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Query: UMLS:C0042571 (vertigo)
 7,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Creutzfeldt-Jakob disease (CJD), the most common form of human prion diseases, is a fatal condition with a mortality rate reaching 85% within one year of clinical presentation. CJD is characterized by rapidly progressive neurological deterioration in combination with typical electroencephalography (EEG) and magnetic resonance imaging (MRI) findings and positive cerebrospinal spinal fluid (CSF) analysis for 14-3-3 proteins. Unfortunately, CJD can have atypical clinical and radiological presentation in approximately 10% of cases, thus making the diagnosis often challenging. We report a rare clinical presentation of sporadic CJD (sCJD) with combination of both expressive aphasia and nonconvulsive status epilepticus. This patient presented with slurred speech, confusion, myoclonus, headaches, and vertigo and succumbed to his disease within ten weeks of initial onset of his symptoms. He had a normal initial diagnostic workup, but subsequent workup initiated due to persistent clinical deterioration revealed CJD with typical MRI, EEG, and CSF findings. Other causes of rapidly progressive dementia and encephalopathy were ruled out. Though a rare condition, we recommend consideration of CJD on patients with expressive aphasia, progressive unexplained neurocognitive decline, and refractory epileptiform activity seen on EEG. Frequent reimaging (MRI, video EEGs) and CSF examination might help diagnose this fatal condition earlier.
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PMID:Creutzfeldt-Jakob Disease Presenting as Expressive Aphasia and Nonconvulsive Status Epilepticus. 2966 11

Creutzfeldt-Jakob disease (CJD) is known for a rapidly progressive decline in cognitive functions due to an underlying infection from a prion. This case involves a unique and atypical variant of CJD that was difficult to diagnose for the investigating medical teams. As such, it highlights the particular challenge of using traditional diagnostic algorithms for atypical variants of CJD, in terms of time-appropriate diagnostics. The patient presented with a sudden episode of vertigo which was treated as an isolated symptom. While succeeding investigations involving neurology and otolaryngology specialists were being carried out, the patient experienced progressively worsening and more frequent episodes of disequilibrium that required multiple visits to emergency care facilities. In such a facility, a repetitive battery of serum and cerebrospinal fluid testing confirmed the diagnosis of CJD. Subsequently, the patient was provided the necessary supportive care. While this case was successfully diagnosed, it showed that common presentations can have significant underlying neurological implications, and such atypical variants should be accounted for in traditional diagnostic algorithms. This can avoid unnecessary delays in therapeutic rehabilitation.
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PMID:An Atypical Presentation of Creutzfeldt-Jakob Disease With a Heidenhain Variant and Balint's Syndrome. 3267 46

BACKGROUND Creutzfeldt-Jakob disease (CJD) is a human prion disease characterized by severe and rapidly progressive fatal neurodegeneration. Currently, there is no cure for CJD, and death from CJD usually occurs within 1 year from the onset of the symptom, and the median survival time is 6 months. CASE REPORT The patient was a 63-year-old woman who presented to the hospital reporting having vertigo for the past 1 week and involuntary muscle contraction resulting in slow repetitive movement and abnormal posture for the past 3 days. A physical examination at the time of admission revealed unsteady gait, dystonia, and dysmetria of the left upper limb. There was no nystagmus on examination. Electroencephalography done on the same day showed focal epileptiform discharge on bilateral temporal lobes, which were more on the right side than the left. It also showed mild diffuse cerebral slowing. Cerebrospinal fluid analysis showed positive for RT-QulC, T tau protein, and 14-3-3. A diagnosis of CJD was made based on clinical course, imaging, and cerebrospinal fluid analysis. CONCLUSIONS The diagnosis of CJD can be suspected based on clinical signs and symptoms and can be confirmed after performing MRI, EEG, and lumbar puncture. Therefore, it is important to recognize vertigo as an unspecific symptom of CJD so that a timely diagnosis can be made and unnecessary procedures can be avoided.
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PMID:Cerebellar Vertigo as an Unspecific Initial Presentation of Creutzfeldt-Jakob Disease. 3322 15