Saturday, May 19, 2007

Listeria encephalitis

case discussion in Reviews in Neurological Diseases. The typical presentation is rhombencephalitis with a fever. It affects often pons and medulla in middle aged adults who are not immunocompromised. The prodrome is low grade fever, headache, encephalitis, vomiting and malaise lasting up to two weeks, followed by abrupt onset of cranial nerve palsies and encephalopathy. The cranial nerve palsies are often strikingly assymetric. The most common findings are dysphagia, dysphonia, dysarthria, facial weakness, diplopia and nystagmus. INO is described in those with a pontine abscess. Urinary retention is described due to involvement of Barrington's nucleus. CSF gram stain is often negative and diagnosis may require serologic tests. There is 75 % chance of survival if treatment is initiated promptly.

Saturday, May 05, 2007

Brain MRI findings in mitochondrial cytopathies

Barragan-Campos H et al. Arch Neurol 2005; 62:737-742. 21 patients were described, including 7 with CPEO, 7 with Kearns-Sayre, 6 with mitochondrial neurogastrointestinal encephalopathy, and 1 with myoclonic epilepsy with ragged red fiber myopathy. MRi showed widespread white matter hyperintensity in 90 %, supracortical atrophy in 18, cerebellar atrophy in 13 ABSENT BASAL GANGLIA HYPERINTENSITY CORRELATED WITH KEARNS SAYRE AND SUPRATENTORIAL ATROPHY WITH mitochondrial neurogastrointestinal encephalopathy.

Clinical criteria: CPEO-- ext opthalmopledia, proximal myopathy and RRF. KS-- ext opthalmoplegia, heart block, pigmentary retinopathy, cerebellar ataxia, eolevated CSF protein (the latter two were "optional"). MNGIE-- leukoencephalopathy, CPEO, PN, chronic intestinal pseudoobstruction MERRF- myoclonic epilepsy, myoclonic ataxia, dementia, and RRF.

Wednesday, May 02, 2007

Cryptococcus/fungals/meningoencephalitis pearls (Dr Perfect AAN 2007 lecture)

1.  Cryptococcus neoformans serotype A and D (grubii and neofomans) and serotype B and C (Cr. gattii) are major serotypes.  Gattii affects more normal hosts and has more torulomas. 
2.  Consider IRIS (immune reconstition syndrome) in HIV patients starting HAART or with any CHANGE in immunosuppressive regiment.  If patient seems to be getting worse in spite of treatment, this may be one of the few times to pay attention to the Crypto antigen titer.  If it is dropping the patient may have IRIS and may not be failing therapy. 
3.  Don't put a shunt in until the patient is already on treatment-- you'll just have to replace it later.  Increased ICP can occur on presentation chronic and indolent, acutely during early therapy, or classic hydrocephalus with therapy.  Consider increased ICP if frequent and severe headaches, pappilledema, hearing loss, or pathologic reflexes present.  Consider repeat LP's as a treatment modality.  Shunts don't work often in comatose patients.
4. Indications for surgery include diagnostic biopsy, toruloma greater than 3 mm, zygomycetes and infarcted tissue, phaeohyphomycosis, shunt placement.
5.  Diagnosis of other fungi clues: neutrophilic meningitis (aspergillus, scedosporium, blastomyces, zygomycetes), large volume CSF cultures (Blasto/histo/coccidio).  Histoplasmosis antigen, sporotrix CSF antibodies, cocciiodes CFA, aspergillus galactomannan.
6.  Wangiella (Exophiala) dermatitidis meningitis occurs due to injectable steroids that are contaminated, with a 1-11 month incubation period.  Therapy with voriconazole. 
7.  Hyalohyphomycosis-- occurs in near fresh water drownings, due to steroids, and diabetes, usually from sinus disease,  causes abscesses, surgery is critical for diagnosis and debulking. 
8.  Drug resistance is rare but it occurs.

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Leprosy Pearls-- lecture by Dr Swift at AAN 1007

1.  Most people (95 %) have immunity to leprosy and cannot get it.
2.  Leprosy is only infection that invades the peripheral nerve. Palpation of nerves is important.
3.  Invasion can take form of nodular or diffuse infiltration.
4.  Erythema nodosum leprosum is an immune reaction that occurs with treatment than can result in severe damage of skin, anterior eye, testes, and nerves and cause infarction/ulcers especially of skin. 
5.  The three types are tuberculous ( immune reaction present), lepromatous (immune reaction absent) and intermediate or borderline.  Borderline is unstable and tends towards lepromatous or tuberculous (reversal reaction).
6.  Signs include affected cool areas with involvement of upper lip (cooler than lower lip), tip of nose, ears, extensor surfaces of extremities, scrotum and testes, anterior third of eye.  May have sparing between toes (warmer).  Failure to close eyes results in corneal drying and lower lid hangs away (lagopthalmos).  Buccinator wrinkles since buccinator is deep.  Ulnar clawing, acute angle glaucoma, or corneal abrasions due to eyelids turning in.  Knee and ankle jerks are normal.  Scalp is only affected in bald patients. 
7.  Treatment of paucibacillary (tuberculoid)  leprosy is with a combination of rifamycin, ofloxacin, and minocycline (check standards which always change).   In paucibacillary intermediate disease use rifamycine 600 mg monthly and dapsone 100 mg daily for 6 months.  In multibacillary (lepromatous) use triple therapy with dapsone 100 mg/day, rifamycine 600 mg/mo, and clofazimine 50 mg per day for 12-14 months.  Also treat reactions with prednisone 60 mg/day or equivalent.  Rehab procedures of affected skin are helpful.  Patients are at high risk of osteomyelitis due to neuropathy.  Casting has been tried.   

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Cerebral Abscess-- Quick Hits from AAN (Bartt lecture) 2007

1.  Mortality has declined in recent years.  In a recent review of 94 patients admitted to intensive care, 26 % died and 36 % had long term morbidity.  Recent rates of death are 13-14 % in both children and adults.  Factors that predicted mortality (independently) were sepsis and GCS < 9 , not location, deficits, organism or need for surgery. (Tattevin et al., 2003, AM J MED).
2.  Resolution of ring enhancing MRI is longer than the six weeks needed to treat the abscess with intravenous antibiotics.
3.  The purpose of surgery is to treat mass effect.
4.  Do not treat mycotic aneurysms (or worry excessively about them) until 4-6 weeks of treatment has already occurred. 
5. Steroids can reduce antibiotic penetration through the blood brain barrier and are not proven in abscess.
6.  Risk factors for abscess 1.7 % for acute or chronic sinusitis (esp ethmoid or sphenoid), 1-2 % for HIV, 4 % for stem cell or bone marrow transplant,  2.1 % of cyanotic heart disease, and 3.1-37.5 for pulmonary AVM's (worse with multiple AVMS's). 
7.  Otitis is more common in young males as a cause.
8.  The most common cause is headache in 70-80 % and less than half have HA, fever and focal deficit.  Less than half have pappilledema.  Nausea, vomiting, pappilledema are more common in otogenic with symptoms in posterior fossa and of increased intracranial pressure.
9.  Sed rate and blood cultures may be positive in less than 20 % and especially if bacterial endocarditis is present.
10. Factors on imaging favoring abscess over tumor (metastasis) include a more regular rim, homogenous enhancement, a thinner wall towards the ventricular surface, daughter satellite lesions and RESTRICTED DIFFUSION ON DIFFUSION WEIGHTED SEQUENCES  all favor abscess. 
11.  Do a TEE to look for paradoxical abscess and PFO in patients with a cryptogenic abscess. 
12.  Therapy suggestions:
Immunocompromised with lobar pneumonia, TMP-SMX; antifungals if bug not identified.
Frontal  (sinus, odontogenic)-- aer/anaer-- PCN + Flagyl or 3rd gen ceph + Flagyl
Temporal (Otogenic) - Str, Entero, Bacter.-- PCN + Flagyl + 3rd gen ceph.
Metastatic/ hematogenous-- nafcillin, Flagyl, cefotaxime
Penetrating trauma/ postop--Steph, enterob, pse.-- vancomycin + ceftazadime

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