NEJM Case records 2009 Case 5 2009 A 47 year old woman with a rash and numbness and pain in the legs. 360:711-720
Also see post on livedo reticularis and neurologic disease: http://strokenotes.blogspot.com/2007/02/livedo-reticularis-and-neurologic.html
Case: 47 year old woman developed slowly progressive numbness then pain in LE's, swelling, mottled discoloration, swelling and blanching nodules. Rheum screen was normal. Biopsy of TA and nodule showed lymphistiocytic infiltrate but not vasculitis. family history positive for Sjogren's ( mother, with AI hepatitis) and "vasculitis" (sister). She atrophie blanche and cytopenias as well.
Teaching Points
1. MMN--with the other findings point to SLE, Sjogren's, APL syndrome, cutaneous PAN, or livedoid vasculopathy. Second tier ddx includes cryoglobulinemia, sarcoid, DM, amyloid, neoplasms and infections.
2. 50 % SLE patients have rash before meeting criteria for SLE. LRET and nodules is enough to count. Small vessel vasculitis characterized by palpable purpura is commonest, but medium size vasculitis with livedo reticularis also occurs. Patients with SLE and medium sized vasculitis have more MMN, visceral vasculitis, and ischemic cutaneous lesions, but less malar rash and discoid lesions than patients without medium sized vasculitis.
3. ANA negative SLE is rare since introduction of testing with HEp-2 cell substrate (10 %) and this patient initially had a FALSE negative ANA due to wrong method for test being performed by the commercial lab. Occassional patients who are ANA negative are anti Ro *SSA) positive or have secondary APL syndrome. Repeat testing showed positive 1:1280 ANA.
4. APL syndrome may be associated with thrombocytopenia and leuokopenia and be primary or secondary. Patient was initially negative, then positive.
5. Cutaneous PAN is challenging since there are no antibodies that are distinctive. Typically one sees elevation of acute phase reactants and anemia which is not the presentation here.
6. The patient's biopsy showed livedoid vasculopathy, she was treated with ASA and plaquenil with warfarin in reserve and she did well.
Saturday, February 21, 2009
Wednesday, January 28, 2009
Styloid syncope
Neuroimages p 1649 Neurology71 2008
Patient had craniocervical pain and 30 years of syncope preceded by neck flexion and brief vertigo. 3D CT showed hypertrophied styloid process.
Eagle syndrome is characterized by a long SP with craniocervical or throat pain. Elongate SP occurs in 4 % of population. Syncope is due to compression of carotid artery. It should be considered in young patients with chronic throat pain and syncope provoked by neck flexion. It is diagnosed with 3d Ct and cured with styloidectomy.
foreign language ictal speech automatisms
Neurology 2008; 71: 1579-1585
Most cases reflect right TLE. However, FLISA occur with spread to temporal neocortex of frontal operculum. Authors suggest a male preponderance although this blogger's only personal observation is a woman.
Tuesday, January 20, 2009
Brain calcinosis syndrome
ref Baba et al. Heredofamilial brain calcinosis syndrome Mayo Clin Proc 2005;80:641-651
(a little dry for this blog)
List of causes:
80+% clinically benign
sporadic or familial I With abnormal calcium or magnesium metabolism
Hypoparathyroidism (idiopathic, postsurgical, external radiation, hypomagnesemia)
Pseudohypoparathyroidism (type II, idiopathic)
Hyperparathyroidism
II Without Ca/Mg abnormalities
Down's syndrome
Mitochondrial neuromyopathy (Kearns-Sayre syndrome, Pearson s.)
SLE
ALL
IgG kappa M proteinemia
Revesz syndrome
infectious (toxopl, mumps, EBV, CMV, HIV)
postanoxic
angiomatous malformations of the vein of Galen
Toxic (lead, CO)
Therapy induced ( XRT, anticancer drugs, mineralizing microangiopathy)
Without systemic involvement
Diffuse NFT with calcification
hyperkinetic mutism
idiopathic (Fahr's dis, striatopalidodentate calcinosis, bg calcification)
Aging
Genetic syndromes with chromosome identified (see reference for actual gene loci)
Familial isolated hypoparathyroidism
Autoimmune polyglandular syndrome
Pseudohypoparathyroidism types Ia and Ib
Aicardi syndrome (in first year of life, may see calcium in thalamus, cerebellum also)
Dihydropterine reductase syndrome (=PKU type 2 atypical form)-- may be arrested by folic
Cockayne syndrome
Krabbe
MELAS
Others (see text)
(a little dry for this blog)
List of causes:
80+% clinically benign
sporadic or familial I With abnormal calcium or magnesium metabolism
Hypoparathyroidism (idiopathic, postsurgical, external radiation, hypomagnesemia)
Pseudohypoparathyroidism (type II, idiopathic)
Hyperparathyroidism
II Without Ca/Mg abnormalities
Down's syndrome
Mitochondrial neuromyopathy (Kearns-Sayre syndrome, Pearson s.)
SLE
ALL
IgG kappa M proteinemia
Revesz syndrome
infectious (toxopl, mumps, EBV, CMV, HIV)
postanoxic
angiomatous malformations of the vein of Galen
Toxic (lead, CO)
Therapy induced ( XRT, anticancer drugs, mineralizing microangiopathy)
Without systemic involvement
Diffuse NFT with calcification
hyperkinetic mutism
idiopathic (Fahr's dis, striatopalidodentate calcinosis, bg calcification)
Aging
Genetic syndromes with chromosome identified (see reference for actual gene loci)
Familial isolated hypoparathyroidism
Autoimmune polyglandular syndrome
Pseudohypoparathyroidism types Ia and Ib
Aicardi syndrome (in first year of life, may see calcium in thalamus, cerebellum also)
Dihydropterine reductase syndrome (=PKU type 2 atypical form)-- may be arrested by folic
Cockayne syndrome
Krabbe
MELAS
Others (see text)
Tuesday, January 13, 2009
localizing signs with seizures
Clinical lateralizing signs are the phenomena which can unequivocally refer to the hemispheric onset of epileptic seizures. They can improve the localization of epileptogenic zone during presurgical evaluation, moreover, their presence can predict a success of surgical treatment. Primary sensory phenomena such as visual aura in one half of the field of vision or unilateral ictal somatosensory sensation always appear on the contralateral to the focus. Periictal unilateral headache, although it is an infrequent symptom, is usually an ipsilateral sign. Primary motor phenomena like epileptic clonic, tonic movements, the version of head ubiquitously appear contralateral to the epileptogenic zone. Very useful lateralization sign is the ictal hand-dystonia which lateralizes to the contralateral hemisphere in nearly 100%. The last clonus of the secondarily generalized tonic-clonic seizure lateralizes to the ipsilateral hemisphere in 85%. The fast component of ictal nystagmus appears in nearly 100% on the contralateral side of the epileptic focus. Vegetative symptoms during seizures arising from temporal lobe such as spitting, nausea, vomiting, urinary urge are typical for seizures originating from non-dominant (right) hemisphere. Ictal pallor and cold shivers are dominant hemispheric lateralization signs. Postictal unilateral nose wiping refers to the ipsilateral hemispheric focus compared to the wiping hand. Ictal or postictal aphasia refers to seizure arising from dominant hemisphere. Intelligable speech during complex partial seizures appears in non-dominant seizures. Automatism with preserved consciousness refers to the seizures of non-dominant temporal lobe.
Wednesday, December 10, 2008
Features of microscopic polyangitis
Cited text from Nadeau's
Clinical Neurology chapter from 2000:
This recently recognized variant of PAN52 is defined by inflammation that is largely confined to smaller vessels than in PAN - arterioles, capillaries and postcapillary venules. Angiograms are correspondingly normal. Major organ infarction is rare, glomerulonephritis is universal, and there is a high frequency of pulmonary hemorrhage. MPA is considerably more common than classic PAN and it is a considerably more common cause of a pulmonary-renal syndrome than Goodpasture's syndrome. A forme-fruste of MPA is characterized exclusively by glomerulonephritis. Evidence of hepatitis B infection is usually absent. In contrast to classic PAN as well as such small vessel vasculitides as cryoglobulinemic vasculitis and Henoch-Schönlein purpura, there is little or no evidence of immune deposits. Fifty to 80% of patients with MPA have circulating ANCA - usually myeloperoxidase (perinuclear or p-ANCA)(see WG), a rare phenomenon in classic PAN. Some have antibodies to proteinase-3 (central or c-ANCA) as in WG. Clinical distinction from WG may be difficult. Polyneuropathy is present in only 10-20% of patients, in contrast to the 50% or greater frequency in classic PAN. Patients with MPA have a high early case-fatality rate due to pulmonary and renal failure and they are considerably more prone to relapse after treatment.
Clinical Neurology chapter from 2000:
This recently recognized variant of PAN52 is defined by inflammation that is largely confined to smaller vessels than in PAN - arterioles, capillaries and postcapillary venules. Angiograms are correspondingly normal. Major organ infarction is rare, glomerulonephritis is universal, and there is a high frequency of pulmonary hemorrhage. MPA is considerably more common than classic PAN and it is a considerably more common cause of a pulmonary-renal syndrome than Goodpasture's syndrome. A forme-fruste of MPA is characterized exclusively by glomerulonephritis. Evidence of hepatitis B infection is usually absent. In contrast to classic PAN as well as such small vessel vasculitides as cryoglobulinemic vasculitis and Henoch-Schönlein purpura, there is little or no evidence of immune deposits. Fifty to 80% of patients with MPA have circulating ANCA - usually myeloperoxidase (perinuclear or p-ANCA)(see WG), a rare phenomenon in classic PAN. Some have antibodies to proteinase-3 (central or c-ANCA) as in WG. Clinical distinction from WG may be difficult. Polyneuropathy is present in only 10-20% of patients, in contrast to the 50% or greater frequency in classic PAN. Patients with MPA have a high early case-fatality rate due to pulmonary and renal failure and they are considerably more prone to relapse after treatment.
Friday, December 05, 2008
Causes of secondary headache to consider
head or neck trauma
dissection
CVT
Low pressure headache
neoplasm
neuroinfectious disease
medication overuse syndrome
systemic infection
acute glaucoma
other disorder of face sinuses
sleep apnea
fasting
somatization
occipital neuralgia
taken from NEJM 2008 359:21:2274
Chronic headaches notes
Case records of MGH nov 20 2008
chronic headaches, negative studies except dural enahncements. Pachymeningitis unlikely to be doe to Wegener's (which is ordinarily the most common cause) due to lack of pulmonary extran CNS signs and because of negative ANCA.
Sarcoid, Churg Strauss considered unlikely (former, no pulmonary findings, latter, normak eosiniphil count). RA has rare meningeal inflammation but this patient lacked arthritis even though RF positive. Sjogren's with dry mouth and eye irritation was considered, but Ro & La antibodies were negative as was a lip biopsy. Sjogren's does not explain pachymeningitis, DI, HA's or jaw claudication.
GCA is consistent with unrelenting headache, myalgia and weakness, PMR< pain in jaw with chewing (is specific but occurs only in 1/3 of patients with disease).
GCA was diagnosed by TA biopsy. Steroids were started with concomitant Calcium, Vitamin D, orla biphosphonate, and antiplatelet therapy, and eventually CPAP for sleep apnea associated with weight gain due to prednisone. They also gave trimethoprim to prevent PCP and PPI's.
Tuesday, November 11, 2008
PSP current nomenclature and types
"The postmortem room has become the temple of truth" (Donald Calne, re PSP) (Neurology 2008)
Current nomenclature "Richardson disease" corresponds with initial descriptions. There is a gradual onset of postural instability and falls within the first two years, with vertical supranuclear gaze palsy, a frontal dysexecutive syndrome, rigidity and bradykinesia that is not responsive to leveodopa, and a life expectancy of six years.
A second milder group at pm (post-mortem) have PSP tau pathology that is more restricted and less severe are called PSP-P (PSP-parkinsonism). They have assymmetric bradykinesia of the limbs, an initial response to levodopa, tremor and limb dystonia without early falls, eye movement problems, or cognitive dysfunction. Most patients with "atypical PSP" are in this category.
The third rarer category is pure akinesia with gait freezing (PAGF). There is gradual onset of unsteady or slow gait and hypophonia progressing to gair freezing and start hesitation, without limb rigidity or tremor. There is no response to levodopa and there is no dementia or opthalmoplegia in the first five years. In types 2 and 3 the median duration of the disease is around ten years.
Other patients with similar tau-PSP pathology present with corticobasal ganglionic degeneration, progressive nonfluent aphasia, or apraxia of speech.
Practice pearl: pay attention to SPEED of vertical saccades
References up to date: Williams DR, de Silva R, Pavour DC et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson' syndrome and PSP - parkinsonism. Brain 2005; 128:1247-1258. Williams DR, Holton JL, Strand C. et al. Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson's syndrome. Brain 2007; 130: 1566-1576. Mizusawa H, Mochizuki A , Ohkoshi N, et al. Progressive supranuclear palsy presenting with pure akinesia. Adv Neurol 1993; 60: 618-621. Josephs KA, Duffy JR, Strand EA et al. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain 2006; 129: April 13. Tsuboi Y, Josephs KA, Boeve BF et al. Increased tau burden in the cortices of progressive supranuclear palsy presenting with corticobasal syndrome. Mov Disord 2005; 20: 982-988.
var pageTracker = _gat._getTracker("UA-3639768-52");
pageTracker._initData();
pageTracker._trackPageview();
Current nomenclature "Richardson disease" corresponds with initial descriptions. There is a gradual onset of postural instability and falls within the first two years, with vertical supranuclear gaze palsy, a frontal dysexecutive syndrome, rigidity and bradykinesia that is not responsive to leveodopa, and a life expectancy of six years.
A second milder group at pm (post-mortem) have PSP tau pathology that is more restricted and less severe are called PSP-P (PSP-parkinsonism). They have assymmetric bradykinesia of the limbs, an initial response to levodopa, tremor and limb dystonia without early falls, eye movement problems, or cognitive dysfunction. Most patients with "atypical PSP" are in this category.
The third rarer category is pure akinesia with gait freezing (PAGF). There is gradual onset of unsteady or slow gait and hypophonia progressing to gair freezing and start hesitation, without limb rigidity or tremor. There is no response to levodopa and there is no dementia or opthalmoplegia in the first five years. In types 2 and 3 the median duration of the disease is around ten years.
Other patients with similar tau-PSP pathology present with corticobasal ganglionic degeneration, progressive nonfluent aphasia, or apraxia of speech.
Practice pearl: pay attention to SPEED of vertical saccades
References up to date: Williams DR, de Silva R, Pavour DC et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson' syndrome and PSP - parkinsonism. Brain 2005; 128:1247-1258. Williams DR, Holton JL, Strand C. et al. Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson's syndrome. Brain 2007; 130: 1566-1576. Mizusawa H, Mochizuki A , Ohkoshi N, et al. Progressive supranuclear palsy presenting with pure akinesia. Adv Neurol 1993; 60: 618-621. Josephs KA, Duffy JR, Strand EA et al. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain 2006; 129: April 13. Tsuboi Y, Josephs KA, Boeve BF et al. Increased tau burden in the cortices of progressive supranuclear palsy presenting with corticobasal syndrome. Mov Disord 2005; 20: 982-988.
var pageTracker = _gat._getTracker("UA-3639768-52");
pageTracker._initData();
pageTracker._trackPageview();
Tuesday, November 04, 2008
Horners syndrome pearls
Note-skipping standard textbook items, this is a(my) blog and I am ONLY including minutiae that I want to remember later and am purposely omitting much common information about horner's that is already well known to me. This post is based on H Wilhelm, "The Pupil" Curr Opin Neurol 21:36-42 2008.
1. Many cases of Horners after evaluation remain idiopathic.
2. Iatrogenic Horners after subclavian/jugular venous puncture is fairly common and well reported
3. Carotid dissection is associated with 25 or 37 % Horners s., and the key clinical pearl is the association of PAIN and acuity. 15 % have a bad outcome so beware of this diagnosis.
4. Beware of VI paresis and Horners which usually localizes to cavernous sinus and indicates a mass lesion there.
5. Previously unknown mediastinal tumors are RARE. Only two percent of bronchogenic tumors are Pancoast tumors . Other tumors that RARELY present are thyroid (usually benign goiter but could be CA) and nasopharyngeal CA.
6. In children neuroblastoma is fairly common and needs to be ruled out. rhabdomyosarcomas and gangliogliomas also are reported in this population.
7. Cocaine testing in texts is difficult to the trouble getting cocaine drops. Apraclonidine can be an effective substitute. Like cocaine, the pupil is only denervated in the third neuron in the arc and only such third neuron lesions dilate with apraclonidine. It does reverse anisocoria in such cases. The jury is truly not back in for apraclonidine. More studies are indicated.
8. To differentiate meiosis in Horners from physiologic anisocoria, check time to dilatation in darkness. Infrared video can be used if available. Normal pupils start to filate by .5-1 s and reach maximal dilatation by 5 s, whereas Horner pupils reach maximal dilatation by 10 seconds. Its only sensitive if repeated about four times; if only done once, it will most likely be false negative, if done four times, is 83 % sensitive.
More facts about Horner's
9. Reported after chest tube insertion
10. reported after cervical block/epidural
11. Klumpke's paralysis, goiter,MS sympathectomy, chiari, lateral medullary infarct,acute otitis, mandibular abscess, neuroblastoma,and cervical rib are other causes
12. Signs may include loss of ciliospinal reflex, heterochromia (iris)
13. In animals leashes if too tight can cause Horner's syndrome
14. Drugs are overlooked cause esp drugs that affect DA levels
14. Sweating pattern analyzed by Morris lee and Lim:
The distribution of sweating on the face was studied in 31 patients with Horner’s syndrome. In patients whose lesion was known to be distal to the bifurcation of the common carotid artery impairment of sweating was confined to the medial aspect of the forehead and side of the nose. In more proximal lesions loss of sweating involved the whole of one side of the face. Facial sweating was normal in 6 patients with avulsion injuries of the brachial plexus and in 2 patients with a lateral meduliary syndrome. These findings suggest that the pattern of sweating in Horner's syndrome may be useful in some patients in localizing the site of the lesion. (Brain, 1984)
1. Many cases of Horners after evaluation remain idiopathic.
2. Iatrogenic Horners after subclavian/jugular venous puncture is fairly common and well reported
3. Carotid dissection is associated with 25 or 37 % Horners s., and the key clinical pearl is the association of PAIN and acuity. 15 % have a bad outcome so beware of this diagnosis.
4. Beware of VI paresis and Horners which usually localizes to cavernous sinus and indicates a mass lesion there.
5. Previously unknown mediastinal tumors are RARE. Only two percent of bronchogenic tumors are Pancoast tumors . Other tumors that RARELY present are thyroid (usually benign goiter but could be CA) and nasopharyngeal CA.
6. In children neuroblastoma is fairly common and needs to be ruled out. rhabdomyosarcomas and gangliogliomas also are reported in this population.
7. Cocaine testing in texts is difficult to the trouble getting cocaine drops. Apraclonidine can be an effective substitute. Like cocaine, the pupil is only denervated in the third neuron in the arc and only such third neuron lesions dilate with apraclonidine. It does reverse anisocoria in such cases. The jury is truly not back in for apraclonidine. More studies are indicated.
8. To differentiate meiosis in Horners from physiologic anisocoria, check time to dilatation in darkness. Infrared video can be used if available. Normal pupils start to filate by .5-1 s and reach maximal dilatation by 5 s, whereas Horner pupils reach maximal dilatation by 10 seconds. Its only sensitive if repeated about four times; if only done once, it will most likely be false negative, if done four times, is 83 % sensitive.
More facts about Horner's
9. Reported after chest tube insertion
10. reported after cervical block/epidural
11. Klumpke's paralysis, goiter,MS sympathectomy, chiari, lateral medullary infarct,acute otitis, mandibular abscess, neuroblastoma,and cervical rib are other causes
12. Signs may include loss of ciliospinal reflex, heterochromia (iris)
13. In animals leashes if too tight can cause Horner's syndrome
14. Drugs are overlooked cause esp drugs that affect DA levels
14. Sweating pattern analyzed by Morris lee and Lim:
The distribution of sweating on the face was studied in 31 patients with Horner’s syndrome. In patients whose lesion was known to be distal to the bifurcation of the common carotid artery impairment of sweating was confined to the medial aspect of the forehead and side of the nose. In more proximal lesions loss of sweating involved the whole of one side of the face. Facial sweating was normal in 6 patients with avulsion injuries of the brachial plexus and in 2 patients with a lateral meduliary syndrome. These findings suggest that the pattern of sweating in Horner's syndrome may be useful in some patients in localizing the site of the lesion. (Brain, 1984)
Oculomotor syndromes PEARLS
cf Tilikete C, Pelisson D. Oculomotor syndromes of the brainstem and cerebellum. Curr Opin Neurol 21:22-28 2008
Head shaking nystagmus (hsn)- usually is a peripheral vestibular lesion. However it can occur in Wallenberg syndrome.
In central positional nystagmus, Dix Hallpike testing induces downbeat nystagmus(DBN). DBN also occurs in floccular disturbance and the gravity dependent component can be suppressed with 3,4 aminopyridine. Other oddball causes of DBN include West Nile virus encephalomyelitis, intrathecal morphine, a particular genetic syndrome of cerebellar ataxia, or another syndrome combining DBN with motor neuronopathy and cerebellar ataxia.
Head shaking nystagmus (hsn)- usually is a peripheral vestibular lesion. However it can occur in Wallenberg syndrome.
In central positional nystagmus, Dix Hallpike testing induces downbeat nystagmus(DBN). DBN also occurs in floccular disturbance and the gravity dependent component can be suppressed with 3,4 aminopyridine. Other oddball causes of DBN include West Nile virus encephalomyelitis, intrathecal morphine, a particular genetic syndrome of cerebellar ataxia, or another syndrome combining DBN with motor neuronopathy and cerebellar ataxia.
Saturday, November 01, 2008
Abnormalities (hemorrhages etc.) conjunctiva-causes
1.Subconjunctival hemorrhages-- common in trauma, rare in SAH and severe HTN
2. Leptospirosis-- injection of conjunctiva plus meningitis and myopathy
3. Filarial migratory phase of loa-loa-- injection is seen
4. telangiectasia in conjunctiva seen in sickle cell disease and ataxia telangiectasia
5. Retroorbital tumors can produce injection
6. Renal failure can produce severe conjunctival injection
Hypertelorism-- list of causes
1. normal
2. congenital absent callosum
3. Aicardi's syndrome
4. Schapiro's syndrome ( hypothermia and other congenital defects)
5. Septo-optic dysplasia
Friday, October 31, 2008
More pearls of fundoscopic exam temporal pallor and atrophy
Pallor is noted in any process affecting maculopapillary bundle. The disk is alabaster white and bound by a gray white cup. Green light of the opthalmoscope shows the fibers as they enter the disk.
Optic atrophy has seven major features: 1) pale aspirin-white disc. 2) sharp margins 3) loss of lamina cribosa 4) increased cup to disc ratio 5) decreased arterioles off disc (less than 14) 6) small arteries and 7) gray pale retina.
Swelling has seven features 1) Loss of disk margin, superior then inferior then temporal Always blurred nasal margins 2) Fat veins without pulsations 3) Erythema off the disk 4) loss of lamina cribosa, whole disc pushed forward 5) Slit hemorrhages off disc margin 6) engorged veins appear and disappear near macula 7) Folds in retina spread towards the macula. Last normal acuity.
Neuroopthalmology testing-- bedside pearls
Riddoch's phenomenon-- patient won't see an object in a damaged field unless it is moved.
shading of a visual field-- if larger objects are seen in a damaged field where smaller objects are missed, , suggests defect is partly caused by edema or pressure phenomenon.
Bjerrum screen (tangent screen) enlarges central meridian to 30 degrees and is most helpful for measuring central scotomata and the blind spot.
Graying of vision (finger does not appear flesh colored) or red desaturation (eg. red pin ) is appreciated before a quantifiable field defect.
Notes bitemporal upper quadrantic defect suggests chiasmal compression from above, but bitemporal lower field defect suggests compression from below.
Arcuate defect- optic nerve lesion prechiasmatic.
Bitemporal scotomata-- early bitemporal field defect or consider bilateral optic nerve lesions eg in kids.
Fundoscopic exam
Venous pulsations are seen only in the middle and not the margins of the disk unless there is a large pulse pressure such as in aortic insufficiency. severe hyperthyroidism or arteriovenous fistula.
Miscellaneous findings include commotio retina, an intense light streak seen with acute head injury of Kohlmeyer-Degos disease (arteritis with atrophic skin lesions).
Hemorrhages from papilledema occur off the disk margin (slit hemorrhages) whereas those from venous occlusion occur in the central retina and macula.
Torsten's syndrome is a hemorrhage that moves with head position following a burst aneurysm, also called preretinal or subhyaloid hemorrhage. Often can identify side of hemorrhage based on.
Lupus patients may have "grains of rice" or cytoid bodies in peripheral retina.
Renal patients may have a macular star (edema outlining the nerve sheath layer).
Hollenhorst plaque or branch point occlusion of cholesterol emboli are larger than occluded vessel birefringent and yellow.
Platelet fibrin emboli from HIT are white and multiple.
shading of a visual field-- if larger objects are seen in a damaged field where smaller objects are missed, , suggests defect is partly caused by edema or pressure phenomenon.
Bjerrum screen (tangent screen) enlarges central meridian to 30 degrees and is most helpful for measuring central scotomata and the blind spot.
Graying of vision (finger does not appear flesh colored) or red desaturation (eg. red pin ) is appreciated before a quantifiable field defect.
Notes bitemporal upper quadrantic defect suggests chiasmal compression from above, but bitemporal lower field defect suggests compression from below.
Arcuate defect- optic nerve lesion prechiasmatic.
Bitemporal scotomata-- early bitemporal field defect or consider bilateral optic nerve lesions eg in kids.
Fundoscopic exam
Venous pulsations are seen only in the middle and not the margins of the disk unless there is a large pulse pressure such as in aortic insufficiency. severe hyperthyroidism or arteriovenous fistula.
Miscellaneous findings include commotio retina, an intense light streak seen with acute head injury of Kohlmeyer-Degos disease (arteritis with atrophic skin lesions).
Hemorrhages from papilledema occur off the disk margin (slit hemorrhages) whereas those from venous occlusion occur in the central retina and macula.
Torsten's syndrome is a hemorrhage that moves with head position following a burst aneurysm, also called preretinal or subhyaloid hemorrhage. Often can identify side of hemorrhage based on.
Lupus patients may have "grains of rice" or cytoid bodies in peripheral retina.
Renal patients may have a macular star (edema outlining the nerve sheath layer).
Hollenhorst plaque or branch point occlusion of cholesterol emboli are larger than occluded vessel birefringent and yellow.
Platelet fibrin emboli from HIT are white and multiple.
Schwartzmann 2 second mental status exam
The tests below are not thorough or well vetted, but they can be done easily at grand rounds, appear novel to the students, and are explained in Robert Schwartzman's book on the neurological examination.
He tests judgment ("What would you do if you found a letter addressed to someone else?); visual praxis (copy a hand posture after seeing it for two seconds); language/memory/frontal four part oral command, and frontal face hand test (touches patient's hand and own face and asks where am I touching you).
Monday, October 27, 2008
Transient visual loss
see also the following behavioral neurology post http://behavioralneurologynotes.blogspot.com/2008/05/hallucinations-and-related-conditions.html
Consider: noninschemic
1. opthalmic surface disorders
a. tear film abnormalities-- visual blurring occurs many times a week often at the end of the day and under one environmental circumstance. Blinking and lubricating eye helps. Patients with blepharitis may be worse in the morning due to accumulated meibomian gland secretion overnight. With dry eyes causing epithelial breakdown, there may be foreign body sensation, pain or redness with a positive Schirmer's test. Patients with rosacea frequently have opthalmic involvement. Also PD.
2. Other ocular causes-- narrow angle glaucoma causes transient blurring with halos. Recurrent spontaneous anterior chamber hemorrhages is such patients needs to be differentiated from those occurring in juvenile xanthogranuloma, malpositioned lens with iris contact, and iris neovascularization.
Patients with corneal dysfunction have episodes of blurred vision that last for hours especially in the mornings, must examine during an episode. Episodes in patients with diabetes due to hyper or hypoglycemia occur.
Ischemic Causes
Causes-- 60 % are due to carotid stenosis/occlusion. Also called TMB, amaurosis fugax, or retinal tia. In usual cases , emboli from carotid are common but low flow also occurs when collaterals from the Circle of Willis and eca are compromised. In these cases symptoms may be inducible by change in position or eating, or when distal flow is inadequate such as chronic ocular ischemia or bright light amaurosis. The latter occurs with TMB after sunlight or looking at a white wall, in the setting of carotid occlusion and is a well known phenomenon.
Risk of stroke per annum is 2 % with 1 % risk of permanent visual loss, v. 5-8 % risk of stroke with hemispheric tia's.
Symptoms are abrupt onset, painless, lasts 1-5 minutes, darkening or fogging (not blurring) of visual field, altitudinal pattern (shade closing) of vision loss, and return of vision over minutes.
Vision loss may be altitudinal, peripheral, central or even vertical. A nasal field defect may suggest emboli due to lodging in temporal retinal circulation. Rarely, scintillating scotomas occur. Pain which is rare may suggest temporal arteritis.
Neuroopthalmic signs-- the most common are retinal emboli, including cholesterol (Hollenhorst) plaques which are refractile, metallic gold appearance and indicate carotid disease and platelet fibrin emboli which are creamy white grey longitudinal intravascular opacifications that fill the lumen, and indicate either carotid thrombosis or thrombosis with recent MI. Chronic severe carotid disease leads to chronic ocular ischemia including episcleral and conjunctival injection, corneal edema, and neovascular glaucoma. Venous stasis causes microaneurysms and blot hemorrhages. Unlike diabetes they are unilateral (ipsilateral to stenosis) and mid periphery instead of posterior pole. Rarely retinal calcific emboli occur in patients with cardiac valvular disease. They are grey white and ovoid and infarct the retina.
In papilledema, episodes of grey black and white vision lasting seconds may occur. Its fleeting and associated with changes in position. This also occurs in pseudopappilledema drusen or colobomas. Gaze evoked amaurosis suggests intraorbital lesion such as glioma, nerve sheath meningioma, or intraconal mass such as cavernous hemangioma. Vision deteriorates seconds after eccentric gaze and returns to normal with straight ahead gaze.
Vision loss lasting seconds
papilledema, IIH, optic disc drusen, optic nerve sheath meningioma (need MRI with contrast),
gaze evoked (orbital tumor suspect). Get T1 image in 3 planes to differentiate MRV clot from physiologic narrowing of vessel
Vision loss can occur due to ACA aneurysm due to leakage of blood into opthalmic sheath
Consider: noninschemic
1. opthalmic surface disorders
a. tear film abnormalities-- visual blurring occurs many times a week often at the end of the day and under one environmental circumstance. Blinking and lubricating eye helps. Patients with blepharitis may be worse in the morning due to accumulated meibomian gland secretion overnight. With dry eyes causing epithelial breakdown, there may be foreign body sensation, pain or redness with a positive Schirmer's test. Patients with rosacea frequently have opthalmic involvement. Also PD.
2. Other ocular causes-- narrow angle glaucoma causes transient blurring with halos. Recurrent spontaneous anterior chamber hemorrhages is such patients needs to be differentiated from those occurring in juvenile xanthogranuloma, malpositioned lens with iris contact, and iris neovascularization.
Patients with corneal dysfunction have episodes of blurred vision that last for hours especially in the mornings, must examine during an episode. Episodes in patients with diabetes due to hyper or hypoglycemia occur.
Ischemic Causes
Causes-- 60 % are due to carotid stenosis/occlusion. Also called TMB, amaurosis fugax, or retinal tia. In usual cases , emboli from carotid are common but low flow also occurs when collaterals from the Circle of Willis and eca are compromised. In these cases symptoms may be inducible by change in position or eating, or when distal flow is inadequate such as chronic ocular ischemia or bright light amaurosis. The latter occurs with TMB after sunlight or looking at a white wall, in the setting of carotid occlusion and is a well known phenomenon.
Risk of stroke per annum is 2 % with 1 % risk of permanent visual loss, v. 5-8 % risk of stroke with hemispheric tia's.
Symptoms are abrupt onset, painless, lasts 1-5 minutes, darkening or fogging (not blurring) of visual field, altitudinal pattern (shade closing) of vision loss, and return of vision over minutes.
Vision loss may be altitudinal, peripheral, central or even vertical. A nasal field defect may suggest emboli due to lodging in temporal retinal circulation. Rarely, scintillating scotomas occur. Pain which is rare may suggest temporal arteritis.
Neuroopthalmic signs-- the most common are retinal emboli, including cholesterol (Hollenhorst) plaques which are refractile, metallic gold appearance and indicate carotid disease and platelet fibrin emboli which are creamy white grey longitudinal intravascular opacifications that fill the lumen, and indicate either carotid thrombosis or thrombosis with recent MI. Chronic severe carotid disease leads to chronic ocular ischemia including episcleral and conjunctival injection, corneal edema, and neovascular glaucoma. Venous stasis causes microaneurysms and blot hemorrhages. Unlike diabetes they are unilateral (ipsilateral to stenosis) and mid periphery instead of posterior pole. Rarely retinal calcific emboli occur in patients with cardiac valvular disease. They are grey white and ovoid and infarct the retina.
In papilledema, episodes of grey black and white vision lasting seconds may occur. Its fleeting and associated with changes in position. This also occurs in pseudopappilledema drusen or colobomas. Gaze evoked amaurosis suggests intraorbital lesion such as glioma, nerve sheath meningioma, or intraconal mass such as cavernous hemangioma. Vision deteriorates seconds after eccentric gaze and returns to normal with straight ahead gaze.
Vision loss lasting seconds
papilledema, IIH, optic disc drusen, optic nerve sheath meningioma (need MRI with contrast),
gaze evoked (orbital tumor suspect). Get T1 image in 3 planes to differentiate MRV clot from physiologic narrowing of vessel
Vision loss can occur due to ACA aneurysm due to leakage of blood into opthalmic sheath
Sunday, October 26, 2008
Patterns of functional visual loss
taken from Liu Volpe and Galetta text p. 388
TOTAL BLINDNESS
1. General observation of functionally blind-- may move into and bump objects without falling and hurting themselves; may not look at examiner speaking to them as a truly blind person might do; may wear sunglasses or mimic Stevie Wonder or other famously blind people.
2. Pupils-- should be abnormal except in cortically blind. A patient with total blindness and intact pupillary reflexes is likely to be functional. A complaint of photophobia with orbicularis oculi contraction to bright light is incompatible with an ocular cause of blindness.
3. OKN's- response is involuntary, but can be blocked by looking away from or beyond stimulus, or by excessive convergence.
4. Proprioceptive tests are performed by truly blind but not by functional. For example, looking at one's hand, bringing the tips of the fingers together from a distance, or signing one's name are better done by truly blind.
5. Mirror test-- a large mirror in front of a blind person, rocked, will produce an involuntary response if the patient is sighted and has better than hand motion vision..
6. Surprise-- atypical behaviors such as making faces, writing shocking words.
7. Evoked potentials-- a normal test can help, but an abnormal test can be ambiguous. My have in evoked fields, have characteristic hemifield loss from patient looking down.
UNILATERAL VISION LOSS
1. RAPD
2. Stereopsis-- requires binocular vision-- Titmus test
3. Placement of green lens should prevent visualization of Ishihara color plates
SEE BOOK FOR MORE
Monday, October 13, 2008
IRIS Immune reconstitution syndrome in HIV
NEUROLOGY 2009;72:835-841
Patients with low CD4 counts initiating cART therapy who then bump their CD4 counts and lower viral counts are at risk. Patients may or may not have concurrent infection. Authors describe 7 patients, with Neuro IRIS.
Presentations include trouble walking, seizure, encephalopathy, and occurred in early phase-- first 2-3 weeks after initiation of therapy (early, due to memory cells) or delayed, after 4-6 weeks phase (due to proliferation of new T lymphocytes)/ Latter tended to be a more prolonged course. Biopsy of one patient showed lots of CD8 and CD40 cells stained positive. CSF showed pleocytosis, and MRI showed diffuse white matter changes with enhancement. Lamivudine was in all cases one of the therapies used. The overall risk was 0.9 % among patients starting cART, but 1.5% when stratified with those having low CD4 count to start (<200). This is much lower than the number of non neuro IRIS cases that have been considered to be as high as 15 % although these numbers may be high.
Patients with low CD4 counts initiating cART therapy who then bump their CD4 counts and lower viral counts are at risk. Patients may or may not have concurrent infection. Authors describe 7 patients, with Neuro IRIS.
Presentations include trouble walking, seizure, encephalopathy, and occurred in early phase-- first 2-3 weeks after initiation of therapy (early, due to memory cells) or delayed, after 4-6 weeks phase (due to proliferation of new T lymphocytes)/ Latter tended to be a more prolonged course. Biopsy of one patient showed lots of CD8 and CD40 cells stained positive. CSF showed pleocytosis, and MRI showed diffuse white matter changes with enhancement. Lamivudine was in all cases one of the therapies used. The overall risk was 0.9 % among patients starting cART, but 1.5% when stratified with those having low CD4 count to start (<200). This is much lower than the number of non neuro IRIS cases that have been considered to be as high as 15 % although these numbers may be high.
Pearls for Multiple System Atrophy
Neurologist July 2008 14L224-237 authors Bhidayasiri R, Ling H.
MSA encompasses sporadic OPCA (MSA-C) and SND (OPC-P) with predominant Parkinsonism and both types including dysautonomia. Tremor is common but not classic pill rolling tremor. Impotence in men and urge incontinence in women are nearly universal, postural hypotension occurs in 2/3 and syncopal episodes in 15 %. Stridor and respiratory insufficiency may be presenting signs. 29 % respond to levodopa AT SOME POINT.
Red flags suggestive of MSA include early severe autonomic dysfunction, spontaneous or L dopa induced orofacial dyskinesias, which may even resemble risus sardonica in tetanus. Pisa syndrome is a form of axial dystonia, and camptocormia is forward flexion of the trunk. These are not specific and may be seen in PD. Disproportionate anterocollis may occur at any point of the disease, although rare, and botox for this can worsen dysphagia; this is a red flag. So is minipolymyoclonus, which is postural or stimulus sensitive small amplitude nonrhythmic movements of a few fingers or the whole hand. Dysarthria may be diagnostic, with a quivering, croaky strained element "reminiscent of myoclonic speech." (cit Arch Neurol 1996 53:545-548). Nighttime stridor is helpful diagnostically but poor prognostically and may present as laryngeal paresis. Sleep apnea, the "cold hand sign" and emotional incontinence comprise the other red flags.
groupings of "red flags: show six categories early instability, rapid progression, abnormal postures, bulbar dysfunction, respiratory dysfunction, emotional incontinence. Presence of two or more categories had high sensitivity (84 % ) and specificity (98%). see Hughes et al. JNNP 1992; 55:181-184.
Exclusion criteria also are important, including age under 30, positive family history of MSA (PD is "OK"), frank dementia at onset, and eye movement abnormalities suggestive of PSP or CBGD.
The Quinn criteria for possible, probable and definite MSA are reviewed. International Consensus Conference Criteria of the AAN are considered better. See NEJM 2004; 351:912-924 (Case Records of the MGH).
MRI signs include atrophy of the cerebellar vermis, ponsmiddle cerebellar peduncles and lower brainstem. Signal change in the pons may resemble a "hot cross bun" (Catholics may understand this). In MSA-P, patients may have putamenal atrophy, with slit like void signal (black reflecting gliosis) which is sensitive but not specific, but is sensitive and specific if coupled with hyperintense T2 signal in the putamen. For a PD/MSA algorithm, see Arch Neurol 2002; 59:835-842). Onuf's nucleus degenerates which accounts for bladder problems. Pathologic confirmation is made by finding of alpah synuclein positive GCI's composed of misfolded alpah synuclein.
Pearls for treatment include, use of levodopa in many, the benefits of paxil 90 in one trial for motor function, botox of submandibular glands for drooling, and into adductor muscles for stridor, with concerns as above for other uses of botox. PT/OT/ST/ gait training and assistive devices are useful. Treatment of dysautonomic can include, if needed, avoiding large meal, alcohol, straining, certain meds; elastic stockings, bed tilt up at night. Drugs used for orthostasis include florinef, midodrine, desmopressin, octeotride, and eryuthorpoeitin. NGB can be treated with intermittent catheterisation, anticholinergics if detrusor hyperreflexia exists, suprapubic vibration devices, alpha adrenergic receptor antagonists, but NOT surgery. Beware of sildafenil for impotence due to hypotension. Macrogol 3350 for chronic constipation is safe and effective, increasing water content of stools. CPAP, may be needed, tracheostomy may be needed but also may be fatal due to hypercapnia. CPAP mat be contra-indicated if the epiglottis is floppy (Neurology 2011; 76:1841)
MSA encompasses sporadic OPCA (MSA-C) and SND (OPC-P) with predominant Parkinsonism and both types including dysautonomia. Tremor is common but not classic pill rolling tremor. Impotence in men and urge incontinence in women are nearly universal, postural hypotension occurs in 2/3 and syncopal episodes in 15 %. Stridor and respiratory insufficiency may be presenting signs. 29 % respond to levodopa AT SOME POINT.
Red flags suggestive of MSA include early severe autonomic dysfunction, spontaneous or L dopa induced orofacial dyskinesias, which may even resemble risus sardonica in tetanus. Pisa syndrome is a form of axial dystonia, and camptocormia is forward flexion of the trunk. These are not specific and may be seen in PD. Disproportionate anterocollis may occur at any point of the disease, although rare, and botox for this can worsen dysphagia; this is a red flag. So is minipolymyoclonus, which is postural or stimulus sensitive small amplitude nonrhythmic movements of a few fingers or the whole hand. Dysarthria may be diagnostic, with a quivering, croaky strained element "reminiscent of myoclonic speech." (cit Arch Neurol 1996 53:545-548). Nighttime stridor is helpful diagnostically but poor prognostically and may present as laryngeal paresis. Sleep apnea, the "cold hand sign" and emotional incontinence comprise the other red flags.
groupings of "red flags: show six categories early instability, rapid progression, abnormal postures, bulbar dysfunction, respiratory dysfunction, emotional incontinence. Presence of two or more categories had high sensitivity (84 % ) and specificity (98%). see Hughes et al. JNNP 1992; 55:181-184.
Exclusion criteria also are important, including age under 30, positive family history of MSA (PD is "OK"), frank dementia at onset, and eye movement abnormalities suggestive of PSP or CBGD.
The Quinn criteria for possible, probable and definite MSA are reviewed. International Consensus Conference Criteria of the AAN are considered better. See NEJM 2004; 351:912-924 (Case Records of the MGH).
MRI signs include atrophy of the cerebellar vermis, ponsmiddle cerebellar peduncles and lower brainstem. Signal change in the pons may resemble a "hot cross bun" (Catholics may understand this). In MSA-P, patients may have putamenal atrophy, with slit like void signal (black reflecting gliosis) which is sensitive but not specific, but is sensitive and specific if coupled with hyperintense T2 signal in the putamen. For a PD/MSA algorithm, see Arch Neurol 2002; 59:835-842). Onuf's nucleus degenerates which accounts for bladder problems. Pathologic confirmation is made by finding of alpah synuclein positive GCI's composed of misfolded alpah synuclein.
Pearls for treatment include, use of levodopa in many, the benefits of paxil 90 in one trial for motor function, botox of submandibular glands for drooling, and into adductor muscles for stridor, with concerns as above for other uses of botox. PT/OT/ST/ gait training and assistive devices are useful. Treatment of dysautonomic can include, if needed, avoiding large meal, alcohol, straining, certain meds; elastic stockings, bed tilt up at night. Drugs used for orthostasis include florinef, midodrine, desmopressin, octeotride, and eryuthorpoeitin. NGB can be treated with intermittent catheterisation, anticholinergics if detrusor hyperreflexia exists, suprapubic vibration devices, alpha adrenergic receptor antagonists, but NOT surgery. Beware of sildafenil for impotence due to hypotension. Macrogol 3350 for chronic constipation is safe and effective, increasing water content of stools. CPAP, may be needed, tracheostomy may be needed but also may be fatal due to hypercapnia. CPAP mat be contra-indicated if the epiglottis is floppy (Neurology 2011; 76:1841)
Subscribe to:
Posts (Atom)
Posts
