-
Categories
-
Pharmaceutical Intermediates
-
Active Pharmaceutical Ingredients
-
Food Additives
- Industrial Coatings
- Agrochemicals
- Dyes and Pigments
- Surfactant
- Flavors and Fragrances
- Chemical Reagents
- Catalyst and Auxiliary
- Natural Products
- Inorganic Chemistry
-
Organic Chemistry
-
Biochemical Engineering
- Analytical Chemistry
-
Cosmetic Ingredient
- Water Treatment Chemical
-
Pharmaceutical Intermediates
Promotion
ECHEMI Mall
Wholesale
Weekly Price
Exhibition
News
-
Trade Service
Spontaneous intracranial hypotension (SIH) is a rare and often missed disease, usually caused by cerebrospinal fluid leakage
.
The typical clinical manifestations of SIH are postural headache and dizziness
.
Less common complications include nausea, neck stiffness, and even coma
.
This article introduces a case where no specific cerebrospinal fluid leakage was found, but a case of SIH is highly suspected
.
The symptoms of this case were completely reversed after 2 months of steroid treatment
.
By understanding this case, it is believed that neurologists can be aware of this diagnosis when evaluating patients with other unexplainable neurological signs and symptoms
.
Yimaitong compiles and organizes, please do not reprint without authorization
.
A 70-year-old female, a 70-year-old female with sudden headache, dysarthria and cognitive decline, presented to the doctor with a 10-day history of severe headache, without related symptoms, and no history of head trauma
.
The patient had similar headaches in the past, but the duration was shorter and the intensity was lower
.
The headache happened suddenly, when she leaned forward or stood up from the lying position
.
The patient described the headache as a strong feeling of stress, and over-the-counter analgesics were ineffective
.
A few weeks after the onset of headache, the patient began to experience sleep attacks, and even fell asleep while standing during the conversation
.
Since the multiple sleep latency test (MSLT) is normal, the possibility of narcolepsy is ruled out
.
In addition, sleep studies have shown mild sleep apnea, although after 6 months of continuous positive pressure ventilation (CPAP), the patient’s sleep symptoms have not improved
.
During the next 6 months, the patient developed severe dysarthria
.
Further examination revealed that the patient's EEG was normal, and MRI of the brain showed mild atrophy of the frontotemporal lobe (Figure 1)
.
Based on these findings, the patient was diagnosed with frontotemporal dementia (FTD)
.
Figure 1 The sagittal (A) and axial (B) T2-weighted MRI images before treatment show prolapse, tonsil hernia, diffuse brain swelling, and venous sinus congestion
.
About 1 year after the onset of symptoms, the patient underwent a brain MRI scan again.
This time the patient was diagnosed with narcolepsy and started taking 100 mg modafinil tablets
.
At the same time, the patient also went to a psychiatrist who prescribed donepezil to treat mild cognitive impairment
.
The patient's speech returned to normal, and his sleep episodes subsided completely
.
Unfortunately, all the patient's symptoms reappeared 3 days after starting treatment
.
About 1 month later, the patient developed urinary incontinence
.
In the next 3 months, the patient developed other symptoms, such as impulsive and inappropriate behaviors such as not paying attention to personal hygiene, eating fruits in the supermarket without paying, and eating with hands
.
At the same time, she also experienced memory problems, involuntary dance movements, such as blinking, stretching the neck while talking, mouth movements, and tapping fingers (not tremors)
.
As new symptoms appeared, the patient underwent immunological examinations, electroencephalograms, brain MRI scans, and neuropsychological evaluations
.
Based on the results of the examination, the patient was diagnosed with FTD behavioral variation
.
The patient developed a peak of symptoms about 18 months after the onset of symptoms, with difficulty in expression and dysarthria
.
The simple mental state score is 20/30, and it has defects in time orientation, short-term memory, calculation, repetition, and copying
.
During the passive movement, the right upper limb had mild spasm, but the rest of the neurological examination was normal
.
A retrospective summary of cranial MRI scans (Figure 1A, B) revealed diffuse dural gadolinium enhancement and brain stem prolapse, which is consistent with intracranial hypotension
.
MRI scan of the whole spine showed 30 perineural cysts in each segment of the spine, but no cerebrospinal fluid leakage
.
Consider epidural blood patch therapy.
However, patients and their families hope to try conservative treatment with tapered steroids
.
Four weeks after starting steroid therapy, the patient's cognitive dysfunction and speech difficulties began to improve
.
After six weeks of treatment, the patient is able to perform activities of daily living, such as cooking and washing dishes
.
After 8 weeks of treatment, the patient's urinary incontinence and fecal incontinence disappeared completely
.
Re-examination of the brain MRI at the end of the 4th month showed that the swelling of the brain was reduced, the congestion of the venous sinuses was reduced, and the herniation of the tonsils subsided (Figure 2A, B)
.
Figure 2 Sagittal (A) and axial (B) T2-weighted MRI images after treatment show that the symptoms of intracranial hypotension have almost completely resolved
.
Discussion The typical characteristics of SIH include paroxysmal postural headaches that appear or worsen when erect, dizziness, diffuse dural enhancement and "ptosis" on MRI, and low pressure to open the cerebrospinal fluid during lumbar puncture
.
As the volume of cerebrospinal fluid decreases, compensatory vascular congestion and interdural edema maintain dynamic balance
.
Although these classic features help diagnose most SIH patients, more and more patients either do not meet the classic diagnostic criteria, or have unusual manifestations, such as taste changes, limb paresthesia, behavior changes, and Parkinson-like symptoms
.
The rare complications of SIH include reversible frontotemporal dementia, Parkinson-like symptoms, quadriplegia, coma, and even death
.
More and more patients diagnosed with SIH are also accompanied by behavioral changes diagnosed with frontotemporal dementia
.
The patient in this case did not meet the typical manifestations of SIH.
The patient presented with progressive cognitive deficits, increased lethargy, dysarthria, chorea, and urinary incontinence
.
Although some previous cases have reported FTD-like symptoms in SIH patients, the reported symptoms are not as extensive as the symptoms in this case, and no sleep episodes or speech-related problems have been mentioned in any previous reports
.
In summary, the clinical manifestations of SIH are not yet fully understood.
When patients have multiple neurological symptoms that cannot be explained by other reasons, SIH should be included in the differential diagnosis
.
Yimaitong compiled from: Ozyigit A, Michaelides C, Natsiopoulos K.
Spontaneous Intracranial Hypotension Presenting With FrontotemporalDementia: A Case Report.
Front Neurol.
2018;9:673.
.
The typical clinical manifestations of SIH are postural headache and dizziness
.
Less common complications include nausea, neck stiffness, and even coma
.
This article introduces a case where no specific cerebrospinal fluid leakage was found, but a case of SIH is highly suspected
.
The symptoms of this case were completely reversed after 2 months of steroid treatment
.
By understanding this case, it is believed that neurologists can be aware of this diagnosis when evaluating patients with other unexplainable neurological signs and symptoms
.
Yimaitong compiles and organizes, please do not reprint without authorization
.
A 70-year-old female, a 70-year-old female with sudden headache, dysarthria and cognitive decline, presented to the doctor with a 10-day history of severe headache, without related symptoms, and no history of head trauma
.
The patient had similar headaches in the past, but the duration was shorter and the intensity was lower
.
The headache happened suddenly, when she leaned forward or stood up from the lying position
.
The patient described the headache as a strong feeling of stress, and over-the-counter analgesics were ineffective
.
A few weeks after the onset of headache, the patient began to experience sleep attacks, and even fell asleep while standing during the conversation
.
Since the multiple sleep latency test (MSLT) is normal, the possibility of narcolepsy is ruled out
.
In addition, sleep studies have shown mild sleep apnea, although after 6 months of continuous positive pressure ventilation (CPAP), the patient’s sleep symptoms have not improved
.
During the next 6 months, the patient developed severe dysarthria
.
Further examination revealed that the patient's EEG was normal, and MRI of the brain showed mild atrophy of the frontotemporal lobe (Figure 1)
.
Based on these findings, the patient was diagnosed with frontotemporal dementia (FTD)
.
Figure 1 The sagittal (A) and axial (B) T2-weighted MRI images before treatment show prolapse, tonsil hernia, diffuse brain swelling, and venous sinus congestion
.
About 1 year after the onset of symptoms, the patient underwent a brain MRI scan again.
This time the patient was diagnosed with narcolepsy and started taking 100 mg modafinil tablets
.
At the same time, the patient also went to a psychiatrist who prescribed donepezil to treat mild cognitive impairment
.
The patient's speech returned to normal, and his sleep episodes subsided completely
.
Unfortunately, all the patient's symptoms reappeared 3 days after starting treatment
.
About 1 month later, the patient developed urinary incontinence
.
In the next 3 months, the patient developed other symptoms, such as impulsive and inappropriate behaviors such as not paying attention to personal hygiene, eating fruits in the supermarket without paying, and eating with hands
.
At the same time, she also experienced memory problems, involuntary dance movements, such as blinking, stretching the neck while talking, mouth movements, and tapping fingers (not tremors)
.
As new symptoms appeared, the patient underwent immunological examinations, electroencephalograms, brain MRI scans, and neuropsychological evaluations
.
Based on the results of the examination, the patient was diagnosed with FTD behavioral variation
.
The patient developed a peak of symptoms about 18 months after the onset of symptoms, with difficulty in expression and dysarthria
.
The simple mental state score is 20/30, and it has defects in time orientation, short-term memory, calculation, repetition, and copying
.
During the passive movement, the right upper limb had mild spasm, but the rest of the neurological examination was normal
.
A retrospective summary of cranial MRI scans (Figure 1A, B) revealed diffuse dural gadolinium enhancement and brain stem prolapse, which is consistent with intracranial hypotension
.
MRI scan of the whole spine showed 30 perineural cysts in each segment of the spine, but no cerebrospinal fluid leakage
.
Consider epidural blood patch therapy.
However, patients and their families hope to try conservative treatment with tapered steroids
.
Four weeks after starting steroid therapy, the patient's cognitive dysfunction and speech difficulties began to improve
.
After six weeks of treatment, the patient is able to perform activities of daily living, such as cooking and washing dishes
.
After 8 weeks of treatment, the patient's urinary incontinence and fecal incontinence disappeared completely
.
Re-examination of the brain MRI at the end of the 4th month showed that the swelling of the brain was reduced, the congestion of the venous sinuses was reduced, and the herniation of the tonsils subsided (Figure 2A, B)
.
Figure 2 Sagittal (A) and axial (B) T2-weighted MRI images after treatment show that the symptoms of intracranial hypotension have almost completely resolved
.
Discussion The typical characteristics of SIH include paroxysmal postural headaches that appear or worsen when erect, dizziness, diffuse dural enhancement and "ptosis" on MRI, and low pressure to open the cerebrospinal fluid during lumbar puncture
.
As the volume of cerebrospinal fluid decreases, compensatory vascular congestion and interdural edema maintain dynamic balance
.
Although these classic features help diagnose most SIH patients, more and more patients either do not meet the classic diagnostic criteria, or have unusual manifestations, such as taste changes, limb paresthesia, behavior changes, and Parkinson-like symptoms
.
The rare complications of SIH include reversible frontotemporal dementia, Parkinson-like symptoms, quadriplegia, coma, and even death
.
More and more patients diagnosed with SIH are also accompanied by behavioral changes diagnosed with frontotemporal dementia
.
The patient in this case did not meet the typical manifestations of SIH.
The patient presented with progressive cognitive deficits, increased lethargy, dysarthria, chorea, and urinary incontinence
.
Although some previous cases have reported FTD-like symptoms in SIH patients, the reported symptoms are not as extensive as the symptoms in this case, and no sleep episodes or speech-related problems have been mentioned in any previous reports
.
In summary, the clinical manifestations of SIH are not yet fully understood.
When patients have multiple neurological symptoms that cannot be explained by other reasons, SIH should be included in the differential diagnosis
.
Yimaitong compiled from: Ozyigit A, Michaelides C, Natsiopoulos K.
Spontaneous Intracranial Hypotension Presenting With FrontotemporalDementia: A Case Report.
Front Neurol.
2018;9:673.
