SUNCT syndrome: a possible bilateral case responding to topiramate.
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Adult↗
Biomedical subjects
Jens Kuhn
Publications and source records attributed to Jens Kuhn.
[Atypical headache and facial pain as a result of hypertrophic pachymeningitis in C-ANCA-positive Wegener's granulomatosis].
BACKGROUND: Wegener's granulomatosis (WG) is a systemic vasculitis involving the nervous system in 20-54% of cases; lesions of peripheral nerves are commonest, while manifestation in the central nervous system (CNS) is rarer. Focal hypertrophic pachymeningitis is a very rare complication of WG. This inflammatory thickening and fibrosis of the dura mater is always associated with headaches, whereas cranial nerve lesions, cerebellar symptoms or epileptic seizures occur more rarely. CASE REPORT: A 67-year-old patient, in whom WG had been diagnosed 2 years earlier and who had been treated with immunosuppressants since then, complained of continuous severe, mainly left-sided headache and facial pain for weeks. Cranial MRI showed thickening of the left tentorium cerebelli with obvious contrast enhancement and led to the diagnosis of hypertrophic pachymeningitis. The inflammatory parameters and the C-ANCA (antineutrophil cytoplasmic antibodies) in the serum were raised and CANCA were detectable in the cerebrospinal fluid. The headaches subsided with several days of intravenous high-dose corticosteroids and a simultaneous increase in the immunosuppressive basic medication. On a follow-up MRI after 3 months, the magnetic resonance changes were less apparent, i. e., the hypertrophic pachymeningitis was resolving; C-ANCA were now no longer detectable in the cerebrospinal fluid. CONCLUSION: With newly occurring, unusually severe and persistent headaches in the presence of WG, the very rare complication of hypertrophic pachymeningitis should be considered.
Adrenal Cortex Hormones↗
Extrapyramidal and cerebellar movement disorder in association with heterozygous ceruloplasmin gene mutation.
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Adolescent↗
[Central pontine myelinolysis with a hyperintense lesion in diffusion weighted MRI: overview by means of a case report].
Central pontine myelinolysis (CPM) is a demyelinating disease of the pons often associated with demyelination of other areas of the central nervous system (CNS). The etiology and pathogenesis of this disorder are still not fully understood. However, almost all cases of CPM are related to severe diseases or chronic alcoholism and occur in the setting of rapidly corrected serum hyponatremia and hypotonicity respectively. Depending on the involvement of other CNS structures, the clinical picture can vary considerably. Magnetic resonance imaging is the most sensitive investigation for the antemortem diagnosis of CPM, although the radiological findings lag behind and do not necessarily correlate with the clinical picture. Quite obviously diffusion-weighted imaging can be useful in the rapid diagnosis of CPM. This short review summarizes the current knowledge on the pathogenesis, clinical presentation, radiological findings, prognosis and therapeutic approaches of CPM. Characteristical clinical features and MR-findings including hyperintensity on diffusion-weighted images are illustrated by a typical case.
Diffusion Magnetic Resonance Imaging↗
Remitting form of hemicrania continua: two new cases exhibiting one unusual autonomic feature.
Hemicrania continua (HC) is an uncommon, primary headache disorder characterized by a continuous, strictly unilateral headache of moderate intensity with superimposed exacerbations of more severe pain and accompanying autonomic features. Whereas in the majority of HC patients the headache is continuous and without pain-free intervals, we report two new cases of the rare remitting form of HC. One patient described unusual olfactory sensation during episodes of exacerbation, which we classify as an olfactory aura.
Adult↗
[Herpes simplex virus type 1 encephalitis].
BACKGROUND: Herpes simplex virus type 1 encephalitis (HSE) is the most frequent cause of severe sporadic viral encephalitis. In spite of the advanced modern methods for the treatment of HSE it is still associated with high mortality. Early diagnosis and prompt antiviral therapy with acyclovir are essential to improve prognosis and to prevent severe neurologic sequels. PURPOSE: This review summarizes the current knowledge on the pathogenesis, diagnosis, clinical presentation, and treatment of herpes simplex virus type 1 encephalitis. Characteristic features including magnetic resonance imaging (MRI) are illustrated by a typical case.
Acyclovir↗
[Multiple cerebral cavernous malformations: typical pattern on MR imaging and appearence of a new lesion in the follow-up MRI].
Cerebral cavernous malformations (CCM) are common, mostly benign vascular anomalies of the CNS. Clinical features are seizures, headache and focal neurological signs. Often they are asymptomatic. Apart from sporadically cases CCM occur as an autosomal dominant condition. Familial cases are associated with a high frequency of multiple lesions. MRI is most sensitive in the detection of cavernous malformations. The MRI findings of CCM are variable, depending on hemorrhage and calcifications. The typical appearence of CCM are heterogenous "popcorn-like" lesions of different size with a mixed signal core and a hypointense hemosiderin rim. Our report concerns a 22 year old man with multiple cerebral cavernous malformations whose follow-up MRI of brain showed the appearence of a new lesion.
Adult↗
Genetic variability of Crimean-Congo haemorrhagic fever virus in Russia and Central Asia.
Hyalomma marginatum ticks (449 pools, 4787 ticks in total) collected in European Russia and Dermacentor niveus ticks (100 pools, 1100 ticks in total) collected in Kazakhstan were screened by ELISA for the presence of Crimean-Congo haemorrhagic fever virus (CCHFV). Virus antigen was found in 10.2 and 3.0 % of the pools, respectively. RT-PCR was used to recover partial sequences of the CCHFV small (S) genome segment from seven pools of antigen-positive H. marginatum ticks, one pool of D. niveus ticks, four CCFH cases and four laboratory virus strains. Additionally, the entire S genome segments of the CCHFV strains STV/HU29223 (isolated from a patient in European Russia) and TI10145 (isolated from H. asiaticum in Uzbekistan) were amplified, cloned and sequenced. Phylogenetic analysis placed all CCHFV sequences from Russia in a single, well-supported clade (nucleotide sequence diversity up to 3.2 %). Virus sequences from H. marginatum were closely related or identical to those recovered from patients in the same regions of southern Russia. Newly described CCHFV strains from Central Asian countries fell into two genetic lineages. The first lineage was novel and included closely related virus sequences from Kazakhstan and Tajikistan (nucleotide sequence diversity up to 3.2 %). In contrast, a newly described CCHFV strain from Uzbekistan, strain TI10145, clustered on the phylogenetic trees with strains from China.
Animals↗
Focal hypertrophic pachymeningitis in association with temporal arteritis.
We present an extremely rare case of a patient with focal hypertrophic pachymeningitis in association with histologically proven temporal arteritis (giant cell arteritis). It is proposed that focal hypertrophic pachymeningitis in itself is the contributing factor to severe headache rather than temporal arteritis alone.
Aged↗