Cerebral amyloid angiopathy related inflammation (CAA-I), previously described under various names, is a treatable encephalopathy usually occurring in older adults. Here, three patients are described with histopathologically confirmed CAA-I, and summarised data from the published literature are presented. CAA-I has a characteristic combination of clinical and radiological features. Definite diagnosis requires brain and leptomeningeal biopsy. A favourable response to immunosuppressive therapy is common and treatment without brain biopsy may be considered in selected patients. Diagnostic criteria for CAA-I are proposed.
Objectives-The frequency and characteristics of the long term sequelae of herpes simplex encephalitis were assessed after treatment with acyclovir. Methods-Patients were included if they were treated with acyclovir and the diagnosis of herpes simplex encephalitis was confirmed by culture of herpes simplex virus (HSV) from the brain, an increase in the CSF HSV antibody titre, or detection of HSV deoxyribonucleic acid in the CSF. Each patient's medical records were reviewed and surviving patients were interviewed and examined. Results-A diagnosis of herpes simplex encephalitis was confirmed in 42 patients. Five patients (12%) died in the first month. Three patients (7%) had severe neurological sequelae and died after a longer interval. All but one of the 34 surviving patients had neurological symptoms, an abnormal neurological examination, or both. Twenty patients (48%) performed everyday activities as well as before herpes simplex encephalitis; nine patients (21%) were living independently, but were functioning at a lower level than before the illness; and five patients (12%) had a severe neurological deficit. Twenty nine of the 34 survivors were assessed six months to 11 years after herpes simplex encephalitis. The most common long term symptoms were memory impairment (69%), personality and behavioural abnormalities (45%), and epilepsy (24%). Short term memory impairment (70%), anosmia (65%), and dysphasia (41%) were the most common signs. Conclusions-Although acyclovir has reduced the mortality of herpes simplex encephalitis, 30% of this group of patients either died or had a severe neurological deficit. The other 70% of the patients regained independence in activities of daily living, but most of these people had persistent neurological symptoms, signs, or both.
Abducens palsy occasionally has been observed after diagnostic lumbar puncture (DLP). 1-3 Its risk is not exactly known. We are aware of only one report, which mentions not a single case among 1,341 DLPs when using 22-gauge needles. 4 At our clinic, an average of 800 inpatients a year undergo DLP. We usually use 22gauge needles, and occasionally (in less than 5%), 20-gauge needles. Over a 14.5-year period, we saw two patients with abducens palsy-one unilateral, one bilateral-after DLP. This translates to a risk of less than 1 out of 5,800 DLPs.Patient reports. Patient 1. A 61-year-old man with type 2 diabetes noticed progressive weakness of the legs. Neurologic examination revealed proximal paraparesis, loss of deep tendon reflexes of the legs, diminished sensation to touch and pain in a stocking-like pattern, and diminished vibration sense. Nerve conduction studies documented sensorimotor demyelinating neuropathy of the legs and, less pronounced, of the arms. DLP was done with a 22-gauge needle and was not followed by postlumbar puncture headache. After 7 days, he noticed horizontal diplopia when looking to the right. There was a right-sided abducens palsy. MRI documented small bifrontal and parietal hygromas and diffuse meningeal gadolinium enhancement (figure). Abducens palsy recovered completely within 4 months.Patient 2. A 33-year-old man with type 2 diabetes noticed exercise-related pain in the calf and thigh muscles (finally attributed to a myoadenylate deaminase deficiency). Neurologic examination revealed bilateral loss of ankle jerks and diminished sensation to touch and pain in a stocking-like pattern. Nerve conduction studies documented sensory demyelinating neuropathy of the legs and, less pronounced, of the arms. After DLP with a 22-gauge needle, he developed mild postlumbar puncture headache recovering within 7 days. Two days later (9 days after DLP), he noticed horizontal diplopia when looking to the left and right. There was a bilateral abducens palsy. MRI documented diffuse meningeal gadolinium enhancement. Abducens palsies completely recovered within 7 months.Discussion. Sixth nerve palsy after DLP is attributed to continuous CSF leakage through the dural hole. 2,5 This results in intracranial hypotension with MRI-documented descent of the brain even in the supine position, causing traction of the VIth nerve and pain-sensitive structures (dura, blood vessels, nerves). 5 This also explains orthostatic headache, which is usually associated with post-DLP abducens palsies. 1,2,5 The needle size seems to be crucial for both post-DLP abducens From the Departments of Neurology (Drs. Thömke, Mika-Grü ttner, and Visbeck) and Neuroradiology (Dr. Brü hl),
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