ABSTRACT
A young patient with PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome developed an unusual cerebral arterial vasculopathy/vasculitis (CAV) that resulted in subarachnoid hemorrhage from a ruptured dissecting posterior cerebral artery (PCA) aneurysm. This aneurysm was successfully treated by endovascular coil sacrifice of the affected segment of the PCA. The patient made an excellent recovery with no significant residual neurologic deficit.
Subject(s)
Acne Vulgaris/diagnostic imaging , Aortic Dissection/diagnostic imaging , Arthritis, Infectious/diagnostic imaging , Intracranial Aneurysm/diagnostic imaging , Posterior Cerebral Artery/diagnostic imaging , Pyoderma Gangrenosum/diagnostic imaging , Vasculitis, Central Nervous System/diagnostic imaging , Acne Vulgaris/surgery , Aortic Dissection/surgery , Arthritis, Infectious/surgery , Cerebral Angiography , Diagnosis, Differential , Endovascular Procedures/methods , Humans , Intracranial Aneurysm/surgery , Magnetic Resonance Angiography , Methicillin-Resistant Staphylococcus aureus , Posterior Cerebral Artery/surgery , Pyoderma Gangrenosum/surgery , Staphylococcal Infections/complications , Subarachnoid Hemorrhage/diagnostic imaging , Subarachnoid Hemorrhage/surgery , Treatment Outcome , Vasculitis, Central Nervous System/surgeryABSTRACT
A young patient with PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome developed an unusual cerebral arterial vasculopathy/vasculitis (CAV) that resulted in subarachnoid hemorrhage from a ruptured dissecting posterior cerebral artery (PCA) aneurysm. This aneurysm was successfully treated by endovascular coil sacrifice of the affected segment of the PCA. The patient made an excellent recovery with no significant residual neurologic deficit.
Subject(s)
Acne Vulgaris/pathology , Aneurysm, Ruptured/pathology , Aortic Dissection/pathology , Arthritis, Infectious/pathology , Intracranial Aneurysm/pathology , Methicillin-Resistant Staphylococcus aureus/isolation & purification , Pyoderma Gangrenosum/pathology , Staphylococcal Infections/pathology , Vasculitis/etiology , Acne Vulgaris/complications , Aortic Dissection/diagnostic imaging , Aneurysm, Ruptured/surgery , Anti-Bacterial Agents/therapeutic use , Arthritis, Infectious/complications , Cefazolin/therapeutic use , Cerebral Angiography , Humans , Intracranial Aneurysm/diagnostic imaging , Methylprednisolone/therapeutic use , Pyoderma Gangrenosum/complications , Staphylococcal Infections/complications , Staphylococcal Infections/diagnostic imaging , Treatment Outcome , Vasculitis/microbiologyABSTRACT
OBJECTIVE: The purpose of this study was to review the experience with herpes simplex encephalitis at the Hospital for Sick Children over the past 12 years. METHODS: All patients who were admitted to our institution with acute encephalitis between January 1994 and December 2005 were enrolled prospectively in an encephalitis registry. Children from the registry with herpes simplex encephalitis were included in this study; we detailed the clinical presentations, laboratory findings, electroencephalographic findings, diagnostic imaging findings, treatments, and outcomes for all cases. RESULTS: Of 322 cases of acute encephalitis, 5% were caused by herpes simplex virus. Initially negative herpes simplex virus cerebrospinal fluid polymerase chain reaction results were found in 2 cases (13%), but results became positive in repeat cerebrospinal fluid analyses. Classic clinical presentations were seen in 75% of cases, cerebrospinal fluid pleocytosis was found in 94%, elevated cerebrospinal fluid protein levels were found in 50%, electroencephalographic changes were observed in 94%, and diagnostic imaging abnormalities were noted in 88%. All patients were treated with intravenous acyclovir. Neurologic sequelae occurred in 63% of cases, including seizures in 44% and developmental delays in 25%. There were no deaths in this study group. CONCLUSIONS: Herpes simplex encephalitis continues to be associated with poor long-term neurologic outcomes despite appropriate therapy. Cerebrospinal fluid polymerase chain reaction results may be negative early in the course of herpes simplex encephalitis; therefore, repeat cerebrospinal fluid analysis should be considered if herpes simplex encephalitis is suspected. Atypical forms of herpes simplex virus central nervous system disease may occur in children.
