Saraf Tepi Flashcards

Question

Ankle Neuropathy / Charcot joint

Answer 1

Joint deformitas yg berkaitan dengan hilangnya sensasi Dan ataxia sensoris yg disebabkan Neuropathy

Answer 2

acute brachial plexitis : neuralgic amyotrophy / Parsonage–Turner syndrome. - Dapat terjadi setelah surgery, vaccination, systemic viral illness, idiopathic. - Gejala acute brachial neuritis : acute onset of severe shoulder and arm pain, kmdn resolve, dengan kelemahan subsequent - Saraf yg terlibat: suprascapular nerve, long thoracic nerve, median, lumbosacral plexus. - sering monophasic with good recovery - beberapa kasus herediter terkait gen SEPT9 kromosom 17

Answer 3

Terjadi saat penempatan arteriovenous shunt pada dialisis. | Klinis : nyeri Dan hilangnya sensoris yg mengelilingi distribusi saraf multipel

Answer 4

Panplexopathy : painless radiation-induced plexopathy. Dapat muncul berbulan bulan setelah radiasi. Myokimia pada EMG membedakan nya dgn carsinomatous invasion

Answer 5

1. Acute inflammatory Demyelinating Polyneuropathy : Demyelinisasi, latensi memanjang, conduction velocity lambat 2. Acute Motor and Sensory Axonal Neuropathy : Tidak Ada gambaran demiyelinisasi, tapi Amplitudo motor Dan sensor turun . terdapat antibody GM1, GM1b , GD1a 3. Acute Motor Axonal Neuropathy : Tidak Ada gambaran demiyelinisasi, tapi Amplitudo motor turun. terdapat antibody GM1, GM1b , GD1a, GalNAc-GD1a 4. Acute sensory Neuronopathy : Terdapat antibody GD1b 5. Miller fisher syndrome : trias ataxia, Opthalmoplegy, areflexia. Terdapat antibody GQ1b

Answer 6

(MADSAM) is a demyelinating neuropathy with evidence of conduction block, presenting with asymmetric motor and sensory symptoms. The progression is gradual and slow, usually involving the upper limbs initially and later the lower limbs. Deep tendon reflexes are diminished in the distribution of the affected nerves. On CSF examination, the protein is usually elevated. Unlike multifocal motor neuropathy, anti-GM1 antibodies are not present, and electrophysiologic studies show abnormalities in motor and sensory nerves, with features of demyelination and conduction block. Patients with MADSAM may improve on steroids.

Answer 7

- defisiensi enzyme Alfa galactosidase A, enzim lisosom. - accumulation of globotriaosylceramide Di kulit, ginjal, jantung. - lesi Di kulit : angiokeratoma ; titik hitam pada badan Dan area intertriginous seperti axilla, scrotum - pada saraf : Peripheral nervous system complications ; small-fiber neuropathy& autonomic neuropathy. Stroke krn cardioembolic atau large or small vessel occlusion. Endothelial deposition of globotriaosylceramide menyebabkan dolichoectasia, increasing risk for thrombosis

Answer 8

Penyakit herediter dgn Gejala utama sensoris. Lebih jarang drpd CMT. - symptoms, including pain, sensory loss, and autonomic features with little motor involvement. Because of the sensory loss, patients with HSANs are prone to painful calluses, stress fractures, neuropathic (Charcot) joints, skin ulcerations that heal poorly, and infections with deep tissue involvement, such as osteomyelitis, leading to disfiguring acral mutilations. Macam : 1. HSAN 1 : paling sering. Tipikal pd dewasa muda. 2. HSAN 2 : infancy. Hilangnya sensitivity pada nyeri shg beresiko mutilasi tangan, bibir, lidah. Gejala Otonom tidak menonjol 3. HSAN 3 : familial dysautonomia or Riley–Day syndrome, an autosomal recessive HSAN with prominent autonomic features. Symptom onset is in infancy with dysphagia, vomiting, recurrent infections, and blood pressure lability. It is particularly prevalent among Ashkenazi Jews. With emotional stimulation, there is hyperhidrosis, skin flushing, and hypertension 4. HSAN 4 : congenital insensitivity to pain with anhidrosis 5. HSAN 5 : congenital insensitivity to pain with partial anhidrosis

Answer 9

involvement of two or more nerves, usually with acute to subacute onset, in which subsequent nerves are involved at irregular intervals. Common causes of mononeuritis multiplex are vasculitic neuropathy, polyarteritis nodosa, Wegener’s granulomatosis, Sjogren’s syndrome, or Churg–Strauss syndrome, sarcoidosis, paraneoplastic processes, amyloidosis, leprosy, systemic lupus erythematosus, rheumatoid arthritis, and lymphoma. Diabetes can present not only with a distal symmetric polyneuropathy, but can also be associated with mononeuritis multiplex.

Answer 10

rare autosomal dominant disorder characterized by episodes of severe burning and erythema of the distal extremities. These episodes may be precipitated by exposure to either heat or cold. Patients are typically asymptomatic between episodes

Answer 11

complement levels are reduced. The clinical manifestation of cryoglobulinemia includes nonspecific constitutional symptoms, palpable purpura, arthralgias, lymphadenopathy, hepatosplenomegaly, and peripheral neuropathy, including mononeuritis multiplex. Cryoglobulins are immunoglobulins that precipitate when exposed to cold temperatures and redissolve on rewarming

Answer 12

Because the dysfunctional enzymes have some residual enzyme activity, symptoms often do not appear until adolescence or later, with some symptoms occurring in episodes with exposure to certain triggers. Common triggers include medications, menstruation, and alcohol . Symptoms typically begin with abdominal pain and other gastrointestinal symptoms, followed by neurologic symptoms, including most prominently manifestations of autonomic instability (tachycardia, labile hypertension, orthostasis, and urinary retention). Neuropsychiatric symptoms including psychosis occur, and seizures may be present as well. In some patients, a subacute predominantly motor neuropathy occurs; the arms may be affected prior to the legs, and proximal muscles are involved more than distal muscles. Involvement of the radial nerve in isolation may occur. The neuropathy is both axonal and demyelinating. Cranial nerve involvement, and even respiratory muscle involvement leading to respiratory failure, may occur

Answer 13

Beevor sign : umbilicus moves upward with neck flexion | Terdapat pada facioscapulohumeral muscular dystrophy (FSHD).

Answer 14

- overstimulation muscarinic receptors systemic krn overdosing pyridostigmine. - Klinis : - Yang membedakan dengan myasthenic crisis : presence of nausea, vomiting, diaphoresis, sialorrhea, excessive bronchial secretions, miosis, bradycardia, and diarrhea

Answer 15

: Acetylcholinesterase inhibitors, including pyridostigmine and neostigmine, inhibit acetylcholinesterase, increasing levels of acetylcholine at the neuromuscular junction (NMJ) - Efek samping : activation of muscarinic receptors outside of the NMJ, and include diarrhea, nausea, abdominal cramps, and increased bronchial secretions. At therapeutic doses, pyridostigmine does not affect central cholinergic pathways, as it does not cross the blood–brain barrie

Answer 16

- Acetylcholine receptor modulating antibodies are seen in autoimmune myasthenia gravis. Anti-striational muscle antibodies can be detected in patients with autoimmune myasthenia gravis or thymoma. s. In approximately half of all patients who are seronegative for antibodies against the acetylcholine receptor, anti MuSK (anti–muscle-specific tyrosine kinase) antibodies are present. - Antibodies against presynaptic P/Q-type voltage-gated calcium channel are seen in Lambert–Eaton syndrome

Answer 17

In approximately half of all patients who are seronegative for antibodies against the acetylcholine receptor, antiMuSK antibodies are present. In patients with anti-MuSK antibodies, the clinical picture may resemble the typical form of autoimmune myasthenia gravis or ocular myasthenia, but more commonly, weakness involves predominantly cranial and bulbar muscles, with prominent dysphagia, neck flexor weakness, and respiratory weakness, with relative sparing of ocular muscles (though eyelid and ocular muscle involvement may occur). This disorder is more common in young women, and typically does not respond to pyridostigmine. Treatment is with immune-modulating therapy, including intravenous immunoglobulins and plasmapheresis

Answer 18

LMGD mrpkn muscular dystrophies dengan proximal weakness , melibatkan shoulder or pelvic girdle with sparing facial muscles. - The mode of inheritance menentukan classification, berdasarkan the genetic defects & gene products involved - Jenis : LGMD1 (autosomal dominant) Dan LGMD 3 (autosomal recessive) - Biopsi : dystrophic changes, with myofiber necrosis, degeneration, regeneration, increased fiber size variation, endomysial inflammation, and fibrosis

Answer 19

amyotrophic lateral sclerosis (ALS). ALS is a neurodegenerative disorder that affects motor neurons in the anterior horn of the spinal cord, but also the motor cortex and brainstem The hallmark of ALS is evidence of both upper motor neuron disease (such as hyperreflexia, clonus, and presence of a Babinski sign) and lower motor neuron disease (atrophy, fasciculations in the limbs, trunk, tongue, and occasionally face) in the absence of sensory symptoms or sensory abnormalities on examination. Clinical presentation is variable but most often muscle weakness begins focally, usually in the extremities (two-thirds of cases) and spreads to involve contiguous regions, though there is significant variability in the pattern of motor weakness. The split-hand phenomenon is a feature of ALS, characterized by weakness and atrophy of the lateral hand (thenar and first dorsal interosseous muscles) with relative sparing of the medial hand (hypothenar) muscles. Approximately one-third of patients present with bulbar symptoms, such as dysarthria or dysphagia (bulbar-onset ALS). Other symptoms of bulbar involvement include sialorrhea. Pseudobulbar palsy (pathologic laughter and crying without a corresponding change in mood) and excessive yawning

Answer 20

Primary lateral sclerosis (PLS) is characterized by presence of upper motor neuron signs at least 3 years from symptom onset without evidence of lower motor neuron dysfunction. PLS is considered along the spectrum of amyotrophic lateral sclerosis (ALS) although it is controversial as to whether or not it is a separate entity. It typically presents in the sixth decade of life with a progressive spastic tetraparesis and later, cranial nerve involvement. Rarely, bulbar onset occurs. Spasticity, rather than muscle weakness or atrophy, is the most prominent feature. It typically progresses slowly over years. Autonomic involvement does not typically occur. Autopsy in patients with PLS has shown significant cell loss in layer 5 of the motor and premotor cortex, predominantly the large pyramidal Betz cells with corticospinal tract degeneration.

Answer 21

Progressive muscular atrophy (PMA) is considered along the spectrum of ALS, although this is controversial; it is a motor neuron disease that affects only the lower motor neurons, distinguishing it from ALS, which affects both upper and lower motor neurons. It often presents with focal asymmetric distal weakness that later involves more proximal regions and other extremities, with lower motor neuron features on examination such as atrophy, hyporeflexia, and fasciculations. PMA begins at an earlier age as compared to ALS, and survival is often longer than ALS, with a median survival of 5 years though more rapidly progressive and more chronic forms of the disease occur

Answer 22

The polio virus infects anterior horn cells, leading to a lower motor neuron pattern of weakness similar to PMA. In those who survive, a post-polio syndrome may emerge years after recovery, marked by progressive fatigue, and weakness in muscles previously affected minimally or seemingly not at all

Saraf Tepi Flashcards

Miopati, Neuropati, Dan NMJ disorder