subacute patients can survive for a long time
.
The imaging manifestations of acute MBD are diffuse swelling of the corpus callosum, body and/or knee, iso or slightly hypointense signal on T1W1, high signal on T2W1 and FLAIR, high signal on DWI, and low signal on ADC.
Appears as round or irregular patchy enhancement
.
Case 8, a 63-year-old female, was first admitted to hospital on September 20, 2021, with slurred speech for 3 days, aggravation and left limb weakness for 7 hours
.
2021.
09.
21 D-dimer (DD) 10.
75mg/L, 2021.
09.
23 D-dimer (DD) 12.
67mg/L, 2021.
09.
28 D-dimer (DD) 19.
30mg/L
.
The patient was readmitted to the hospital on October 4, 2021 due to "slurred speech and left limb weakness for half a month, aggravating for 1 day"
.
2021.
10.
5 D-dimer (DD) 24.
56mg/L
.
Answer: Trusseau Syndrome
.
The patient had multiple metastases from cervical cancer
.
For unexplained markedly elevated D-dimer levels, even in the absence of clinical manifestations, the possibility of VTE should be considered; after excluding thrombotic diseases and liver and kidney diseases, the possibility of malignancy should be highly suspected
.
Case 9 is a 79-year-old male who was admitted to the hospital due to "memory loss for more than 20 days"
.
Past history of lung cancer
.
Answer: Brain metastases
.
Studies have shown that brain metastases calcifications are more common in lung adenocarcinoma (56%), followed by breast ductal invasive carcinoma (20%) and small cell lung cancer (11.
8%).
Calcification is multiple
.
Calcification types can be divided into punctate, nodular, annular, clustered, and large granular (below)
.
Most of the calcification types are punctate calcifications, and there is no specific correlation between the type and the original tumor histology
.
Case 10 female, 1 year and 3 months old, had severe motor and cognitive developmental delay after birth, frequent focal epilepsy after 10 months, progressive aggravation, head circumference gradually increased, and bone suture cracking in the later stage
.
Brain MRI and CT are as follows: Answer: GM1 gangliosidosis
.
GM1 gangliosidosis is a rare autosomal recessive genetic disease.
The main clinical features of patients are psychomotor regression, abnormal muscle strength, convulsions and other manifestations of central nervous system damage
.
The gene encoding β-galactosidase, GLB1, is a disease-causing gene.
Mutation of the gene can reduce the activity of β-galactosidase, leading to the degradation of GM1 gangliosides and accumulation in the body, resulting in a series of clinical manifestations
.
According to the age of onset and the severity of the disease, GM1 gangliosidosis can be divided into infantile (type I), juvenile (type II) and adult (type III)
.
Infant type, often onset within 6 months after birth, manifested as severe central nervous system, bone and other damage, extremely serious disease, more than 2 years of age before death; juvenile type, more than 7 months to 3 years after birth Onset, the course of the disease progresses slowly, often manifested as progressive psychomotor retardation, convulsions, abnormal muscle strength, and skeletal developmental abnormalities, but less severe than infantile type; adult type, the onset time span is longer, average 3-30 years old It can be onset, and the clinical manifestations vary greatly.
It can be mild dystonia and language impairment, or severe extrapyramidal damage and intellectual disability
.
Imaging features: On FLAIR/T2-weighted sequences, there are bilaterally symmetrical high-intensity signals in the basal ganglia, mainly in the putamen
.
Significant neuronal loss in the head of the putamen and caudate nucleus with secondary gliosis is thought to be responsible for the MRI findings
.
Case 11 was a male, one month old, with refractory hypoglycemia in the neonatal period
.
Answer: Pituitary Stem Block Syndrome (PSIS)
.
Pituitary stalk blocking syndrome refers to the clinical syndrome caused by the absence or obvious thinning of the pituitary stalk, combined with posterior pituitary ectopic, and the hormone secreted by the hypothalamus cannot be transported to the pituitary through the pituitary stalk, resulting in hypoplasia of the anterior pituitary
.
Most children with PSIS are diagnosed with delayed growth and development in childhood, delayed sexual development, or lack of development beyond puberty
.
The main manifestations of PSIS in the neonatal period are hypoglycemia and delayed resolution of jaundice caused by cholestasis
.
Neonatal hypoglycemia is not uncommon, the more common hypoglycemia caused by insufficient intake and hyperinsulinemia
.
Hypoglycemia occurs in the neonatal period.
If it is associated with other neurological manifestations, endocrine diseases should be considered in addition to ingestion, absorption, and metabolic diseases.
In addition to insulin and C-peptide, thyroid function and adrenal function should also be checked.
, Once multiple pituitary hormone deficiencies are found, pituitary disease should be considered
.
